L25 Cystic Fibrosis 1.

Mutation- CFTR gene, codes for protein that alters normal trafficking of salts across cell membrane. Autosomal Recessive. (mother and father must carry) a. Pathophysiology- less salt is pumped out of cell, less water in airway, thicker mucous, cant get rid of mucous, mucous traps bacteria causes inflammatory response, compounds problem. 2. Clinical manifestations a. Sinuses/pulmonary i. Sinusitis ii. Nasal polyps iii. Bronchitis, pneumonia iv. Asthma v. Allergic bronchopulmonary aspergillosis b. GI Tract i. Meconium ileus (newborns) ii. Distal intestinal obstruction iii. Biliary chirrhosis / portal HTN iv. Obstructive jaundice v. Rectal prolapse, pancreatitis, gallstones c. Nutritional i. Malabsorption, esp fat- Vit A, D, E, K ii. Failure to thrive d. Other i. Delayed puberty (2 yrs avg) ii. Impaired fertility iii. Diabetes mellitus iv. Hyponatremia v. Coagulation defects vi. Anemia vii. Digital clubbing 3. Evaluation a. Sweat choride, genetic screening, spirometry/ lung volumes, chest/sinus x-ray CT, blood gas, sputum Cxs (including AFB, fungal) b. Unusual bacteria found in CF patients c. Fecal fat and elastase, CBC differential, electrolytes, Vit A, D, E 4. Bronchiectasis a. Dilated airways in lungs (1.5x), grossly visible b. Symptoms i. Chronic cough, hemoptysis, pleuritic chest pain, weight loss

ii. Saccular, cystic on CT most severe; cylindrical- most common; varicose- focal constrictions 5. P. aeruginosa high in CF patients, those with this have quicker decline of lung function and overall death rate 6. Treatment a. Bronchodilator therapy, chest physiotherapy, antibiotics, mucolytics b. Enzyme and vitamin supplements c. Flutter device- blow on them, creates back pressure, causes vibration in lungs, helps break up and push mucous up; also chest vest done same thing, externally. d. Nebulized antibiotics i. Colisitn, Tobramycin (TOBI) e. Inhale salt water f. CF surfers in Australia had better lung functions g. Transplantation- end stage option, not highly effective PEDS Sleep Disorders 1. Sleep debt a. Affects immune system, nervous system, affects cycling of growth hormone b. Sleep impairs ventilation in ALL children 2. 5 stages of sleep: 1-4, REM, 1-REM appx 100 mins., if awakened, have to start over. a. Stage 1: light sleep, in and out, sense of falling, muscle contractions, eyes move slow b. Stage 2- eyes stop, slower brain waves, bursts called sleep spindles c. Stages 3 and 4- no eye or muscle movements, when awakened- groggy, disoriented, bedwetting, night terrors, walking i. Stage 3- extremely slow delta waves with smaller faster waves ii. Stage 4- delta waves d. REM- rapid irregular breathing, eyes jerk rapidly, HR, BP, first cycle 70-90 mins., erections. 3. Apnea a. Adults 10 mins breath cessation b. Children- 2.5 missed breaths i. in children 2-6 years- same age of adenoidal and tonsillar hypertrophy. Continues through adolescense ii. African Americans, children with URI c. Due to upper airway collapse d. Worse in REM sleep e. Etiological factors i. Obesity, craniofacial abnormality, other genetic disorders f. Symptoms i. Snoring, labored breathing, difficulty in school ii. Ask about snoring

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iii. ADHD, enuresis, cyanosis, morning headaches from hypercapnia g. Diagnosis- Gold standard is nocturnal, observed full sleep study (polysomnogram) h. Treatment i. Tonsillectomy, adenotonsillactomy, uvulopalatopharyngoplasty (UP3) ii. CPAP, BiPAP, lifestyle changes Snoring a. 2% of snorers have OSAS, differs from primary snoring, have complications SIDS a. No co-sleeping, child needs to sleep on back Somnambulism a. 11-12 year olds, into adult hood b. First 1/3rd of sleep episode c. Treatment i. Get plenty of sleep, Bedtime routine ii. Topamax, benzodiasepines iii. Ground floor bedroom Night Terrors a. Sudden arousal from slow wave sleep (stage 3 or 4) Sleep Hygiene a. Avoid stimulants: caffeine, nicotine b. Avoid exercise within 6 hours of bedtime c. Alcohol initially slows brain waves, then disrupts sleep later, avoid within 4-6 hours of bedtime d. Avoid heavy meals before sleep, tryptophan (dairy) good. e. Nap- less than 1 hour, before 3 f. Special time, but no bargaining Yawning a. Color test bar pattern- 6 yawns/min b. MTV like video- 3.4 yawns/min L27 Chest Pain Exam Dx

1. General Principles of Diagnosis a. Location, radiation, duration b. Quality, intensity c. What makes it better or worse d. History of similar episodes, associated symptoms 2. Physical Exam (general for all chest pain conditions discussed below) a. Vitals, optic fundi (av nicking, yellow arterioles), neck vein distension and carotid upstroke b. Murmurs, rubs, clicks, c. 3rd or 4th heart sounds

d. Rales, wheezes, precordial thrust or heave e. Abdominal mass or tenderness f. Peripheral edema or pulse abnormality g. Atrophic skin changes- skin becomes dry and hairless- is a sign of vascular impedence. 3. Laboratory a. ECG and Chest X-ray b. Cardiac enzymes, echocardiogram, 4. Coronary Ischemiaa. History i. Classic stable angina 1. Substernal location radiation to left arm, jaw, or shoulder (Levine signfist in center of chest) 2. Often with Dyspnea (LV becomes stiffer, pressure builds up, blood backs up in lungs= shortness of breath), nausea and diaphoresis 3. Worse with exertion, relieved with rest 4. Women often have atypical signs (ex: ischemia presents as nausea) 5. May occur after eating or with cold exposure (it takes energy to digest food, requires increased blood- causing strain on myocardium). In cold, increased peripheral resistance, more volume shunted to heart- causes pain. ii. Unstable Angina 1. Similar type of pain but either: a. New onset b. Change in quality, intensity, frequency, or duration c. Becomes refractory to therapy d. Occurring at rest e. Most frequent time for MI- right before awakening. iii. Mixed Angina 1. Same symptons as other anginas, but is unpredictable when it occurs b. Physical i. Signs of Atherosclerosis 1. Arcus senilis (white gray or blue opaque ring in the corneal margin), 2. optic fundi, atrophic skin, bruits ii. Cardiac ausculations 1. S3 or S4 (both diastolic sounds. S3 reflects passes filling of ventricle in diastole, S4 represents active filling of ventricle) too much blood filling in ventricle causes sound. S4 due to stiffening of ventricle. iii. Chest examination 1. Precordial diffuse lift or displaced PMI 2. Pulmonary rales c. Laboratory i. ECG: ST segment elevation or depression; may be normal.

1. CXR: silhouette sign, calcification of aortic arch 2. Blood test- hyperlipidemia- presents risk only d. Testing i. Stress test, nuclear stress imaging, fast computed tomography, cardiac catherization Pericarditis a. History i. Pain similar to ischemia ii. Relieved by sitting forward (angina will not find relief) iii. Associated with viral syndrome, post infarction, or post sternotomy (surgery) iv. Often seen in chronic inflammatory like RA b. Physical i. Pericardial friction rub (you can hear it) ii. No signs of atherlerosclerosis c. Testing i. Echocardiogram looking for fluid (which causes the rub sound) Mitral Valve Prolapse a. History i. Substernal chest pain with or without radiation ii. Most common in young thin healthy females, family history of MVP b. Physical i. Classic mid-systolic click and murmur ii. Pectus excavatum c. Testing i. ECG best way Pulmonary Embolism a. Historyi. sudden onset of unilateral chest pain (on side where embolis is) ii. Pain often worse with inspiration b. Physical i. May have pleural rub Esophageal Spasm a. History i. Mimics angina so difficult to distinguish, occurs after eating ii. Dysphagia or reflux may be associated, will help to Dx b. Physical i. Epigrastric tenderness c. Testing i. Upper GI barium swallow (will first get work up for cardiac) Musculoskeletal Pain a. History

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i. Associated with sudden increase in physical activity (occurs afterward, whereas MI would occur during) ii. No or little radiation pattern 10. Pleurisy a. History i. Similar to PE. Pleurisy is result on infection or inflammation, worse with position or inspiration 11. Aortic dissection a. History i. Tearing pain worst pain I have ever experienced, worse then MI pain ii. Sudden onset, radiation across chest b. Physical i. Different BP in arms or legs L28 Non-Invasive Cardiac Testing 1. Echocardiography (ultrasound)- for assessing structures, wall motion a. Transthoracic, transesophageal, echo with stress test b. Air and fat obstruct view c. Gives info on structural things (chamber size, masses or thrombi, wall motion, septal defects, etc) d. Clinical i. Chamber size: cardiomyopathy or LVH ii. Wall motion abnormalities: ischemia or infarction iii. Anatomic wall defects iv. Cardiac tumors or vegetation e. Doppler Echocardiogram i. Not separate in real life, doppler is included in echo. For the below, if asked on test, answer Doppler (Doppler detects motion) 1. Murmur assessment 2. Doppler shows motion of blood through heart 3. Detecting regurgitant lesions, volumes 4. Detect and quantify shunts (doppler shows how much flow going through hole) f. Stress Echocardiography i. Applications 1. Detect ischemic myocardium 2. Detect viable myocardium 3. Must have good technician/knows what they are doing ii. Advantages 1. Improves accuracy of EKG iii. Methods

