Rheumatology

Emma Firth

AIM
To be confident dealing with common rheumatological conditions which you may come across as a junior doctor within multiple settings

Objectives
Refresh bony anatomy of hand Competent joint pain history taking To be confident in the aetiology, features, investigations and management of the know about rheumatological conditions in the core curriculum To get you excited about rheumatology?!!!

Know about:
Osteoarthritis Rheumatoid arthritis Septic arthritis* Gout / Pseudogout Ankylosing Spondylitis Reactive arthritis Polymyalgia rheumatic Temporal arteritis* Vasculitis

Know of:
Seronegative arthritis Fibromyalgia Connective tissue diseases e.g. SLE, scleroderma Not scope to cover the last two today

GROSS ANATOMY

carpometacarpal

HISTORY

Joint pain history

HPC SOCRATES Joint Stiffness, swelling, red/hot, tender, locking, deformed, Instability Neuro weakness, numbness, paraesthesia Systemic weight loss, fever, malaise Extra-articular: Skin rashes, Eye pain, raynaud's syndrome, GI or respiratory

PMH Previous episodes Trauma Psoriasis or IBD Diabetes DH Iatrogenic

FH Seronegative spondarthritis (arthritis, psoriasis, ankylosing spondylitis or IBD) Autoimmune

SH Occupation
Risks for joint problems Affect of joint problem on work

ADL / Hobbies / sleep / mood Recent travel Marital/sexual status

Systems

CONDITIONS

1. Osteoarthritis

Primary causes
Age Obesity ( pressure) Occupation Golfers 1st MTP joint Footballers knees

Secondary causes
Bone: Pagets, acromegaly Arthritis: RA, gout, chondrocalcinosis Metabolic: hemochromatosis, Wilson's

History / Pattern
DIP + PIP joints of hand Asymmetric large joint involvement Deep ache Triggered by activity, relieved by rest Decreased movement / function Locking / catching +/- myelopathy / spinal stenosis

On examination
Name three clinical signs of osteoarthritis visible on inspection

Look : Osteophytes Heberdens nodes: DIP Bouchards nodes: PIP Squaring (carpo-metacarpal joint) Hip: Fixed flexion Wasting

Feel: Effusions Move: Crepitus Internal rotation of hip Pain on passive movement of hip at end of range

Investigations
Blood tests: -ve Rh factor, ANAs, ESR normal Aspirate: cartilage fragments, viscosity X-ray Name five typical changes that can be seen on xray.

Treatment
Non pharmacological Physiotherapy OT: Assistive devices e.g. sticks, tap turners Low impact aerobic exercise Weight loss Heat / cold packs / TENS for pain relief. Pharmacological 1st : Paracetamol cocodamol 1st : Topical NSAIDs 2nd : Oral NSAIDs, Opioids 2nd : Topical capsaicin 2nd : Intra-articular corticosteroid injections Surgical Athroscopic lavage and debridement (only IF locking) Replacement arthroplasty

Complications:
Cervical / lumbar OA mild myelopathy or radiculopathy Spinal stenosis lower back or leg pain worse back extension Deformity / tendinitis + contractures

2. Rheumatoid arthritis

Pathology
Synovial tissue proliferation = Pannus characteristic pathological lesion in RA. Cells in pannus produce collegenases and proteases cartilage destruction

Thickened, inflamed granulation tissue within the joint

Subluxation, mechanical disarray and instability of joints

History / Pattern
What is the typical course of rheumatoid arthritis?

Symmetrical, peripheral polyarthritis PIP joints, MCP, and small foot joints. NOT DIPs Early morning stiffness >30 mins, eased by exercise

On examination
Name four DEFORMITIES OF THE FINGERS / THUMB

Look Subcutaneous nodules Ulnar deviation at MCP joint (i.e. fingers point towards ulnar side) MCP joint swelling

 Feel Hot, swollen (boggy synovial hypertrophy) Tender

Move ROM Tenosynovitis thickening, tenderness & crepitations

Name six extra articular features of rheumatoid arthritis

Investigations
Rheumatoid factor Anti- CCP (cyclic citrullinated peptide) antibodies ESR and CRP FBC: Anaemia, thrombocytosis ( platelets) U+E, LFTs (pre treatment) Joint aspirate: Cloudy, can also check for sepsis

