Beruflich Dokumente
Kultur Dokumente
Case
A 52-year-old woman has a history of progressive ulnar deviation and PIP
enlargement. She also has lumbosacral spine and hip, knee, and shoulder
joint involvement. Two years ago, she required right knee
replacement. Eight years ago, she had nephrolithiasis with several recurrent
episodes. The kidney stones have not been analyzed. She notes that there
are dark stains on her undergarments. She has a long history of
rosacea. One week ago, she began treatment for rosacea with minocycline.
Radiographs of the lumbar sacral spine reveal loss of disc height, disc
calcification, very thick syndesmophytes, and “bamboo spine” (see figure
below).
Figure
Question
Which of the following is the most likely diagnosis?
A. Rheumatoid arthritis
B. Minocycline-induced hyperpigmentation
C. Alkaptonuria
E. Ankylosing Spondylitis
Correct Answer
C
Answer Rationale
Alkaptonuria is an autosomal recessive inborn error of metabolism. The
enzyme homogentisic acid oxidase is deficient resulting in large volume
excretion of
homogentisic acid (HGA) in the urine and sweat. Urine that contains HGA
will turn dark as the acid is oxidized to melanin-like product. This may result
in dark stains on clothing and undergarments. HGA may also accumulate on
cartilage, tendons, sclerae, skin, and intima of large vessels. Nephrolithiasis
is a manifestation of the disease. Alkaptonuria resembles ankylosing
spondylitis involving the spine and large joints, however, it differs in that the
sacroiliac joints (SI joints) are spared. Minocycline has been associated with
hyperpigmentation resembling alkaptonuria, however, urine has normal HGA
measurement.
Question Number: 39
Case
A 56-year-old man has a maculopapular rash over the lower extremities that
comes and goes. He notes some edema when he has the rash. He has
fatigue and some joint pain but no paresthesias. He has been losing weight.
Review of other systems is unremarkable. He takes no medications.
Examination shows a normotensive man with palpable purpura and no
abnormal neurological or musculoskeletal features. Findings on the
remainder of the general examination are normal.
ESR: 98 mm/hr
ANA: 1:40 speckled
dsDNA: negative
ANCA: negative
RF: 450
Anti-CCP antibody: negative
Total protein: 8.8 gm/dl
Urinalysis: 1+ protein, 2+ blood, no casts
Question
Based on the information above, which of the following is the most likely
cause of the patient’s elevated rheumatoid factor?
A. Rheumatoid arthritis
B. Sjögren’s syndrome
C. Lupus
D. Hepatitis-associated cryoglobulinemia
E. Wegener’s granulomatosis
Correct Answer
D
Answer Rationale
The patient is a middle-aged man with palpable purpura, which is highly
suggestive of vasculitis. There is little in the history to suggest infection as a
trigger, and there are no symptoms referable to the upper respiratory tract,
which are present in about 80% of patients with Wegener’s
granulomatosis. He is not taking any medications that might trigger a
hypersensitivity vasculitis. The examination is unrevealing other than
showing purpura, and the laboratory studies show renal insufficiency, an
active urinary sediment, hepatitis, a low titer ANA with negative anti-dsDNA
antibodies, and markedly elevated rheumatoid factor. The anti-CCP antibody
is negative, arguing against RA, especially in light of the absence of joint
inflammation.
Rheumatoid arthritis is not likely, given the absence of joint findings and
negative anti-CCP antibody. Sjögren’s syndrome is in the differential
diagnosis, but there is little in the clinical history to suggest its presence.
Results of SSA and SSB antibodies are not given. Wegener’s granulomatosis
is not likely given the absence of symptoms referable to the upper
respiratory tract and the negative ANCA, which are seen in the majority of
patients with Wegener’s granulomatosis. Lupus is also a possibility but the
patient does not appear to fulfill sufficient criteria to support the diagnosis.
Question Number: 40
Case
A 30-year-old man has gradually increasing right knee pain for the past 21
days. He is a long-distance runner who averages 30 miles a week. He says
the pain began after a “speed work out.” The pain is on the lateral aspect of
the knee. It resolves with rest to some degree, but any running causes it to
return and causes him to limp for a day or 2. He can also bring on pain by
standing on his right leg and flexing his knee. The knee does not lock.