1. Treadmill (best one), bicycle, arm iv. Indication (reason to do it) 1. #1- evaluate for chest pain 2. Evaluate arrhythmias v. Absolute contraindications (use common sense) 1. Acute MI 2. Uncontrolled CHF 3. Severe aortic stenosis 4. Uncontrolled arrhythmia vi. Relative contraindications 1. IHSS with significant obstruction 2. HTN 3. Physical limitations (patient with walker???) vii. Endpoints (goal of stress test) 1. Angina 2. Patient cant go anymore 3. Stop when other concerning symptoms begin to occur viii. Interpretation 1. ST segment depression 1mm or more a. 1.5 mm is 90% specific b. Women have 50% false positive rate ix. False Negatives 1. Women 2. Poor exercise tolerance 3. Nitrate, beta blocker, antiarrhythmic medication therapies x. Nuclear Medicine Cardiac Imaging 1. Increases sensitivity/specificity of stress test 2. Shows uptake in cardiac muscle 3. Cold spot imaging a. Thallium and technetium are taken up by viable myocardium b. Cold spot seen in areas of ischemia or scar c. Amount of uptake correlates with blood flow to area 2. Long Term Ambulatory Monitoring (Holter) a. Records all beats over 24 hours b. Detects arrhythmias, ischemia c. Patient must activate to record, holds up to chest when feels palpation, etc d. Continous loop monitor i. Patient does not need to activate, continuous monitoring 3. CT Angiograpy a. Injected die shows epicardial vessels shown in 3D b. Determines degree of coronary calcification c. Correlates well with coronary events

d. Not effective in determining lesion flow 4. Cardiac MRI a. No dye needed, shows 3D of heart, MRI good for structure, but thats about it.

Knee
1. Focused History a. Onset- Acute vs. Chronic b. Location, mechanism, associated symptoms c. Dont forget to check hip! 2. Most common Dx- patella femoral pain syndrome a. Pain at patella on femur b. Movie theatre sign, or students getting out of class, or pain going down stairs c. J sign patella moves laterally when knee extended d. Xray not necessary for Dx e. Treatment i. Ibuprofen, PT, or home exercise to strengthen quadricep, kinesia tape 3. Meniscus Tears a. Medial most common, twisting injury, popping, locking b. Mechanical loss of motion = surgery 4. ACL tear a. Twisting of fixed leg, hyperextension or anterior translation of tibia b. Female soccer players are most common affected c. Unhappy triad: ACL, MM, MCL tears; bone contusion d. Treatment i. RICE and ROM exercises ii. Young or active patient = reconstruction 1. Hamstring tendon, quads tendon, cadaver, bone patella bone grafts iii. Older patient- rehabilitation and bracing e. Post surgical repair i. Rehab!!! ii. Loss of motion most common complication iii. Proprioception iv. Bracing v. Return to play- 6 mos. 5. Degenerative meniscus, Osteoarthritis injury a. 4 Rads signs of osteoarthritis i. Joint line space, sclerosis, sub chondral cysts, osteophytes b. Treatment i. NSAIDS, activity!!, heat, intra articular injections 6. Osgood-Schlatters Disease a. Apophysitis of Tibial Tuberosity

b. Peds ages 11-13 c. Pain with kneeling, running, jumpin 7. IT band syndrome a. TFL running from ASIS to Gerdys tubercle b. Test with Obers test c. Common in runners and cyclist, worens with flexion/extension 8. Pre-petallar bursae (nurse maids) Hip 1. True hip pain is felt in groin 2. Lateral femoral cutaneous can cause hip pain 3. DDH- Developmental dysplasia of hip a. Risk factors i. Family history, Oligohydroaminos, first born, female, breech presentation, Caucasian, and Native American b. Signs i. Unusual skin fold, leg shorter than other c. Tests i. Ortolani; abducted reduces an unstable hip ii. Barlow: adducted, displaces an unstable hip clunk d. Treatment i. Dynamic positioning devices ii. Surgery for open reduction 4. Legg- Calve- Perthes disease a. Avascular necrosis of the femoral head b. Idiopathic c. Males 4-8 yrs old d. Ligamentum teres vessels e. Symptoms, i. Limp, groin pain, knee pain, pain worse at night, guarding, flexion contraction, limited Abduction and internal rotation. f. Dx- on x-ray crescent sign i. MRI- head of femur is gone g. Treatment i. Activity restriction, abduction bracing, surgery , may need early total hip 5. Slipped Capital Femoral Ephiphysis a. SCFE b. Symptoms i. Limited internal rotation; external rotation on flexion of knee ii. Kleins line- line does not dissect head of femur c. Treatment

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i. Non weight bearing until surgery ii. Delay in treatment = AVN iii. Surgical fixation with 1 or 2 screws Transient Synovitis of the hip a. Sterile effusion of the hip b. Children ages 2-7 c. Limp, abductor lurch (trundlenburg Dislocated hip a. Trauma, posterior 90% b. Affected hip is Flexed, internally rotated, adducted c. Reduce! ASAP Hip Fracture a. High mortality and morbidity b. Clinical symptoms i. Pain after a fall; hip, groin, butt ii. Affected limb ext. rotated and abducted and shortened c. Treatment i. Surgery Stress fracture of the hip a. Fatigue fracture b. Often missed and delayed Dx c. Military recruits and runner d. Tension vs compression type e. Vague groin pain f. Dx- usually use MRI Osteoarthritis of hip a. Usually bilateral b. Cardinal x-ray signs- minimal joint space, subchondral cysts, osteophyte, hypertrophy of femoral head c. Thigh pain, groin pain, decreased ROM (stuck- cant internally rotate) d. Treatment i. Activity, as long as does not cause pain ii. Hip arthroplasty Greater Trochanter Bursitis a. Hurts when getting out of seat b. Tool belt neuropathy pain at lateral femoral cutaneous n. (sensory nerve) Ischemic Syndromes (Atherosclerosis- #1 killer in US)

1. Pathophysiology a. Ischemic Cascade i. : O2 availability, ATP, actin myosin unbridging, diastolic stiffness, contractility

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ii. : end-diastolic filling iii. ECG changes, Pain b. Plaque Morphology i. Endothelial injury > monocyte/macrophage infiltration > LDL oxidation/fixation > endothelial/medial hyperplasia > fibrous cap > central degeneration c. Placque rupture i. Thin cap is ruptured by shear force. Presentation of Angina a. Substernal heavy or squeezing pain b. Radiation down left arm, to jaw, or neck; may occur in epigastrium; may have dyspnea instead of pain Classification of Angina a. Stable angina i. Related to physical exertion, never at rest, reproducible. Stability not correlated with severity. Sedentary lifestyle people dont get this. b. Unstable Angina i. Any change in pattern, frequency, severity or quality of angina ii. New onset angina; angina at rest c. Mixed angina i. Component of stable angina ii. Often has pain variably at rest and activity d. Crescendo angina i. Progressively severe symptoms over short period of time ii. Requires most aggressive therapy e. Myocardial infarction i. Prolonged, severe pain or dyspnea ii. Plasma enzyme assay shows myocardial damage Consequences of Coronary Ischemia a. MI, arrhythmias, ischemic dilated cardiomyopathy, CHF, ischemic papillary muscle dysfxn with mitral regurgitation Prognosis a. Diabetes and metabolic syndromes > highest risk factors for coronary disease b. Crescendo angina most likely to dev into MI c. Diabetes, HTN, centripetal obesity, Treatment strategies a. Aggressive/Interventional i. Hospitalize in critical care unit 1. IV nitrates, beta blocker, anti-platelet therapy, anticoagulant 2. Cath lab to find source of blockage b. Conservative i. For higher risk patients, Hospitalize in ICU 1. IV heparin, nitroglycerin and 2b3a inhibitor

a. Aspirin, beta blocker b. No thrombolysis ii. For lower risk patients, begin oral therapy without heparin 1. May only need 24 hr observation, no ICU 2. Oral nitrates, beta blocker, aspirin 3. Heparin not needed and NO thrombolysis c. Outpatient management i. Aggressive control of glucose, BP, lipid intake ii. Attain ideal body weight, exercise, smoking cessation 7. Prognosis a. If no therapy, risk of angina, MI b. If aggressive therapy given > reduces event risk by up to 50% (in most cases) c. Conservative therapy- up to 50%; comorbid risk factors play major role in success Myocardial Infarction (MI) 1. Risk Factors a. : cholesterol, lipidemia, HTN, smoking; low HDL; hypervlycemia, DM, obesity, type A personality, genetic factors 2. History a. Prodromal chest pain seen in 40-60% of patients b. Substernal squeezing or ache (pretty severe). Can be sharp or heavy. 5-30 min, if longer, patient is infracting c. Radiation to left arm, hand, neck or jaw d. Diaphoresis, nausea e. Atypical characteristics i. Pain radiating to the right side ii. Pain in fingers rather than the arm iii. Pain lasting less than 5 ins or more than 1 hour 3. Physical Examination a. Appearance/Vital signs i. Pallor, diaphoresis, dyspnea ii. Normo to HTN iii. Low grade fever hours after infarct b. Jugular venous pulse i. If elevated, may indicate right ventricular involvement ii. If associated with HTN or rales, consider CHF c. Heart Sounds i. S4 common at APEX ii. S3 indicates severe damage with impending heart failure iii. New murmur indicates mitral regurgitation due to papillary muscle iv. Pericardial friction rub can be heard late in course in 20% of all cases