 Loss of joint space Articular erosions

Periarticular osteopenia Soft tissue swelling

Treatment
Non pharmacological Pharmacological Pain: NSAIDs/COX-2 inhibitor Glucortioids: e.g. IM methylprednisolone Disease modifying 1st line: Methotrexate + other +/ST glucocorticoids 2nd line: DMARD monotherapy with fast of dose 3rd line: Anti-TNF- agents e.g. infliximab 4th line: Rituximab (monoclonal antibody against B cells) + methotrexate

Methotrexate
Mechanism Inhibits folic acid reductase Suppresses immune system / inflammation Indications RA; Psoriatic arthritis (licenced) Connective tissue disease: SLE, vasculitides, myositis (unlicensed)

Contraindications x Alcohol excess x Pregnancy (teratogenic!) x Suspected infection x Marrow failure

What are the side effects of methotrexate

Prescribing It is your first day as an FY1 and a nurse shows you documentation that the consultant on ward round has said Plan: (1) Start methotrexate What else do you know about prescribing methotrexate?

3.

CASE STUDY
A 53 year old woman presents to A&E with a swollen left painful knee. PMH: RA, being treated with methotrexate and hydroxychloroquine. 2-3 day hx of generally unwell, fever and chills O/E: warm, swollen, decreased ROM, tachycardia, T 37.9

3. Septic arthritis = orthopaedic emergency

History
Evolves over hrs days Limp / referred pain Exquisite pain Unwell: fever, rigors +/- vomiting

O/E
Systemically septic Swollen, red, tender and warm joint +/- Effusion ROM, held still (pseudoparesis, pseudoparalysis)

Pathogenesis:
1) Haematogenous spread 2) Direct inoculation 3) Direct extension

Risk factors
Prosthetic joint Immunosuppression DM Skin infections / bacteraemia IVDU Trauma

What is the most appropriate next step?

1. 2. 3. 4. 5. 6.

Analgesia Oral antibiotics and discharge Aspiration of knee and injection with steroid Aspiration of knee and send fluid for MC&S Add adaluminab Inform a senior

Investigations
Bloods: raised WCC, CRP +ve Blood cultures USS (cant determine sterile effusion vs pus ) Urgent aspiration in all suspected cases PUS! BUGS! A high leucocyte count of >50,000/ml is highly suggestive even if no organisms are seen Most common organism?

Management
1) IV antibiotics as soon as joint fluid obtained. e.g. Flucloxacillin 2g IV qds & Gentamicin 5mg/kg IV od 2) Prompt surgical washout/drainage +/debridement 3) Joint should be splinted and rested

Complications
Septic shock Osteomyelitis Secondary osteoarthritis Avascular necrosis Growth disturbance in children

4. SERONEGATIVE ARTHROPATHIES
= A group of inflammatory disorders predominantly affecting the spine, sacroiliac joints and larger peripheral joints, characteristically asymmetrical. Rheumatoid factor typically ve. Ass with Human Leukocyte Antigen B27.

4a. Ankylosing spondylitis

Symptoms
Inflammatory back pain: pain and stiffness, lasting >1 hr; worse am/on rest Peripheral arthritis: larger joints, lower limbs usually, 1/3 pts Peripheral enthesitis: Achilles tendonitis, plantar fasciitis, and at tibial tuberosity, 1/3 pt Systemic: fatigue

Signs
spinal flexion (Schobers test*) and neck movements (occiput test*) Excessive thoracic kyphosis + loss of lumbar lordosis (question mark) Crepitations at lung apices LATER: para-spinal wasting, spine #

Complications the 5 As

Investigations
Bloods Raised ESR, Raised/n CRP RF +, anti CCP ve What are the classical signs seen on sacroiliac and lumbar spine xrays?

Treatment
1st: NSAIDs, physio 2nd line: different NSAID 3rd line: anti-TNF e.g. etanercept

Steroids to tx flares but not LT DMARDs dont work!

4b.

Hans Conrad Julius Reiter German physician convicted of war crimes for medical experiments at concentration camps Wrote a book on racial hygiene Reiter's syndrome was later renamed to Reactive arthritis when his Nazi affiliation came to light

Precipitating infections
GU: Chlamydia trachomatis (>2/3), Gonorrhoea GI: Salmonella, Shigella, Campylobacter and Yersinia Other: Parvovirus B19 (slapped cheek), post-streptococcal

Cant see, cant pee, cant bend my knee

1) Inflammatory arthritis 2) Conjunctivitis (or anterior uveitis) 3) Urethritis (or cervicitis)

Other than the classical triad, what other features may be present in reactive arthritis?