Physical examination shows tenderness over the lateral knee and lateral tibial
condyle. There is a creaking over the tender area with knee flexion and
extension. The knee is stable and there is no effusion.
Question
Which of the following is the best course of action at this time?
A. Prescribe NSAID and avoid running
D. Apply an elastic bandage (ACE wrap) and have the patient use crutches for 4-6 weeks
Correct Answer
A
Answer Rationale
Iliotibial band syndrome is a common injury in long-distance runners that
usually develops with over-training and after vigorous running or hiking. The
treatment involves avoiding running and NSAID therapy. In severe cases,
physical therapy and local corticosteroid injection may be helpful.
There is no role for intra-articular injection or avoiding weight bearing in this
condition.
Surgery is not indicated for this condition unless it is chronic and refractory
to all other therapies.
Question Number: 41
Case
A 6-year-old girl with polyarticular juvenile idiopathic arthritis has a negative
rheumatoid factor and positive ANA.
Question
How often should she undergo slit-lamp examination to screen for chronic
iritis?
A. Every three to four months for four years then every six months
E. When she is moved to the front of the class because of difficulty seeing the blackboard
Correct Answer
A
Answer Rationale
Iritis in children is usually chronic and asymptomatic until extensive scarring
has occurred. Those at highest risk of developing iritis are children with
young onset oligoarticular disease and who are ANA positive. However,
polyarticular ANA positive disease also carries a significant risk of iritis,
although lower than the oligaorticular group, and should be screened at the
same intervals.
Question Number: 42
Case
A 38-year-old woman is undergoing evaluation of new-onset polyarthritis
involving the small joints of her hands. She also has had a recurrent
urticarial eruption over her shins (see figure below) for the past 3-4
days. Both symptoms developed over the past 6 months.
Review of systems is notable for the recent need to switch from contact
lenses to traditional lenses because of persistent corneal irritation. Although
her previous dental health was excellent, she has recently developed three
new caries. She takes no medications.
Figure
Question
Which of the following additional findings is most likely in this patient?
A. Hilar adenopathy
B. Cryoglobulins
C. Anti-SSA/Ro antibody
D. Anti-thyroglobulin antibody
Correct Answer
C
Answer Rationale
The patient has secondary Sjögren syndrome in the setting of what is most
likely rheumatoid arthritis, given the symmetrical involvement of the small
joints of her hands. Sjögren syndrome may have a variety of cutaneous
manifestations, including urticarial eruptions. Sjögren syndrome patients with
anti-SSA/Ro antibody are more likely to have such extraglandular
manifestations of their disease. Dental caries at the gingival margin, as seen
in this patient, are characteristic in patients with oral sicca symptoms,
autoimmune or otherwise.
Question Number: 43
Case
A 38-year-old man has polyarticular arthritis. Despite his relative youth and
absence of any history of joint trauma, he has widespread arthritis affecting
his axial skeleton, shoulders, and hips. He reports no visible articular swelling
or erythema or palpable heat. He has otherwise been in excellent health and
has no family history of early-onset arthritis, either inflammatory or
mechanical.
Figure 1 Figure 2
Question
Which of the following tests is most likely to confirm the diagnosis?
A. COMP haplotype analysis
Correct Answer
D
Answer Rationale
The patient has alkaptonuria, an inborn error of metabolism resulting in
inability to metabolize homogentisic acid. Homogentisic acid is deposited in
the soft tissues and cartilage causing ochronosis, darkening of the sclerae,
pinnae, and other soft tissues, as well as premature osteoarthritis. The urine
will also darken if exposed to air.
The COMP haplotype, FRZB 2-marker SNP haplotype, and type II collagen
polymorphisms have all been associated with premature osteoarthritis, an
effect that varies with the gender and ethnicity of the patient and the specific
joint studied, but will not cause the pigmentary changes seen in ochronosis.
Question Number: 44
Case
A 55-year-old woman has noted a sensation of grit in her eyes for the past 2
years. She has been using artificial tears 2 or 3 times a day, particularly
while watching TV or working at a computer. Within the past year, she has
also noted dryness of her mouth. She keeps a bottle of water at her bedside
and must drink water frequently to aid in swallowing dry food. She has not
noted any recent increase in dental caries. She does not have a rash, joint
pain, or fatigue. She does not have clinical evidence of systemic lupus
erythematosus, rheumatoid arthritis, or scleroderma.