d. Lab evaluation i. Troponin T and I are sensitive and early markers of myonecrosis (1-2) hours ii. Myoglobin early marker, but is non-specific iii. CPK 1. MB band- myocardial muscle 2. Rises within 4-6 hrs, peaks at 12-36 hours 3. Size of infarct related to total CPK measured over time iv. SGOT (AST) 1. Rises 8-12 hrs post infarct 2. Peaks b/w 24-36 hours 3. Falls to normal in 3-4 days 4. Elevated with liver disease and skeletal muscle injury v. EKG findings 1. First finding in ST segment2. ST segment- (STEMI)- worst kind of MI 3. Non-STEMI- less total damage then STEMI, associate with more extensive coronary plaque vi. Hemodynamic classification 1. Clas 1-4: a. 1- normal b. 2- filling pressure with normal CO c. 3- normal filling pressure with CO d. 4. CO and Filling pressures 4. Complications a. Arrythmias i. Ventricular premature beats 1. Benign unless complex 2. Common in acute phase ii. Ventricular tachycardia 1. Lethal, requires emergent therapy 2. Treat with DC cardioversion or antiarrythmics and cardioversion iii. Accelerated idioventricular rhythm 1. Common sign of reperfusion after thrombolysis 2. Usually well tolerated and self-limiting 3. May need anti-arrhythmic if persistent iv. Atrial fibrillation/flutter 1. Occurs in 10-15% 2. Usually transient, and is marker of LV dysfunction b. Conduction Disturbances i. First degree AV block: common with inferior MI. Usually asymptomatic ii. Second degree AV block type I (Wenkebach) 1. Indicates more advanced AV node disease

2. More common with inferior MI, and often benign iii. Second degree AV block type II 1. Similar to type I, but usually requires pacing unless completely stable and asymptomatic iv. Complete heart block 1. Requires pacing c. LV failure i. Signs: pulmonary congestion and low cardiac output ii. Highest risk marker for subsequent cardiac mortality d. Mitral regurgitation i. Due to papillary muscle dysfxn e. LV aneurysm i. Late complication, usually of anterior MI ii. May cause refractory heart failure, arrhythmia, or embolus f. RV infarction i. Associated with inferior wall MI ii. Suspect when BP remains low in spite of therapy iii. Treat with high volume infusion g. Thromboembolism i. Most common with anterior or large MI h. Cardiac rupture i. More common in elderly, females, first MI. ii. Detected by sudden loss of pulse and electrochemical dissociation. 5. Treatment a. IV thrombolysis i. Requires ST segment elevation in two leads, NOT ST depression ii. Time window less than 12 hours, ideally less than 6 from onset of pain iii. Contraindications include active bleeding, recent CVA, surgery, bleeding disorder, trauma b. Adjunctive therapy i. Full dose heparinization ii. IV nitroglycerin, aspirin, IV beta blocker followed by oral, ACE inhibitor, 2b3a inhibitor, O2 c. IV therapry (acute angioplasty) i. Effective if less than 6 hrs from pain onset ii. Depends on skill of operator d. Misc therapy i. Morphine for pain relief Ostearthritis

1. Athritis Cascade a. Noninflammatory i. Hemophelia, PVNS, synovial sarcoma, osteoarthritis, avascular necrosis, mechanical problems b. Inflammatory i. Infectious 1. Viral, bacterial, etc a. Distribution i. Primarily monoarticular ii. Source is the most important question to ask! ii. Crystalline 1. Gout- uric acid 2. Pseudogout- calcium pyrophosphate 3. Basic calcium deposition 4. Distribution a. Mono to polyarticular b. May be underlying condition c. Occasionally systemic manifestations iii. Autoimmune 1. Erosive a. RA, spondyloarthropathy, IBD associated b. Symmetrical: RA, PsA i. DIP vs. PIP involvement c. Assymetrical: PsA, spondyloarthropathy, IBD assoc. 2. Non- erosive a. Lupus, MCTD, Vasculitis, myopathy 2. Things to consider a. Onset, distribution, stiffness, swelling, back pain/spinal involvement b. RA and Lupus do not involve the back!!! c. systemic manifestations i. Weight loss, fatigue, anemia, organs involvement, mucocutaneous manifestations 3. Back pain: Selected Causes a. Can be caused by spread of prostate cancer, or intestinal diverticulum b. Infections c. Non-Infectious/inflammatory d. Noninfections/noninflammatory 4. Classification of joint effusions (leukocytes counts) a. <200- normal; non-inflammatory- 200-2000; inflammatory 2k-10k; septic >50k 5. Osteoarthritis demographics a. Hand and knee in women and AA; increase with age, leading cause of disability; 80% over 65 have evidence of OA of hands

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b. Risk Factors i. Age, female, obese, hereditary, trauma, neuromuscular dysfxn, metabolic disorder Secondary Osteoarthritis Causes a. Dysplastic i. Chondrodysplasias, epiphyseal dysplasia, dev disorder, leg length inequal b. Skeletal failure 1. osteonecrosis, osteochondritis, pagets c. Postinflammatory 1. infection, inflammatory arthropathies d. Endocrine and Metabolic i. Acromegaly, ochronosis, hemochromatosis, crystal deposition disorders e. Connective Tissue i. Hypermobility syndromes ii. Mucopolysaccharidoses f. Misc i. Frostbite, hemoglobinopathies Osteoarthritis general features a. Am stiffness, crepitus, angulation deformity b. Lab: non-inflammatory synovial fluid c. Heberdens and Bouchards nodes and synovial cysts (Beverly Hills d. Dx made by H and P!! , labs not very helpul e. Primarily occurs in weight/gravity bearing joints Diifuse idiopathic skeletal muscle hypertosis a. Occurs in: lumbar spine and C spine Underlying Disease Association of OA and CPPD Disease: a. Hs, neuropathic joints, trauma, aging Management of OA a. Decrease pain to increase fxn, exercise, patient education on lifestyle, self treat b. Drugs- opiod and nonopiod analgesics, topical agents, intra-articular agents, NSAIDs, unconventional therapies Co-morbidities a. Prior Hx, age >65, smoking and ETOH b. Gastric protection i. PPI, MIsoprotosol, COX-2 selective NSAID c. H2 blockers are ineffective and increase the risk of PUD d. CHF and chronic renal insufficiency Strengthening Exercises a. 2 steps i. 1- lift against gravity ii. 2- increase resistance, free weights Reconditioning Exercises

a. Low impact, continuous movement for 15-30 mins 3x /wk b. Aquatics, minimal tension exercises 14. Nonopiod Anagesic Therapy a. Acetaminophen is first line b. NASAIDs i. Use prescription strength (Aleve, double dose if not working) ii. ALL NSAIDS INCREASE CV RISK!!! c. Tramadol i. May be added to NSAIDs ii. Affect opioid and serotonin pathways Septic and Reactive Arthritis 1. Infections Arthritis Presentations a. Septic i. Nongonococcal ii. Gonococcal b. Responses due to systemic infection i. Viral, rheumatic fever ii. Reactive arthritis 1. Reiters disease, post streptococcal (nonRF), subacute bacterial endocarditis (valve) 2. Demographics a. Greater incidence with RA or joint replacements b. Septic arthritis is rheumatologic emergency! 3. Septic Athritis a. Mono-arthritis is septic arthritis until proven otherwise! Dd b. Abcess- CDTR- calor, dolor, tumor, rubor c. Organisms i. MRSA, gram positives (70%) 4. Gonnococcal Arthritis a. Most common in younger, sexually active b. 1 day to 2 mos after sexual contact, but avg is 5 days c. Disseminated gonococcal arthritis (DGI) d. Risk factors i. Menstruation, pregnancy, C5-9 deficiency 5. Know your bugs! a. Some groups more prone to some than others i. Salmonella- SLE, HIV, sickle cell anemia ii. Pseud aerug- IVDA iii. Staph epi- joint replacements b. Age has risk factors for certain infections

6. Sites of infection a. Large joints (Knee, hip, shoulder) more common than smaller ones 7. Dx- Arthrocentesis a. Mandatory if septic arthritis suspected b. Has diagnostic and therapeutic effects (drain abcess, synovial biopsy) c. No absolute contraindications (red around joint is though, could be cellulitis) d. Potential complications- infection, bleeding, local pain, cartilage injury 8. Arthrocentesis tests a. Culture and sensitivity: MOST IMPORTANT! b. Others: gram stain, crystals, glucose, cell count with differential 9. Fluid Results a. 75% of time is Gram Positive b. Non-gonococcal drops sharply after antibiotic therapy 10. Imaging in septic arthritis a. Xray, CT, MRI, etc b. Elevated white count is inflammation 11. Most important question: How did the infection get there? a. Hematogenous most common i. Transient bacteremia from other sources: lung, renal/GU, GI ii. Direct extension from skin (cellulitis) iii. Trauma b. Gonoccocal i. Could be reoccurrence of STD. check for chlamydia, HIV status 12. Spinal Septic Arthritis a. Chronic, unrelenting back pain, fever, tenderness b. Thoracocolumbar region, infection usually crosses disc space c. TB (Potts disease): T10-L2 region; paraspinal abcess 13. Infected joint prosthesis a. S. aureus, s. epidermidis b. Prophylaxis prior to invasive denta, GI or GU procedure 14. Treatment of Septic Arthritis a. Needs to be drained daily b. Watch for MRSA c. Oxacilin/Nafcillin with Cetriaxone, Ceftazidime d. IVDA most frequently has pseudomonas infection 15. Arthritis associated Systemic infections a. Viral, Hep B, C, HIV, rubella, parvovirus 16. Hep associated Musculoskeletal syndromes a. HAV- transient arthralgia b. HBV- abrupt, symmetrical, severe polyarthritis in prodrome c. HCV- often Rh factor positive 17. Parvovirus- looks just like RA (joint swelling in hands)