Investigations
Urethral discharge / stool MC&S Joint aspirate: MC&S ve, giant macrophages on histology RF and anti CCP ve ESR / CRP: /

Treatment
1st = NSAIDs GUM referral

Prognosis
Usually resolves in 6-8 weeks May become recurrent (50%) or lead to chronic symptoms (25%)

4c. Psoriatic arthritis

Features

1. 2. 3. 4. 5.

Polyarteritis indistinguishable from RA (MCPs, PIPs) Asymmetrical oligoarthritis affecting large + small joints Predominant sacroiliitis and spondylitis (mimics ank spond) DIP joint arthritis Arthritis mutilans (1%): pencil and cup appearance from telescoping

Investigations Blds: 5-10% RF +ve, Anti CCP ve Synovial fluid: no crystals X-ray: Erosions, Minimal juxta-articular osteopenia, Pencil-in-cup deformity

Treatment Physio Steroids for flares but not LT 1st line: DMARD methotrexate 2nd line: NSAIDs, other DMARDs e.g. sulfasalazine / Anti-TNF e.g. etanercept, Infliximab

4d. Enteropathic arthritis

Features
Hx of IBD Peripheral arthritis: Usually asymmetrical, lower limbs, larger joints, Often transient and migratory Sacroilitis/spondylitis Ocular disease: anterior uveitis, conjunctivitis, scleritis, episcleritis

Investigations
RF and anti CCP ve ESR and CRP / X-rays (MRI in the young): spine/sacroiliac joints (as for ank spond) Investigation for IBD: colonoscopy etc

Management
Control IBD DMARDs Anti-TNF if severe

5. CRYSTAL ARTHROPATHIES

5a. GOUT

URATE
Purines hypoxanthine * xanthine *urate
[2/3 excreted renally; 1/3 intestinally CO2 + NH3] *enzyme xanthine oxidase; uricase

Causes of high urate

High dietary purines Impaired renal excretion Hypoxic conditions/oxidative stress Lesh-Nyhan syndrome (rare)

Risk factors
Idiopathic in uric acid excretion CKD Thaizides Alcohol Beer, shellfish, bacon Dehydration

Features
Sudden onset *Acutely* painful Monoarthritis Able to weight bear Swollen, hot, red

Investigations
Blds: Urate, FBC, U+E, CRP, BC? USS / Xray Aspiration and fluid MC&S

What is seen on microscopy?

Management
Risk reduction: Dietary advice Diuretics r/v Pharmacological Acute: 1st NSAIDs, 2nd Colchicine Preventer: ALLOPURINOL

5b. PSEUDO-GOUT aka Calcium pyrophosphate dehydrate (CPPD) crystal deposition disease

Pathology
Calcium pyrophosphate deposition in cartilage

Features

Risk factors
Bone: OA, acromegaly Endo: Hyperparathyroidism, hypothyroidism, LT steroids Metabolic: Haemochromatosis

Usually affects knee / wrist / shoulder and ankle joints Similar presentation to gout acute joint pain and swelling

Investigations
Bloods: FBC (WCC), RF and anti-CCP ve, U+E, LFTs, cutlure Uric acid levels if suggests gout Xray: chondrocalcinosis, hook osteophytes on medial side of joints Aspiration: MC&S What do you see on aspiration?

Management Joint aspiration response rate to DMARDs but try: hydroxychloroquine, sulphazalazie Low dose colchicines prevention NSAIDs

6. VASCULITIS

Causes
Primary Large vessel: Polymyalgia rheumatica, Takayasu's, Temporal arteritis Medium vessel: Polyarteritis nodosa, Kawasakis, Buergers disease Small vessel: Behet's syndrome, ChurgStrauss syndrome, HSP, Wegener's granulomatosis Secondary RA, SLE, ank spond, dermatomyosits, malignancy e.g. lymphoma, infection e.g. hepatitis C, certain drugs

Features
Unexplained ischaemia: claudication, angina, TIA/CVA, mesenteric/cutaneous/limb ischaemia, MI, gangrene, HTN General symptoms: Fever, weight loss Skin: Palpable purpura Muscles and joints: Myalgia or myositis, arthralgia Nervous system: Mononeuritis multiplex, headache, stroke, tinnitus, reduced visual acuity, acute visual loss Respiratory tract: Epistaxis, haemoptysis GI tract: Abdominal pain, PR bleeding Kidneys: Glomerulonephritis

Vasculitis Affected organs Cutaneous small vessel Skin, kidneys vasculitis

Wegeners granulomatosis Churg-Strauss syndrome Kawasaki disease Nose, lungs, kidneys Lungs, kidneys, heart, skin Skin, heart, mouth, eyes