WBC: 5600/mm3
Hgb: 13.2 gm/dl
Platelet count: 253,000/ mm3
ANA testing (multiplex assay): negative antinuclear antibodies, negative SS-
A and SS-B antibodies
Question
Which of the following additional findings is an absolute requirement for
establishing the diagnosis of primary Sjögren’s syndrome in this patient?
A. Schirmer’s I test with wetting of 7 mm in the right eye and 4 mm in the left eye.
Correct Answer
C
Answer Rationale
Although there have been several sets of diagnostic criteria for Sjögren’s
syndrome over the past 40 years, the set that is currently accepted was
derived by an international consensus group in 2002. According to the 2002
International Consensus Criteria for Sjögren’s Syndrome, the diagnosis of
primary Sjögren’s syndrome requires sicca signs/symptoms in the presence
of either Sjögren’s antibodies (SS-A and/or SS-B) or a positive labial gland
biopsy, defined by the presence of a focus score of 1 or more. With the
exception of the absent salivary pool, the other tests (abnormal Schirmer’s,
unstimulated salivary flow, or parotid sialography) would provide objective
evidence of the ocular or oral component of the disease and at least one
would be required to establish a diagnosis of Sjögren’s syndrome in concert
with the abnormal labial gland biopsy.
Question Number: 45
Case
A 34-year-old woman with a 1-year history of scleroderma is seen in your
practice as a new patient after moving from another city. She has had
Raynaud’s phenomenon in her hands as well as skin tightening on the hands
and face. She is currently taking lisinopril, 20 mg/day and ASA 325
mg/day. She is a nonsmoker and has no family history of autoimmune
disease. Recently, she has developed a persistent dry cough and she
becomes short of breath after walking one block.
Blood pressure is 135/70 mmHg. She has tightening of the skin on the face
and fingers with digital pitting but no cyanosis. Velcro crackles are present
at both lung bases and she has borderline tachycardia at 105 bpm. Lower
extremities are without edema. Findings on chest radiograph are
normal. Echocardiogram shows a normal ejection fraction and an estimated
PA pressure of 25 mmHg. She is referred to a pulmonary specialist who does
bronchoaveolar lavage, and the fluid from this procedure shows 4%
neutrophils. Cultures and stains on the fluid are negative for infectious
agents.
Question
Which of the following medications should be initiated at this time?
A. Prednisone 60 mg/day
E. Infliximab 3 mg/kg
Correct Answer
B
Answer Rationale
The use of cyclophosphamide in scleroderma lung disease is based on results
from the Scleroderma Lung Study that compared in a blinded fashion oral
cyclophosphamide to placebo. Data from this 1-year study showed a
statistically significant improvement in lung function parameters in patients
treated with cyclophosphamide. Improvements were also observed in
dyspnea and in quality of life indices, and these improvements persisted in
the year following cessation of cyclophosphamide treatment. The patient in
this case has neutrophilia on BAL consistent with the presence of
alveolitis. Prednisone in high doses is relatively contraindicated in
scleroderma because of the risk of precipitating renal crisis. The use of
mycophenolate mofetil has been assessed only in limited retrospective
studies and data regarding utility of rituximab and infliximab are largely
limited to case reports. There have been some reports suggesting that TNF
blockade might induce a scleroderma-like syndrome.
Question Number: 47
Case
A 30-year-old man was given the diagnosis with of rheumatoid arthritis 2
years ago after he was evaluated for intermittent episodes of migratory
painful polyarthritis involving the knees, ankles, and elbows. The initial
episode lasted two weeks and on recurrence, synovitis of both elbows and
tenderness in the wrists and hands was noted. At that time, rheumatoid
factor was positive and anti-citrullinated peptide antibodies were
negative. Therapy was initiated with methotrexate up to 20 mg orally every
week without improvement. Between episodes he has polyarthralgias. His
father died at age 36 years from a myocardial infarction. Findings on
physical examination are normal with the exception of small nodules on the
Achilles, olecranon, and tibial tubercle. His hands and foot films are normal.
Question
Which of the following tests is the most appropriate next step to establish the
diagnosis?