18. Lyme disease a. B. burgdorferi, 3 stages i. Stage 1- erythema migrans, lymphadenopathy, Bells palsey, flu-like ii. Stage 2- possible carditis, Bells, conjunctivitis, myosititis, diffuse erythema, migratory arthritis iii. Sensorimotor neuropathies, acrodermatitis chronica atrophicans, meningoencephalitis iv. Elisa testing. Positive test followed up by western blot. 1. IgM two or more bands positive 2. IgG 5 or more bands positive 3. Frequently false positive with 41-kD flagellin protein v. Treatment 1. Oral antibiotic- Doxycycline 2. Parenteral (IV) antibiotics for severe neurologic and cardiac treatment 19. Rheumatic Fever a. Has skin and joint involvement b. Jones criteria (Joints, Cardiac, Neuro (chorea), erythema marginatum, skin (nodules) i. 2 major, or 1 major + 2 minor and recent strep infection c. Treat with Benzathine Penicillin IM initiall, followed by 10 days or Penicillin or Erythromyocin 20. Osteomyelitis (of back) a. Bone resistant to infection b. If infection occurs, neutrophils > puss > necrosis c. Most common causes- open fracture, surgery, prosthetic device d. Elderly and children most likely i. Diabetics, in feet, chronic ulceration e. Labs i. Elevated WBC ii. Must identify organism 1. Neonates- S. aureus, Streptococcus 2. Adults- S. aureus 3. Elderly- gram (-) f. Imaging- CT, MRI, Nuc i. MRI- sensitive but non specific ii. Bone scan g. Prophylaxis i. Ortho procedures 1. Anti-staph PCN or 1st, 2nd, 3rd gen cephalosporins h. Antibiotic therapy i. IV x 4-6 wks ii. Children- possible change to oral antibiotic iii. Possible surgical debridement

1. Use of skin, bone grafts L35 Shoulder 1. 4 articulations a. Glenohumoral, Acromioclavicular, scapulothoracic, sternoclavicular 2. Rotator Cuff a. Supraspinatous i. Motion- elevate shoulder up to 90 degrees ii. Innervation- suprascapular n. b. Infraspinatous i. Motion- abduction, external rotation ii. Innervationc. Teres Minor i. Motion- external rotation ii. Innervation- axillary n. d. Subscapularis i. Motion- internal rotation ii. Innervation- subscapular n. e. Function i. Stabilize joint, depresses humerus into glenoid 3. Exam- always start with neurologic exam!!! a. Neurologic i. C5- Deltoid- sensory to cap of shoulder; reflex: none ii. C6- biceps, sensory to thumb and finger; reflex- biceps iii. C7- triceps, sensory to middle finger, refelex- triceps b. Inspection i. Appearance, alignment, position ii. Neurovascular exam (C5-T1), brachial and radial pulses, color and warmth iii. Atrophies or wasting (deltoid, rotator cuff, winging scapula) c. Palpation d. ROM i. Apleys 4. DDx of shoulder trauma a. Glenohumoral subluxation or dislocation b. Ac joint separation c. Fracture d. Brachial plexus injury 5. Case 1 a. 25 y/o male landed directly on shoulder during rugby game. Has severe pain, holding affected arm close to his body, obvious discomfort and pain.

i. No deformity, pain limiting full abduction and forward flexion, pain and mild edema over AC joint, clavicle has 2 mm horizontal translation vs non-affected side, normal strength test, pain with cross arm resisted adduction ii. Dx = AC joint separation 6. AC Joint Separation Grade 1 2 3 AC ligaments Sprained Disrupted Disrupted CC ligaments Intact Intact Disrupted Distal Clavicle Intact Intact Superior displacement

7. Treatment a. Type I and II- conservative treatment- sling; Type III, consider surgery 8. Case 2 a. 32 y/o female FOOSH from bicycle. Falls with arm planted away from body (aBd), and she rotated around her arm as she fell (external rotation). Has severe shoulder pain. i. Inspection- holding are in slight abduction and external rotation; normal neuro exam, very limited ROM; tender to palpation ii. Imaging- humeral head displaced from glenoid 9. Shoulder reduction a. Hippocratic method i. Foot in pit, traction at 45 b. Traction and counter traction i. Uses towel c. Stimsons method i. Use weight, 5 lbs, slow gradual return to position 10. Glenohumora dislocation a. 90% are anterior i. Occurs when arm in abducted and external rotation (FOOSH) ii. Compications 1. Axillary artery or nerve injuries 2. Fractures of the glenoid (bankart lesion) and humeral head iii. High incidence of recurrence b. Posterior in seizure or electrocuted patient (boards questions) 11. Clavicle fractures a. 80% midshaft; 15% distal clavicle, 5% proximal b. Treatment i. Figure 8 strap or sling for 6-8 wks ii. Surgery 12. Proximal Humeral head fracture a. Axillary a. and n. injury b. Most managed with sling

13. Case 4 a. 56 y/o male 7 mos anterior lateral shoulder pain, + at night when trying to sleep on affected side, + pain with reaching and overhead activities. No radicular pain, weakness or numbness. No event i. DDx- chronic shoulder 1. Osteoarthritis, rotator cuff impinged or tenidonosis, adhesive capsulitis, cervical disc disease, thoracic outlet syndrome ii. Pain at mid arc of abduction iii. Positive Neers and Hawkings (pain with respective motions) 1. Neers- forward flexion 2. Hawkings- forward flexion and internal rotation iv. DDx = shoulder impingement v. 4 signs of arthritis on x-ray 1. Joint space, osteophytes, sclerosis 14. Rotator Cuff Tears a. 25% of patients older than 60 b. Rare in patient under 40 i. Overhead athletes, trauma c. Degen process that originate in supraspinatus d. Tears dont heal, but symptoms may improve 15. Case 5 a. 38 y/o female with DM presents with R. sided shoulder pain after heavy lifting. b. Passive assistance shows little improvement in ROM c. Dx- Adhesive Capulitis i. Imaging rules out other suspected Dx ii. Treatment- OMM, NSAIDS, Cortico injection, aggressive physical therapy iii. 80-90% will improve in 4-6 mos w/out surgery 16. Case 6 a. 42 y/o male with chronic shoulder pain presents with acute worsening of pain after doing yard work yesterday. Lifted heavy bag, heard pop in shoulder. Now has bump and bruising on anterior shoulder. Diffuse but mild pain. i. Dx- ruptured proximal biceps tendon 1. + Yergasons, +Speeds, +Hawkings, +Neers 2. Normal strength with exception of supination 3. Long head of the biceps from the supraglenoid tubercle, usually in older patient with hx of chronic rotator cuff 4. MRI not necessary d 17. Brachial Plexus Injury a. Burning and paresthesia in are of nerve affected b. Weakness of muscles innervated by affected nerve c. Athlete will try to shake it out d. Treatment

i. Rest, short as 1-2 hrs, long as 4-6 wks e. Bilateral Sx = spinal cord injury until proven otherwise! f. Acute brachial neuritis (Parsonage- Tuner syndrome) i. Uncommon, mistaken for shoulder radiculopathy or other shoulder injury. Unknown etiology, may be viral ii. Acute onset of servere shoulder pain, wake from sleep, usually axillary nerve iii. Dx- of exclusion iv. Tx- rest from activity for improvement, 3-4 mos before regain strength. Pagets Disease 1. Focal, Metabolic disorder in aging bones a. Accelerated remodeling, overgrowth but impaired bone integrity b. Skull, spine, pelvis, long bones 2. Presentation a. Often asymptomatic b. Incindental finding on imaging for other reason c. Chem panel - alkaline phosphatase 3. Clinical Manifestations a. If symptomatic i. Bone pain, deformity, fractures, nerve root impingement, tumor, headache, osteoarthritis, surgical bleeding due to excessive vascularity of affected bones 4. Occurrence a. 2-9% of affected pops b. Typical patient 55 y/o Euro male 5. Pathogenesis a. Disease of osteoclasts b. nucleoli number in each osteoclast c. Too many osteoclasts d. Hypersensitive to Vit D e. Treatments aimed to suppress osteoclasts activity 6. Genetic Association a. AD, variable penetrance b. P 392L of SQSTM1 domain, and other mutations can cause/effect i. Affects osteoclasts signaling via receptor activator for nuclear factor kB ligand (RANKL) ii. Viral etiologies being investigated 7. Histopathology a. Irregular cement lines, mosaic patterns b. Normal bone marrow replaced by vascular stromal tissue 8. Pain a. Later in disease course

9.