Histopathology Neutrophils, fibrinoid necrosis

Neutrophils, giant cells Histiocytes, eosinophils

Lymphocytes, endothelial necrosis Neutrophils, granulomas

Buergers disease

Leg arteries and veins (gangrene)

Investigations
Raised ESR, CRP, WCC, eosinophils Anaemia ANCA +ve: Wegener's granulomatosis, ChurgStrauss syndrome, microscopic polyangiitis Biopsy of affected organ Angiogram

Treatment
Dependent on type / progression Steroids Immunosuppressants

6a)

 A 60 year old woman presents non-specifically unwell with fever and weight loss. She feels pain and stiffness in her shoulders and hips. She has also noticed she has a temporal headache and she has noticed her scalp tender when brushing her hair. What is the most likely diagnosis?

6a) TEMPORAL ARTERITIS

Aetiology
Subacute granulomatous inflammation - Vasculitis

Epidemiology
Age >50 years usually 3F:1M 50% of pts with GCA also have polymyalgia rheumatica 15% of pts with polymyalgia rheumatic also have GCA

Symptoms
Unilateral throbbing/boring pain Constant Temporal artery tenderness: Pain when combing hair Jaw claudication B symptoms: Fever, Weight loss, night sweats, lethargy, depression, arthralgia Visual problems: amaurosis fugax, diplopia, sudden loss of vision.

Signs
Thickened, tender or nodular with pulsation temporal and/or facial artery Proximal myalgias

Investigations
Bloods: ESR (usually >30, but may be normal) +/- normocytic anaemia Temporal artery biopsy: giant cell arteritis Exclude other diagnoses e.g. malignancy, RA, myeloma. When would you biopsy? What if the biopsy was negative for giant cell arteritis?

Complications
Blindness due to involvement of ophthalmic artery (Anterior ischaemic optic neuropathy)

Management
No ocular symptoms: Prednisolone 40-60mg daily Tapering dose Monitor ESR Treatment should not need to be continued for more than a year Ocular symptoms / vision loss: 1mg/kg prednisolone (6080mg) or IV methylprednisolone Same day ophthalmology referral emergency!

6b) POLYMYALGIA RHEUMATICA

Features
Symptoms: Aching, and morning stiffness > 1 hr Pattern: Proximal, symmetrical Systemic: fatigue, fever, weight loss, anorexia and depression Onset: days 2 weeks

Diagnosis if >3 of the following: Bilateral shoulder pain or stiffness Morning stiffness lasting >1hr Bilateral tenderness in the upper arms Weight loss and/or depression Age 65 Onset of illness <2wk ago Initial ESR >40mm/h

Investigations
ESR and CRP FBC : anaemia Alkaline phosphatase, BG, creatinine, lipids, BMD, TB testing USS of hip and shoulder

Management
Physiotherapy Oral corticosteroids e.g. prednisolone 15-20mg daily Tx for initially ~3 years then trial slow withdrawal over 6 months

Plenary
Refreshed gross anatomy of hand Reviewed joint pain history taking Covered all the know about conditions in the curriculum, and the seronegative arthropathies in the know of conditions and seronegative arthritides Equipped to deal with common rheumatological conditions which you may come across as a junior doctor within A&E, GP, dermatology, colorectal/gastroenterology, GUM, ID including rheumatological emergencies Rheumatology is not just about joint pain!!

Question 1
A 24 year old male presents with knee pain, red watery eyes and dysuria. He has also noticed plaques on his hands. What is the most likely diagnosis?

What are the plaques?

Question 2
Which of the following is an xray feature in rheumatoid arthritis? a) b) c) d) e) Loosers zones Widened joint space Periarticular osteopenia Osteophytes Lytic lesions

Question 3
In a patient with rheumatoid arthritis, what is the most likely cause of a low haemoglobin with a normal MCV, normal WCC and platelet count? a) Iron deficiency b) Feltys syndrome c) Methotrexate d) Anaemia of chronic disease e) Hypothyroidism

Question 4
A 65 year old female presents with a painful right knee. An xray of the knee shows linear calcification of the articular cartilage and lateral meniscus. Given the diagnosis, what would you find on aspiration of the joint? a) Pus b) Positively birefringent crystals c) Negatively birefringent crystals d) No abnormality e) Raised leucocyte count

Question 5
A 62 year old man presents to his GP practice with joint pain affecting his knee and wrist and feeling generally unwell. On examination there is generalised lymphadenopathy and a rash on his shin
What diagnosis do you suspect? How would you confirm it?