A. Anti-citrullinated peptide antibody titer
Correct Answer
D
Answer Rationale
Lipoprotein disorders result from defect in either the synthesis or catabolism
of lipoprotein. Familial hypercholesterolemia is an autosomal dominant
disorder caused by mutation in the gene encoding for low density lipoprotein
(LDL) receptor. Deficiency results in high concentration of LDL in connective
tissues and arterial beds causing xanthomas and atherosclerosis. In patients
with the heterozygous from of familial hypercholesterolemia, tendinous
xanthomas usually occur in the second decade of life and coronary artery
disease events after the third decade. Tendon xanthomas are typically seen
within the extensor tendons of the hands, Achilles tendon, and tibial
tubercle. Arthritis (seen in 40%-50% of patients) most commonly involves
the knees and ankles. Patients can develop intermittent episodes of either
migratory, pauciarticular, or polyarticular arthritis lasting for several
days. Assay for IgA antibodies to endomysium are sensitive and specific for
celiac disease and although peripheral and/or axial arthritis may be present,
most patients have other autoimmune and/or intestinal manifestations not
present in this patient. There is no indication for ANA, Anti-CCP, or PRP as
the probability of an autoimmune systemic disorder, RA, or disseminated GC
presenting with two years of migratory arthritis is very low and given low
pretest probability these tests are not the diagnostic studies of choice in this
case.
Question Number: 48
Case
A 50-year-old woman with a 17-year history of rheumatoid arthritis is
undergoing routine follow-up evaluation. She has no new symptoms except
the presence of a new skin lesion on the nailfold of the right little finger (5th
digit) (see Figure). She does not recall any trauma to the area and she says
that it is not painful. There is no discharge from the lesion and she does not
have any similar lesions anywhere else on her body. She describes a
sensation of numbness and tingling and a perception of weakness in both
hands and both feet.
Findings on physical examination include the skin lesion shown in the figure.
Examination of the joints shows mild swelling over the MCP joints 2-3-4 of
both hands and minimal swelling of both wrists. There are rheumatoid
nodules over both olecranon surfaces, each measuring about 1.5 cm in
diameter, unchanged in size since the last visit.
There is intact diminished sensation to light touch and pinprick in both hands
and feet in a stocking and glove distribution.
CBC: normal
ESR: 33 mm/hr
C-RP: 5.1 mg/dL
C3: Low
C4: Low
Rheumatoid factor: 298 U
CCP: < 10 U
Figure
Question
Which of the following is the most appropriate next step in management?
A. Add an anti-TNF agent
D. Begin cyclophosphamide
E. Add aspirin
Correct Answer
D
Answer Rationale
This patient has a nailfold infarction and sensory symptoms. This should raise
concern about the possibility of the development of rheumatoid vasculitis
(RV). RV refers specifically to a protean, destructive inflammatory process
that is centered on the blood vessel wall itself and associated with substantial
morbidity. It may affect a wide range of blood vessel types, from medium
muscular arteries to somewhat smaller arterioles to postcapillary venules.
Within a given patient, clinical features of both medium- and small-vessel
disease may be found. RV leads to necrosis, blood vessel occlusion, and
tissue ischemia in a manner that resembles other forms of systemic
vasculitis, particularly polyarteritis nodosa.
The areas of the body involved most commonly by RV are the skin, digits,
peripheral nerves, eyes, and heart. The most devastating form of RV is
characterized by a medium-sized vasculitis reminiscent of (and histologically
identical to) polyarteritis nodosa. Many of the clinical manifestations of RV
reflect this predilection for medium-sized vessels, even though purpura,
petechiae, and papules (manifestations of small-vessel involvement) may
also occur.
Question Number: 49
Case
A 66-year-old man with a history of ongoing chronic alcohol abuse, comes to
the emergency department because of bilateral knee pain. Physical
examination shows a thin man with stigmata of liver disease, poor hygiene,
several nonblanching ecchymoses on both upper and lower extremities (see
picture) and bilateral knee effusions. There are no skin abrasions or
ulcers. He is able to flex his knees to 95 degrees with discomfort; both
knees are warm to touch. Arthrocentesis of the right knee is performed and
shows a hemorrhagic effusion. A nontraumatic tap of the left knee reveals a
hemorrhagic effusion also. (Image 1)
Figure
Question
Which of the following is the most likely cause of the hemarthrosis?