10.

11.

12.

13.

14.

b. Mild-moderate deep aching c. All day, at rest, night, worse with weight bearing d. Possible causes: hyperemia, microfractures, periosteal changes Addtl. Clinical Features a. Skull: hydrocephalus: unsteady gait b. Angiod streaks, dilated scalp veins c. Malocclusion, periodontal disease d. Vascular steal: apathy e. Imaging: Tam O Shanter, basilar invagination f. High ouput CHF g. Lumbar spine stenosis, compression fracture h. Kidney stones Neoplasia a. Osteosarcoma- rare though b. In extensive Pagets c. Giant cell tumor- usually benign Lab a. Normal serum calcium, phosphorous b. serum (sAP) usually sufficient c. Urine hydroxyproline Imaging a. Diagnosis is primarily radiographic b. Tunnelling c. Early ostyolysis d. Skull- early osteoporosis circumscripta e. Trabecular markings f. Distorted bone overgrowth g. Subchondral progression in one direction flame shaped h. Protrusion of acetabulum into pelvis i. Triangular pelvis i. Enlarged, dense vertebrae ivory (superior may be larger than inferior vertebrae, usually is the opposite) j. Pseudofracture fissuring on surface of bowed bones k. Bone scan more sensitive than xray l. MRI helps differentiate lytic Pagets from sarcoma m. PET may give false + for neoplasm n. Bone biopsy: only for atypical lesions or to diagnose osteosarcoma Goals of Treatment a. Ease pain b. bone remodeling, bone vascularity, disease progression, bone scan isotope uptake; deposition of lamellar bone, healing of lytic lesions on x-ray Treatment but no symptoms

a. Serum Alk. Phos. > 2-4x upper limit of normal b. Sites where complications could occur i. Skull, spine, weight bearing bones, bone abutting joint c. If no Rx, monitor yearly 15. Other reasons to treat a. Plans for surgery at active site b. Multiple sites who are immobile with Ca of no other etiology (no hyperthyroid, cancer) 16. Antiresorptive treatments a. Target i. Osteoclasts 1. resorbtion in days to weeks = urine hydroxyproline, n-telopeptides 2. bone formation in weeks to months = bone alkaline phosphatase b. N containing bisphosphonates (**work better and longer) i. Zolindronic acid, pamidronate, risendronate, alendronate, ibandronate ii. Sustained suppression of bone turnover without impaired mineralization iii. Better than simple biosphosphonates 1. Etidronate, tiludronate iv. Calcitonin: less potent and less likely to produce sustained remission 1. Use if patient intolerant to bisphosphonates 17. Images a. Tam OShanter Skull i. Increased skull density ii. Multiple osteoblastic and lytic lesions. salt and pepper appearance iii. Prominent temporal artery, deafness b. Lower extremity deformity c. Hydrocephalus d. Histologica- giant osteoclasts resorbing bone i. Marked irregular thickening of trabeculae e. Pelvis- lytic and sclerotic lesions, pelvis is enlarged L37 Rheumatoid Arthritis 1. What is RA? a. Chronic, systemic, idiopathic, non-suppurative, inflammatory, arthropathy, diarthrodial joints, extra-articular features 2. Immunopathogenesis of RA a. Rh factors produced in RA synovium > Rh factors fix complement > complement consumed in RA joint > complement factors recruit and activate PMNs > new antibodies now implicated. 3. RA Pathophysiology a. Genetic factors

4. 5.

6. 7.

8.

9.

10.

11.

12.

13.

b. Activation of T cell by a putative antigen in a genetically susceptible patient c. B lymphs produce autoantibodies, secrete pro-inflam cytokines and can act as antigen presenting cells. d. Synovial proliferation leads to pannus that invades cartilage and bone. Military with anti-CP, 49% developed RA Epidemiology a. Women > men b. Peak age: 24-45 yrs c. Disconcordance in monozygotic twins d. Disease improves during pregnancy, and flares 4-6 weeks post partum Why is RA importuning a. Morbidity, peak in young adults, disabling, economic cause, no entirely satisfactory Tx 2010 RA Criteria a. 6/10 = definite RA b. Categories i. Joint involvement, serology, acute phase reactants, duration of symptoms RA Involved areas a. Almost always hands and feet, rarely cervical spine b. OA rarely involves ankle, unless b/c of trauma c. RA is a symmetric disease d. Prayer sign e. Those with Rh nodule are Rh factor positive 100% of the time Hand Deformities a. Swan Neck (DIP hyperflexion, PIP hyperextension) , Boutonniere (PIP flexion, DIP extension) Extra-articular manifestations a. Rh nodules, Sjogrens, Feltys, vasculitis, Rh lung, cardiac disease, neuromyopathy, inflammatory Eye Dx, osteoporosis, lymphadenopathy, hyperviscosity, cryoglubulinemia, dermatologic, amyloidosis Cervical spine involvement a. Swelling in bursa around odointed process (AA joint), allows dens to move posterior when neck is flexed b. Severe neck pain radiating to occiput c. Loss of motor function in arm, legs. Dyasthesias of the fingers and feet d. Disturbed bladder function e. Spinal cord involvement is called myelopathy Eye involvement a. Inflammatory eye disease i. Scleritis, scleromalacia Sjorgens Syndrome a. Sicca symptoms include dry eyes, dry mouth, vaginal dryness, trachea-bronchial dryness b. Primary Sjorgens syndrome associated with SS-A (Ro) and SS-B (La) + antibodies

14. 15.

16.

17.

18.

19.

c. Treated sx i. Anti-inflam and immunosuppressive d. Autoimmune Exocrinopathy Lab Findings a. Rh facro, anti- CCP, ESR or CRP, anemia, thrombocytosis, ANA+, splenomegaly Anti-cyclic citrullinated Peptide anti-bodies a. Marker for RA b. RF+ and Anti-CCP = 99.5% specifity for RA Treatment a. Mainly Pharmacologic i. NSAIDS 1. Dont halt disease progression 2. Associated with toxicities that may be costly 3. Efficacy/toxicity lead to frequent switching ii. Corticosteroids 1. Chronic use associated with many side effects iii. DMARDs 1. Discontinuation rate high (toxicity/efficacy) 2. Need for continued monitoring 3. Delayed onset of action iv. Analagesics 1. Absolutely necessary to control pain v. Non-pharmacologic 1. PT, rest, articular rest, exercise, heat and cold, assistive devices, splints, weight loss DMARDS a. Hydroxychoroquine, sulfasalazine, gold compounds, azathioprine, cyclosporine, methotrexate, leflunomide Methothrexate a. Prosi. once a wk dose, long term clinical experience, favorable rate of continuing therapy, proven efficacy in moderate to severe RA b. Cons i. Lab monitoring every 4-8 wks 1. CBC, LFTs, Creat ii. Toxicities: hepatic, myelosuppression, pulmonary, abortive agent Leflunomide a. Pros i. Well absorbed p.o., early onset of action, stabilized benefit of long term use, selectively targets autoimmune lymphocytes to reduce AEs, rapid excretion w/ cholestryramine b. Cons

i. Lack of clinical experience ii. Toxicities: hepatic, gastrointestinal, teratogenic!!! Abortive agent 20. Biologic Therapies a. Most effective, but cost ineffective. b. Increased risk of infection, reactivation latent TB, neoplasia, MS, autoimmune disease 21. Remission Criteria a. >5 criteria must be met for >2 mos b. AM stiffness < 15 mins, no fatigue, no joint pain, no joint tenderness or pain in ROM, no STS in joints or tendon sheaths, ESR <30 males; <20 female Juvenile RA 1. Mono, oligo (2-4), Pauci (<5), extended Pauci - >5, Poly 6+ joints 2. Demographics a. Onset usually < 9 y/o b. F > M 3. Classification criteria a. Age of onset < 17 yrs b. Arthritis in 1 or more joints c. Duration of disease >6 weeks d. Type of onset of disease during the first 6 mos i. Polyarthritis, pacuciarthritis, systemic disease e. Exclusion of other forms of JRA 4. JRA- Differential Dx a. Severa: SLE, reactive arthritis, vasculiits, septic arthritis, neoplasia, Kawasake, Rheumatic Fever 5. Characteristic of Growing Pains a. Most common in children 6-13 yrs of age b. Usually lower extremities c. Pain localized to thighs calves and shins (not joints) d. Correlation with strenuous exertion is variable 6. Subtype Classificationa. Systemic Onset (10-15%) i. 1 or > joints involved ii. Extra-articular features > 6 weeks iii. Systemic signs and symptoms, musculoskeletal symptoms iv. Usually < 5 y/o; F = M < 5 y; F > M > 5 y v. High fever, rash, leukocytosis, myalgia, malaise, arthralgias, adenopathy, arthritis, hepatosplenomegaly, serositis, hepatitis, anemia, DIC vi. JIA Rash- salmon pink, associated with fever, splenomegaly and nodes, non palpable, circumscribed macular. b. Systemic onset disease Investigations

i. leukocyte count (30-50k, looks like leukemia), thrombocytosis ii. ESR- high iii. Anemia iv. IgM RF- neg, ANA- negative c. Systemic Onset Disease Course and Prognosis i. have recurrent episodes, 1/3 have progressive arthritis ii. Younger age of onset correlates with increased risk of poor health, both somatic and joints iii. Amyloidosis occurs in some children with persistent disease activity d. Systemic Onset Disease Management i. Splinting to prevent deformity ii. Physical and occupational therapy iii. NSAID- pain, fever and inflammation iv. Corticosteroids in severe cases v. DMARDS- ? benefit e. Pauciarticular Onset (Oligoarticular) ( 50%+) i. < 5 joints involved ii. < 6 y onset, F>M, most common type iii. Several subtypes 1. Subtype I, younger group a. Clinical features i. Early growth abnormalities ii. Risk (1/3) of Chronic uveitis w/in 5 yrs (asymptomatic) b. Investigations i. ESR, Hb/Hct, WBC, platelets are all normal ii. ANA- freq + (40-75%) c. Course and Prognosis i. Exacerbations and remissions ii. Alter in growth of affected limb (premature epiphesyal plate closure) iii. Course dependent on joints and eye involvement d. Management i. Splinting, NSAIDs, PT and OT ii. Intra-articular steroid injections, freq opth assessment 2. Subtype II a. Older (Juvenile Spondyloarthropathy) b. General Characteristics i. >9 y, M>F c. Clinical Features i. peripheral arthritis, Primarily lower extremities ii. Enthesopathies iii. Acute iritis

f.