A. Thrombocytopenia
D. Circulating anticoagulant
Correct Answer
C
Answer Rationale
Vascular fragility due to vitamin C deficiency may lead to hemarthrosis and
subperiosteal bleeding. Scurvy is a clinical syndrome seen with ascorbic acid
deficiency largely due to impaired collagen synthesis with disordered
connective tissue. Symptoms (occurring as early as three months after
deficient intake) include ecchymoses, bleeding gums, petechiae,
hyperkeratosis, Sjögren-like syndrome, arthralgias, and impaired wound
healing. Generalized systemic symptoms are weakness, malaise, joint
swelling, arthralgias, edema, coiled hair, depression, neuropathy, and
vasomotor instability. In the United States, ascorbic acid deficiency occurs
mostly in severely malnourished individuals, drug and alcohol abusers, or
those living in poverty. Although as many as 80 percent of hospitalized
alcoholics have mild thrombocytopenia, in most the platelet function remains
normal. Decreased production of coagulation factors occurs in hepatic failure
but this patient’s PT and PTT are normal, which also eliminates the possibility
of a circulating anticoagulant as the cause of the hemarthrosis. Pigmented
villonodular synovitis or diffuse tenosynovial giant cell tumor is a benign
neoplasm that typically presents as an intra-articular tumor expanding along
the synovial surface into the soft tissue. Clinical presentation of PVNS
usually involves the knee with pain, swelling, and dysfunction. Bloody
effusions are common. These tumors present at a younger age with most
patients younger than 40, monoarticular, and without systemic
manifestations.
Question Number: 50
Case
A 36-year-old man has a 14- month history of intermittent pain and stiffness
in the hands and feet. One year ago, he was evaluated for the persistent
symptoms in his hands and feet. He had Achilles tendon pain. Occasionally
he would have lumbar spine pain; however, activity helped improve the
symptoms.
Physical examination one year ago showed swelling of the right 2nd and 4th
toes and the left 3rd finger. A modified Schober test was 10 cm to 15 cm.
ESR: 25 mm/hr
He now has had progressive low back pain and stiffness for the past 4
months. He describes morning stiffness that lasts for 2 hours. Two months
ago, he had iritis that was treated with topical corticosteroids.
Radiograph of the sacroiliac joint (SI joints) reveals erosive changes on the
iliac sides.
ESR: 25 mm/hr
Question
Which of the following is the best treatment option at this time?
A. Leflunomide
B. Cyclosporine
C. Methotrexate
D. Azathioprine
Correct Answer
E
Answer Rationale
Ankylosing spondylitis affects mainly the axial skeleton. The majority of
patients will have sacroiliac joint involvement. If a patient has peripheral
joint arthritis then sulfasalazine, cyclosporine, methotrexate or Arava may be
effective. However, axial involvement is best treated with TNF alpha
antagonist. Concomitant use of methotrexate with TNF alpha antagonist has
not been found to be superior to TNF alpha antagonist monotherapy for
treatment of axial manifestations.
Question Number: 51
Case
A 19-year-old African-American man has joint pain and a positive ANA (1:80
dilution). Approximately 3 years ago, he started having intermittent
episodes of severe bilateral knee, ankle, and hip pain. During these episodes
he has difficulty getting around. They occur 2 to 3 times a year, last for 2 to
3 days, and resolve spontaneously. He has not had any associated
symptoms, such as fever, rash, swollen joints, or cardiopulmonary
problems. NSAIDs, oxycodone, and morphine did not provide any
benefit. He is a smoker, nondrinker, and denies any illicit drug use. He
plays golf but has no other physical activities. He has not traveled outside
the USA. His mother has systemic lupus erythematosus. Findings on
physical examination are normal.
Hgb: 11 gm/dl
Hct: 36%
ENA: negative
Chemistry profile: normal
Urinalysis: normal
Plain radiographs of chest and knees: normal
MRI of the right knee: Infarct of the medial femoral condyle, small infarct of
the anterior distal femoral metaphysis and multiple small areas of edema.
Question
Which of the following studies is most likely to establish the diagnosis?