iv. Sacroiliac pain in some, axial disease in some (either can be presenting feature) d. Investigations i. ESR- wnl to high, CBC- normal, RF- negative ii. HLA-B27 + e. Course and Prognosis i. Good in 2/3 cases ii. Some joint extension may occur iii. 1/3 may dev serious hip problems iv. Biologic agents have had big impact f. Management i. Anti- TNF agents (biologics) ii. PT, OT; corticosteroid injections, etc. 3. Subtype III (Psoriatic Arthritis) a. General Characteristics (3 of 4 minor criteria = Dx) i. Approx. 8 y. ii. Asymmetric in peripheral joints iii. May be destructive iv. May have associated PSA. v. Minor Characteristics (3 of 4 = Dx) 1. Dactylitis (swollen digits) 2. Nail pitting 3. PSA rash 4. family Hx of PSA vi. F>M b. Clinical Features i. Flexor tenosynovitis ii. Asymmetric c. Investigations (not very helpful, mostly clinical Dx) d. Course and Prognosis i. Young onset (+/-) assoc with iritis ii. Remitting and relapsing, even into adulthood iii. Occasionally severely destructive, spondylitis dev. e. Management i. Biologicsii. NSAIDs, splinting iii. Immunosuppressive- Methotrexate (MTX) Polyarticular Onset JRA (RF negative) i. General characteristic 1. Any age, occas. < 1 y 2. F > M ii. Clinical features

1. Can affect any joint 2. Reduced neck and TMJ ROM 3. Flexor tenosynovitis 4. +/- low grade fever 5. Mild lymphadenopathy and hepatosplenomegaly iii. Investigations 1. Anemia, mild leukocytosis, thrombocytosis, RF negative, ANA occas. positive. 2. Look like adult RA, but are RF (-) iv. Course and Prognosis 1. Variable 2. May be monocyclic; recurrent episodes tend to cause progressive deformities v. Management 1. DMARDs 2. Anti-TNF agents g. Polyarticular Onset- RF (+) i. General Characteristics 1. > 8 y old onset 2. F > M ii. Clinical Features 1. Polyarthritis of any joint 2. Rheumatoid nodules 3. Vasculitis- uncommon and late iii. Course and Prog 1. Persistent activity with serious joint destruction and poor functional outcome 2. Additional long term hazards include C1-C2 subluxation, aortic insufficiency and amyloidosis iv. Management 1. DMARDs (MTX) 2. Biologics 3. Surgical 7. Treatment of JRA a. NSAIDs b. Gold c. Sulfasalazine d. Anti-TNF agents (Etanercept) e. Dd

L39 Vascular Disease 1. Peripheral Vascular/Artery Disease (PVD/PAD) a. Most common- atherosclerosis b. Worsens with age, mostly affects the lower extremities c. Often associated with coronary artery disease (CAD), and vise versa d. Symptoms i. Claudication- aching, cramping, fatigue, weakness in the calves, thighs, buttocks brought on by walking and completely relieved after a few minutes of rest ii. Symptoms may occur at rest as disease progresses iii. Ischemic foot ulcers may develop e. Physical Exam** i. Smooth, shiny, hairless skin (lower extremities)* ii. Muscle atrophy iii. Diminished or delayed distal pulses* iv. Audible bruits over the involved arteries v. Severe ischemia > may lead to gangrene f. Dx i. Made by signs and symptoms ii. Ultrasound iii. Ankle brachial index**- in normal persons, systolic BP is legs is slightly higher then in arm (ankle: brachial >1). In PVD, the index decreases <.9 = PVD* iv. Angiography- helps define vascular anatomy for surgery prep v. MRA (Magnetic resonance angiography) vi. CT angiography g. Tx i. 3 phases: conservative, medical, surgical 1. Conservative a. Lifestyle changes i. Regular walking* may increase distance prior to claudication by 100-400%! ii. Smoking cessation iii. Switch to another from of Rx if resting ischemia or ulcers develop 2. Medical a. Cilostazol* b. Pentoxifylline*- leads to vasodilation, decreased platelet aggregation, and increases walking distance 28-100% c. Lipid lowering agetns- Statins d. Hypertensive agents. Goal BP <140/90 e. Aspirin f. Clopidogrel (Plavix)*

3. Surgical Treatment a. Revascularization (percutaneous or surgical) is indicated for i. Severe claudication resistant to other medical therapy* ii. Limb threatening ischemia* iii. Vasculogenic impotence 2. Acute Limb Ischemia- Vascular Emergency! a. Caused by thrombosis or embolism b. Arterial emboli usually originate in the cardiac chambers c. Symptoms i. Sudden onset with no prior claudication, pain!* ii. Thrombosis- history of claudication with suddenly assumes a crescendo patter over a few days d. PE i. Cold, cyanotic extremity ii. Absent pulses distal to the occlusion* iii. Diminished motor and/or sensory function e. Dx i. Hand held Doppler- can assess flows at different arterial segments ii. TEE to determine potential cardiac source f. Tx i. Anticoagulation! IV Heparin* ii. Embolectomy iii. Catheter-directed infusion of plasminogen activator (tPA), a fibrinolytic iv. Emergent amputation if tissue necrosis 3. Abdominal Aortic Aneurysm (AAA) a. A bulging area of the aorta b. Normal abdominal aorta = 2 cm, Dx AAA = 3 cm, 5 cm = rupture, a life threatening condition c. Common in older adults d. Risk factors i. Age, smoking, HTN, Family Hx e. Causes i. Atherosclerosis, cystic medial necrosis (Marfans, Ehlers-Danlos), vasculitis w/ CT disease, chronic infection f. Gradually grow in size g. Symptoms/Signs i. Nontender pulsatile mass above umbilicus if >5 cm ii. Hypotension, acute abdominal/back pain should prompt consideration of prompt consideration of aneurysm rupture***** iii. Usually asymptomatic until just before rupture iv. Tearing, ripping describes pain h. Dx

i. CT, MRA** preferred ii. Duplex ultrasonography i. Tx i. Smoking cessation, cloase blood pressure control, cholesterol reduction ii. Surgical repair if > 5 cm, or endovascular graft 4. Aortic Dissection a. General Features i. Intimal layer is torn away, creating false lumen parallel to true lumen ii. Typically occurs in the thoracic aorta b. Risk Factors i. **HTN (most important), cocaine use, trauma, CT disease (Marfans), vasculitis c. Classification Systems i. Stanford- A and B 1. A involves ascending aorta 2. B involves descending aorta ii. Debakey System 1. Type I- involves entire aorta 2. II- only ascending 3. III- only descending iii. Type A symptoms 1. Severe chest or back pain** 2. Abdominal pain- common* 3. Syncope* (passing out) 4. Stroke*- common 5. Retrograde propagation of the dissection can cause pericardial tamponade or coronary artery dissectin and acute MI 6. Aortic valve involvement 7. May propagate anteriorly and affect carotid and subclavian arteries leading to stroke or upper limb ischemia iv. Type B symptoms 1. Acute chest or back pain** 2. Lower extremity ischemia or ischemic neuropathy* 3. Pulse deficits* 4. Neurologic deficits 5. Narrow pulse pressure 6. Hypotension, jugular venous distension (JVD) d. Dx i. CT angiography* ii. TEE iii. MRA e. Tx i. Type A- fatal without emergent repair*

ii. Type B- medical therapy* 1. Surgery indicated if compromised blood flow to the legs, kidneys, or other viscera iii. Tight BP control to avoid aneurysms and further growth of dissections 5. Raynauds Phenomenon a. General Features i. A vasospastic disease of the small arteries, mainly affecting the toes and fingers* ii. Episodic bilateral digital palor, cyanosis and rubor* iii. Precipitated by cold or emotional stress* iv. Relieved by warmth v. If idiopathyic, Primary Raynauds, if associated with other disease, called secondary vi. Affects women > men vii. Recurrent episodes of digital ischemia, with characteristic white-blue-red color** b. Symptoms i. Pallor, cyanosis ii. Erythema (reactive hyperemia) iii. PE can be completely normal between attacks iv. May have digital ulcers or thickening of fat pads (sclerodactyly) c. Tx i. Avoid cold temps ii. Protect hands iii. Aspirin iv. Ca channel blockers to reduce frequency and severity 6. Buergers Disease (Thromboangitis Obliterans) a. General Findings i. Non-atherosclerotic disease of the small-medium-sized arteries, veins and nerves of the arms and legs. Affects mostly young men before age 45 ii. Inflammatory process* of the vessels = vasculitis iii. These patients have a hyper coaguable condition b. Cause i. Unknown, but all patients have Hx of heavy tobacco addiction** ii. Progress of disease linked to continued tobacco use c. Symptoms i. Claudication of hands, feet, legs, arms** (mainly hands and feet**) ii. Skin changes (ischemia, ulcerations) found mainly in the extremities d. Dx i. Clinical presentation and biopsy* ii. Histological feature- inflammatory intramural thrombi within the arteries and veins with sparing of internal elastic lamina and other arterial wall structures

iii. + Allens test- occluding both ulnar and radial arteries, when released from either artery the blanching will persist in affected arteries e. Tx i. Complete tobacco abstinence ii. Iloprost- Prostacyclin analog reduces limb ischemia and improves healing iii. Surgical amputation required in >40% of those who do not quit smoking 7. Giant Cell Arteries a. Pathophysiology i. Inflammation of the lining of the med-large arteries* ii. Large vessel vasculitis* iii. Immune-mediated* iv. Elderly* (>50 yrs) v. Female>males b. Sx i. Headache from temporal arteries* ii. Jaw claudication from ischemia of masseters* iii. Visual loss due to ophthalmic artery dz* iv. Chest pain may suggest aortic aneurysm or dissecting aneurysm c. PE i. Scalp tenderness in the temporal artery area!** ii. Low grade fever iii. Pale, edematous fundus* iv. Diastolic murmur of aortic regurgitation d. Studies i. Elevated C-reactive protein and Sed. Rate (>50-70)* ii. Anemia iii. Dx confirmed by biopsy of arterial tissue* e. Tx i. High dose corticosteroids** ii. Start Tx when highly suspicious (before biopsy results)* iii. Methylprednisilone* 8. Takayasus Arteries a. General Features i. Granulomatous vasculitis of the aorta, its main branches and the pulmonary artery* ii. Common in young women of Asian descent iii. Inflammatory process in the vascular wall can lead to stenosis and/or aneurysm formation* iv. HTN is the most common sign** b. Physical Findings i. Bruits over subclavian a. and aorta ii. Diminished brachial pulses

iii. Low brachial a. BP c. Dx i. Made primarily on clinical presentation d. Tx i. Corticosteroids are 1st line** ii. Immunosuppressive agent: Methotrexate* and cyclophosphamide help prevent relapse and disease progression iii. Revascularization (surgical or percutaneous) 9. Arteriovenous Fistulas a. General Features i. Abnormal vascular communications which shunt blood flow from the arterial system directly to the venous system ii. May be congenital or acquired (trauma) b. Sx i. Pulsatile mass* ii. Sx related to compression of an adjacent organ iii. Bleeding from spontaneous rupture* iv. Neurologic deficits or seizures* (if AVM is in brain) c. Dx i. MRA, CT-A, angiography 10. Venous Thromboembolic Disease* a. General Features i. Presence of coagulated blood or thrombus in a vein ii. Includes DVT and PE b. Predisposing Factors i. Virchows Triad ** (only need 1 to develop) 1. Endothelial damage (common w/surgery or trauma) 2. Venous stasis (bed rest or long travel, or cast) 3. hyper coagulation (cancer causes it, birth control pills) c. Trousseaus Syndrome: i. General Features 1. Migratory thrombophlebitis with non-infectious vegetations on the heart valves typically in the setting of mucin-secreting adenocarcinoma d. Hypercoagulable states i. Include hereditary diseases such as deficiencies in antithrombin II, protein C, or protein S* e. Etiology i. Surgery (esp. leg or hip)* ii. Immobility, long travel iii. Birth control pill* iv. Atrial fibrillation* v. Cancer*

f.

vi. Heart failure* vii. Pregnancy* viii. Venous catheters* Deep Vein Thrombosis (DVT) i. General Features 1. Most begin in calf 2. If propagates to proximal calf, then risk of PE increases ii. Sx 1. Pain and or swelling!!** 2. Many are asymptomatic 3. UE DVT can lead to SVC syndrome of facial swelling, blurred vision and dyspnea 4. Thoracic outlet obstruction a. Can compress brachial plexus leading to unilateral arm pain associated with hand weakness iii. PE 1. Tenderness, erythema, warmth, and swelling below site of thrombosis 2. Pain with dorsiflexion of the foot (Homans sign) may be present 3. Palpable tender cord 4. Dilated superficial veins 5. Low grade fever 6. Thoracic Outlet Syndrome (TOS) 7. Adsons test, Wrights test iv. Dx 1. D-Dimer (fibrin degradation product)highly sensitive indicator, ut are commonly falsely elevated 2. Duplex ultrasonography 3. MR angiography can help detect subclavian vein DVT 4. Contrast venography no longer the gold standard v. Tx 1. 1st- Heparin (IV), Lovenox (SQ) a. Prevent thrombus propagation and to maintain patency of venous collaterals nd 2. 2 - Warfarin (Coumadin)- start after/with above medications vi. Upper Extremity DVT 1. Removal by fibrinolytic infusion thru a catheter inserted directly into affected vein 2. Mechanical fragmentation of the thrombus vii. IVC filter*- consider in patients with proximal DVT who: 1. Either have an absolute contraindication to anticoagulation or 2. Develop recurrent PE despite adequate anticoagulation

3. These filters reduce the incidence of PE, but increase the risk of recurrent DVT g. Pulmonary Embolism i. General Features 1. Fat embolis- after long bone fracture 2. Very common, severely underdiagnosed 3. Predisposing factors same as for DVT 4. In acute PE, lung areas are ventilated, but under-perfused ii. Sx*** 1. Sudden onset dyspnea 2. Pleuritic chest pain 3. Angina chest pain 4. Hemoptysis 5. Syncope iii. Physical Findings 1. Tachypnea** 2. Tachycardia** 3. Crackles, wheezing, pleural rub iv. Dx 1. Spiral chest CTA = quick, effective. Modality of choice** 2. Spiral CT of chest** 3. CXR not very helpful 4. D-dimer* 5. Hemoptysis 6. Arterial blood gas (ABG) 7. VQ study = WAS gold standard, too nonspecific 8. Pulmonary angiography- only use if above studies are inconclusive v. Tx 1. Anticoagulation- Immediate! IV UFH (unfractioned Heparin)** 2. Warfarin (Coumadin)*- After starting UFH 3. Thrombolytics*- reserve for patients with hypotension and/or severe hypoxemia 4. How long to continue anticoagulation? a. 3-6 mos after a PE due to trauma or surgery b. Indefinitely for VTE due to cancer or for idiopathic VTE c. Stop anticoagulation if unmanageable bleeding h. VTE Prophylaxis i. For high risk patients 1. Involves: Heparin, LMWH, and pneumatic stockings, TED hose, early ambulation ii. Surgical patients

1. Hospitalized with CHF, acute illness, respiratory illness, acute inflammatory disease and those expected to be immobilized for 3 days or longer iii. Imprecise science

L40 Aortic Valvular Disease 1. Definitions a. AI- insufficient = regurgitation b. AS- aortic stenosis 2. Aortic Stenosis a. Etiology i. Rheumatic Fever ii. Calcific/degenerative iii. Congenital = bicuspid most common (normally aoritic valve has 3 leaflets) iv. Post-endocarditis b. Pathophysiology i. Decades before uni or bi congenital valve develops calcification ii. Calcification due to wear and tear, esp. in hypertensives iii. High outflow resistance causing concentric LVH iv. High LV filling pressure (the pressure needed to fill the ventricle- a stiff muscle requires higher pressure to fill, b/c need to stretch the muscle, which is stiff) v. LV filling pressure reflected to atrium vi. Systolic function preserved until late vii. Hypertrophied ventricle becomes less compliant, stiff viii. Atrial contraction provides proportionally more LV filling volume as opposed to passive filling ix. As gradient increases, peak ejection delayed later in systole (gradient = pressure drop over an obstruction, so pressure difference between ventricle side on a stenotic aortic valve and the pressure in the aorta) x. Pulse volume decreases xi. Ventricular hypertrophy and high filling pressures lead to subendocardial ischemia c. Clinical Presentation i. Triad of Symptoms- occur sequentially (know these*) 1. Chest pain 2. Exertional syncope or near syncope 3. Dyspnea/orthopnea- CHF ii. Delayed and weakened carotid upstroke (pulsus tardis et parvis) iii. Late peaking crescendo/decrescendo murmur at base with radiation to carotids