A. Bone marrow biopsy
B. Bone scan
C. Hemoglobin electrophoresis
E. Antiphospholipid antibodies
Correct Answer
C
Answer Rationale
The clinical diagnosis of osteonecrosis is appropriately made in a
symptomatic patient when imaging findings are compatible with this disease
and other causes of pain and bony abnormalities are either unlikely or have
been excluded by appropriate testing as in this case. Although there are
many conditions that lead to avascular necrosis, the most likely cause in this
case is hemoglobinopathy. Clues to this are young age of onset, the painful
crisis, his ethnicity, and lack of systemic symptoms. Hemoglobin
electrophoresis will help to determine the cause of the painful crisis
associated with bone infarct. Long-bone infarct occurs most commonly with
HgBSS (sickle cell disease) but also with HbSC, HbS alpha thal, and least
commonly in sickle cell trait.
Question Number: 52
Case
A 58-year-old man has a 3-year history of progressive muscle weakness
involving his shoulder and pelvic girdles. He had some difficulty swallowing
liquids. He has been treated for 3 months with prednisone 60 mg/day with
no benefit.
Physical examination shows 4/5 strength in shoulder abduction and hip
flexion.
Previously, he has had CK levels that were 3 to 4 times the upper range of
normal. Electrophysiologic studies revealed increased insertional activity,
fibrillation, and positive waves. Motor units were of low amplitude and short
duration. A muscle biopsy showed some atrophic and triangular fibers with
a few lymphocytes in and around muscle fibers. Centralized nuclei were seen
but no necrosis or regenerating cells were present. Vacuoles were seen in
some fibers. Trichrome staining revealed rare ragged red change.
Histochemistry with ATPase staining revealed fiber type grouping, and Congo
red staining was positive in fibers with vacuoles.
Question
In addition to physical therapy for muscle strengthening, which of the
following management options is most appropriate at this time?
A. Taper the prednisone
B. Sart azathioprine after checking thiopurine methyltransferase (TPMT) activity and slowly taper
prednisone
C. Start methotrexate and azathioprine after checking thiopurine methyltransferase (TPMT) activity
and slowly taper prednisone
D. Add IVIg
E. Add methotrexate
Correct Answer
A
Answer Rationale
Muscle histology in patients with inclusion body myositis may look identical to
that from patients with polymyositis; that is, degenerating and regenerating
muscle fibers with lymphocytes infiltrating the muscle and inside muscle
fibers. Over time, these inflammatory changes tend to resolve. In addition,
Trichrome staining may show ragged red change within some fibers. This
change is indicative of mitochondrial abnormalities and would suggest a
mitochondrial myopathy. However, ragged red fibers are reported with aging
and a mitochondrial myopathy would not be associated with the
inflammatory changes seen in this patient. In addition, patients with
inclusion body myositis may have neuropathic abnormalities. On physical
examination, these may manifest as distal or asymmetric muscle
weakness. In muscle tissue, these are indicated by finding triangular cells
and fiber type grouping. Amyloid, as demonstrated with Congo red staining,
is present in IBM muscle fibers and in some vacuoles.
Question Number: 53
Case
A 46-year-old man has had hypertension and diabetes mellitus for 20 years
and has now developed chronic kidney disease. Ten years ago, he had what
appears to have been his first attack of gout. Synovial fluid aspirated from
his right knee at the time of a second attack contained monosodium urate
crystals. Over the next few years, he had multiple attacks of gout and visible
tophi developed on his elbows.
Two years ago, when his serum creatinine level was 2.0 mg/dl, he was
prescribed allopurinol 100 mg. This treatment led to a drop in his serum
urate level from 11.0 mg/dl to 9.0 mg/dl. One year ago, the dose of
allopurinol was increased to 300 mg and colchicine 0.5 mg every other day
was added.
He has not had a gout flare in the past 9 months but the tophi remain
unchanged. Currently blood pressure is 165/100 mmHg, creatinine level is
2.3 mg/dl, and serum urate is 6.9 mg/dl (normal urate level in men is listed
as 3.6-8.5 mg/dl).
Question
Which of the following interventions is most appropriate at this time?
A. Discontinue colchicne
Correct Answer
E
Answer Rationale
Although many laboratories list the upper limit of normal for serum urate
levels above 6.8 mg/dl, the true physiochemical upper limit of normal of
serum urate is 6.8 mg dl. Above this concentration, body fluids are
supersaturated with urate and crystals can form and deposit. When levels
above 6.8 mg/dl are listed as normal, they are based on statistical norms for
a population and are misleading regarding the pathophysiology of the
gout. To eliminate attacks and shrink tophi, serum urate levels must be
maintained below 6.8 mg/dl and preferably below a target value of 6.0
mg/dl.