iv. Soft or absent S2 (aortic valve not opening all the way, so doesnt make sound because cant slam shut)- one of the most prominent markers of AS v. Hyperdynamic apical impulse- indicates how hard the heart is working vi. S4 at apex vii. Murmur may decrease with standing viii. Significant systolic HTN- rare (over 180) ix. Paradoxic S2 split (normally, S2 splits with inspiration- with AS, during expiration S2 splits, causing a double sound) x. Palpable thrill over base common in severe AS (a rumbling sense over murmuralways pathologic) xi. Rare association with colonic angiodysplasia and lower GI bleed d. Evaluation i. CXR: slightly enlarged LV, but overall CXR not very helpful ii. 2D echo- best test to do. Structure and mobility of valve leaflets, thickness and function of ventricular walls iii. Doppler: quantify gradient and calculate valve area (most diagnostic) iv. Cardiac catheterization v. ECG: LVH e. Natural History i. Very predictable course if not corrected 1. From onset of angina, mortality in 5 yrs 2. From onset of syncope, mortality in 3 yrs 3. From onset of CHF, mortality in 18 mos ii. Congenital bicuspid valve may be asymptomatic for decades f. Therapy i. No medical therapy to significantly alter prognosis or symptoms ii. Surgery 1. Most appropriate intervention 2. Can successfully be done even in severely impaired ventricles, with improvement in symptoms and mortality 3. Critival valve area for intervention is .75 cm ^2 4. Surgery should not be delayed until CHF, but must be planned after gradient established iii. Balloon Valvuloplasty 1. Initial success rate excellent 2. Restenosis rapid and profound, most within 6 mos (so only a temporary solution, to get immediate results) 3. Present indivation only for bridge iv. Transcutaneous Valvular Surgery 1. Placement of and expandable trileaflet valve within a stent into the stenotic aortic orifice 2. Technically difficult

3. Significant residual stenosis due to device size 3. Aortic Regurgitation (AI) a. Etiology i. Endocarditis ii. Rheumatic Fever iii. Calcific degeneration iv. Trauma v. Aortic Root Disease (aortic root balloons out, expands- pulling valves apart, cant touch to close, so they leak), caused by: 1. Cystic medial necrosis 2. Marfans Syndrome 3. Annulo-ectasia 4. Aortic aneurysm/dissection 5. Syphilitic aortitis 6. Seronegative arthropathies b. Pathophysiology i. High pressure leak increases LV filling volume, raises LVEDP (filling pressure), and causes dilation. The increased volume is the key difference from AS* ii. High pressure leak also causes LV hypertrophy iii. Cor bovinum- large, thick-walled heavy hearts iv. Wall tolerated for years until myocardial compensation outstrips vascular supply v. Systolic function preserved vi. High stroke volume causes most peripheral physical findings of AI vii. SVR normal to low viii. Wide pulse pressure (>60 mm Hg) common c. Clinical Presentation i. Bounding central pulses (Corrigans or water hammer) ii. Diastolic crescendo murmur at 2nd interspace right, radiating to apex, heard best in end expiration, leaning forward iii. Laterally displaced PMI, with hyperdynamia and enlargement iv. Anterior heave v. Wide pulse pressure vi. Quinckes pulses (nailbed capillary pulsations) vii. Duroziez sign- to and from murmur across femoral artery viii. Severity correlates with duration of murmur d. Evaluation i. History- focus on functional capacity ii. Careful PE iii. 2D echo: LV chamber dimensions, wall thickness, and motion iv. Doppler v. L. heart catheterization vi. CXR: cardiomegaly prominent

vii. ECG: LVH e. Natural History i. Tolerated well for years until dilation exceeds metabolic accommodation ii. Symptoms appear after development of irreversible LV dysfunction iii. Acute AR poorly tolerated, and leads readily to PE and death if not corrected f. Therapy i. Afterload reduction ii. Inotropes, late iii. Preload reduction only in presence of PE iv. Strenuous exercise limited v. Maintain sinus rhythm vi. Antibiotic prophylaxis vii. Surgery 1. Indicated at onset of symptoms, of if functional capacity fails 2. LV end systolic dimension 5 cm or diastolic dimensions 7 cm indicates need for surgery 3. If root aneurysmal, need combined valve/root replacement L41 Mitral Valve Disease 1. Mitral Valve Stenosis a. Etiology i. RF ii. Degenerative calcific disease iii. CT disorders (RA, SLE) iv. Congenital b. Pathophysiology i. Rheumatic fusion at tips: calcific fusion from cusps ii. Normal orifice 4-6 cm sq., critical stenosis <1 cm sq. iii. Gradient develops, raising atrial pressure iv. Pulmonary HTN- longstanding v. Atrial dilation leads to atrial fibrillation vi. LV pressure normal vii. Normal LV contraction viii. Tachycardia decreases LV filling volume and cardiac output ix. Rising pulmonary artery pressure can impair RV function. c. Clinical Presentation i. Exertional dyspnea/orthopnea ii. Hemoptysis with pulmonary HTN iii. Palpitations iv. Thromboembolism v. Endocarditis

vi. Diastolic low pitched apical murmur vii. Opening snap after S2, before murmur viii. S1 loud (especially with rheumatic) ix. RV heave x. Normal PMI and function d. Evaluation i. CXR: large RV; pulmonary vascular engorgement. No LV dilation. Large left atrium ii. Thickened, fused leaflets on echo iii. ECG- atrial abnormality, RVH iv. Doppler: gradient and valve area v. R and L heart catherterization e. Therapy i. Diuresis for acute PE ii. Maintain sinus rhythm: anti-arrhythmics where indicated iii. Prevent tachycardia: beta blockers iv. Digoxin useful for control of rate in atrial fibrillation v. Oral anticoagulants vi. Antibiotic prophylaxis vii. Balloon valvuloplasty viii. Open commissurotomy ix. Valvular replacement x. Surgery timed to prevent irreversible pulmonary HTN f. Natural History i. 5 yrs to progress from mild to severe disability ii. 80% 5 yr survival rate after surgery iii. Valvuloplasty restenosis rate 80% in 10 yrs 2. Mitral Regurgitation a. Etiology i. Leaflet destruction 1. Infection (SBE) 2. Calcification 3. CT disorder 4. Trauma ii. Myxomatous degen (MVP, Marfans) iii. Papillary muscle dysfxn 1. Ischemia/infarction 2. Myxomatous degen 3. Spontaneous rupture iv. Dilated cardiomyopathy v. Hypertrophic cardiomyopathy b. Pathophysiology

Leak unloads LV into low pressure atrium Leak worsened by increased systemic afterload Left atrial pressure and pulmonary wedge pressure rise Atrium dilates LV dilates to accommodate increased filling volume Contractility eventually decreases Pulmonary HTN and Edema ensue Acute MR is tolerated very poorly compared to chronic due to insufficient time for atrial and ventricular adaptation c. Clinical Presentation i. Symptoms depend on severity of leak ii. Exertional dyspnea/orthopnea are late findings iii. Palpitations with arrhythmia iv. Atrial fibrillation: tolerated better than in MS v. R heart failure at end-stage vi. Fatigue, weakness due to decreased cardiac output vii. Laterally displaced PMI with enlargement viii. S3 common ix. Holosytolic murmur at apex x. Murmur radiates to back, left axilla most commonly, but may radiate to base xi. Pulse upstrokes full d. Evaluation i. CXR: LV dilation, LA dilation, pulmonary vascular engorgement ii. Echo: LV and LA enlargement. Wall motion normal until late iii. Doppler ID and quantify the regurgitant jet iv. ECG: LAA in >50%, RVH in 15%, atrial fibrillation common v. Ejection fraction normal early, reduced late vi. Cardiac catheterization e. Therapy i. Preload reduction for acute heart failure ii. Afterload reduction to improve forward flow iii. Inotrope to maintain contractility iv. Antiarrythmics to control ectopy and maintain sinus v. Antibiotic prophylaxis vi. Surgery 1. Annuloplasty 2. Valvuloplasty (not ripping it up, but repairing what is broken) 3. Valve replacement 4. Coronary revascularization 5. Surgery timed to prevent irreversible LV dysfxn f. Natural History i. Small leaks tolerate for normal life span

i. ii. iii. iv. v. vi. vii. viii.

ii. Indication for need of surgical intervention is when end diastolic LV dimension of 7 cm, or end systolic dimension of 5 cm iii. LVEF < 40% indicates severe, if not inoperable LV dysfxn 3. Mitral Valve Prolapse (MVP) a. Etiology i. Myxomatous degenerative valve ii. Genetically mediated iii. Associated with other dyscollagenoses (Marfans, OI) iv. Females 4x vs males b. Pathophysiology i. Leaflets redundant and bulge into atrium during systole ii. Occasionally regurgitant iii. Can continue to degenerate and cause severe MR iv. High circulating catecholamines, thus symptoms are catecholinergic c. Clinical Presentation i. Chest pain ii. Palpitations iii. Exertional dyspnea iv. Occasional syncope v. Usually in 2nd -3rd decade vi. Early to mid systolic click at apex vii. Mid to late systolic murmur if MR present viii. No other findings ix. Click/murmur prolong and increase with maneuvers which decrease LV filling volume (standing, valsava, nitrates) d. Evaluation i. Echo for proplapse ii. Doppler for MR iii. Holter for arrhythmia e. Natural History i. Generally benign ii. Can progress to symptomatic MR in older age iii. Men progress more often and more rapidly than women iv. Small increased risk for SBE, embolus and arrhythmia f. Therapy i. Reassurance ii. Beta blocker may alleviate some symptoms iii. Ascultory follow up for progression iv. Antibiotic prophylaxis if murmur present