Question Number: 54
Case
A 52-year-old woman has recently received the new diagnosis of
osteoarthritis in the knees, which was made by her primary care
physician. She is otherwise generally healthy. She was a very active runner
for many years and participated in 5K races until about 2 years ago. Now
she just jogs recreationally because she is somewhat limited by pain in her
knees. She does not recall that anyone in her family had arthritis, and she
wants to know if there is anything she can do to improve her prognosis.
Figure
Question
Which of the following has most likely contributed to cartilage loss in this
patient?
A. BMI of 24.1
B. Jogging
C. Use of cigarettes
Correct Answer
C
Answer Rationale
While weight can impact osteoarthritis of the knee, this woman has a normal
BMI, so loss of weight is not likely to have significant benefit. Several
studies have now discounted the role of jogging or distance running in
accelerating the progression of knee OA. Furthermore, strength of the
quadriceps muscles that can be developed by this kind of exercise appears to
be an important factor in protection of the knee joint. Recent data suggest
that smoking is a modifiable risk factor for cartilage loss, so quitting cigarette
use is something that she can do to improve her joint health. Estrogen
supplements may slow rather than accelerate cartilage loss, so discontinuing
the patch probably will not help her joints.
Question Number: 55
Case
A 28-year-old woman with a history of systemic lupus erythematosus,
manifesting in the past as pleurisy, arthralgia, malar rash, and prior second
trimester fetal loss, now has a positive pregnancy test. Her last menstrual
period was eight weeks ago. She feels fatigued but has no other
symptoms. She takes hydroxychloroquine 400 mg once daily and a prenatal
multivitamin. On physical examination, blood pressure is 110/70 mmHg and
pulse rate is 88 bpm. No rashes are present.
Question
Which of the following is the most appropriate additional treatment for this
patient at this time?
A. Aspirin 81 mg per day
Correct Answer
D
Answer Rationale
This patient is in her first trimester of pregnancy and has a history of
antiphospholipid syndrome with moderate titer anticardiolipin IgG antibody
positivity and prior fetal loss. Her risk for miscarriage is increased. Studies
comparing heparin and aspirin versus aspirin alone show superiority of
combination therapy. Prednisone use poses greater risk for corticosteroid
complications without improvement in outcome.
Question Number: 56
Case
A 72-year-old woman has had a 3-month history of an erythematous rash
over her face, forearms, and upper torso. Serologic testing revealed the
presence of ANA (1:640, speckled pattern) and hydroxychloroquine 400
mg/day was prescribed. She has had no joint swelling or muscle weakness
but has had some mild dysphagia for liquids. Physical examination shows
normal blood pressure; there is a confluent erythematous papular rash over
the upper torso and sun-exposed areas of her arms extending onto the
dorsal aspect of the hands with periungual erythema and mild scale
formation over the dorsal PIP and MCP joints. Erythema is also present over
the sun-exposed areas of the face and eyelids. Oral and ocular membranes
are normal and lungs are clear without audible rub or crackles. Joints are
normal without limitation or synovitis. Strength in the upper and lower
extremity muscle groups is normal.
CBC: normal
Chemistry profile: normal
Urinalysis: normal
CK: normal
Aldolase: normal
Question
Which of the following studies is most appropriate at this time?
A. Biopsy of affected dermis
Correct Answer
D
Answer Rationale
Erythematous rash occurring on the face, eyelids, neck, and upper torso
along with papular lesions on the dorsum of the hands with periungual
erythema are very characteristic of dermatomyositis. The presence of orbital
violaceous erythema (heliotrope rash) and erythematous papules over the
dorsum of the hands is sufficient to establish the diagnosis of
dermatomyositis. Skin biopsy frequently reveals interface dermatitis similar
to that seen in lupus and, for patients with these classic cutaneous
manifestations, adds little information to guide management. For patients
with demonstrable muscle weakness or CK elevation in the context of these
characteristic skin lesions, muscle biopsy is also not required to establish the
diagnosis.
Case
A 42-year-old man has had bilateral hand pain for the past 2 years. The
symptoms are variously characterized as paresthesias and numbness. A
nerve conduction study revealed an axonal neuropathy with conduction delay
at his wrists. Nocturnal wrist splints failed to lead to improvement in his
symptoms, and he underwent bilateral carpal tunnel release. However, his
symptoms became progressively worse.
Question
Which of the following laboratory studies is most likely to determine the
cause of the patient’s progressive symptoms?
A. Hemoglobin A1c
C. Vitamin B12
Correct Answer
C
Answer Rationale
Clinically, the patient had carpal tunnel syndrome at his initial presentation.
While rheumatologists frequently encounter this in the setting of wrist
synovitis, peripheral neuropathy resembling carpal tunnel syndrome may
also result from other metabolic and systemic processes, including
acromegaly, amyloidosis, diabetes mellitus, hypothyroidism,
hyperparathyroidism, pregnancy, and vitamin B12 deficiency.
This patient has vitamin B12 deficiency as noted by the abnormal sensory
examination in his lower extremities, as well as mild anemia and marginally
elevated mean corpuscular volume.
Diabetes mellitus is a much less likely cause of underlying peripheral
neuropathy, given the low-normal fasting glucose level. There is no
association between folic acid deficiency and carpal tunnel syndrome.
Acromegaly, diagnosed by the detection of an elevated insulin-like growth
factor I level, is also unlikely to be the cause of the worsening peripheral
neuropathic symptoms given his slight build and fine features, as opposed to
the more coarse features and broad hands expected in an individual with the
onset of acromegaly after puberty.
Question Number: 58
Case
A 55-year-old woman with diabetic glomerulosclerosis started hemodialysis 3
years ago. One year ago, swelling of her feet, lower legs, and hands
developed. Within a few weeks, this swelling progressed to a woody
induration, leading to flexion contractures of her fingers. There was also
progression of the induration to the forearms, upper legs, and thighs.
Currently the affected skin has a brawny induration with areas of a peau
d’orange appearance as well as deep furrowing and loss of skin appendages.
Skin biopsy shows diffuse dermal fibroplasia with spindle cells extending into
the subcutaneous tissues. There are thick collagen bundles separated by
large clefts in the dermis. Increased mucin is present.
Question
Which of the following additional findings is most likely in this patient?
A. Monoclonal gammopathy
B. Peripheral eosinophilia
D. Raynaud’s phenomenon
E. Myocardial fibrosis
Correct Answer
E
Answer Rationale
Nephrogenic systemic fibrosis (NSF), formerly known as nephrogenic
fibrosing dermopathy, has been observed exclusively in patients with renal
insufficiency, most but not all of whom have undergone dialysis for renal
failure. The disease was initially thought to be confined to the skin. However,
recent reports have documented involvement of the liver, myocardium,
lungs, diaphragm, and skeletal muscle. Increased ESR and C-reactive protein
level are commonly present. Paraprotein is a feature of scleromyxedema but
is not seen in NSF. The disease has been linked recently to the use of
gadolinium-based contrast agents for magnetic resonance imaging.
Question Number: 59
Case
A 72-year-old woman has had right knee pain and stiffness for the past six
months when arising from sitting. The knee pain worsens when she climbs
stairs. She has had no trauma. She has a history of gastroesophageal reflux
disease and hypertension for which she takes omeprazole and enalapril. On
physical examination, her weight is 110 pounds. Blood pressure is 130/80
mmHg. Right knee crepitus, medial joint tenderness, and a fluid wave are
present. Radiograph of the right knee shows mild joint space
narrowing. She is treated with three weekly intra-articular injections of hylan
G-F 20.
Three days after her last injection, she has severe worsening of pain and
swelling of the right knee. Arthrocentesis of the right knee is performed and
reveals 55 cc of cloudy yellow fluid.
Question
Which of the following is the most likely diagnosis?
A. Osteonecrosis
B. Pseudogout
C. Pseudoseptic reaction
D. Rheumatoid arthritis
E. Septic joint
Correct Answer
C
Answer Rationale
This patient has a pseudoseptic reaction to injection with a hyaluronate. This
type of reaction may occur in 11% of injections with hyaluronate and is most
commonly noted with hylan G-F 20. This reaction usually occurs in the first
week after the injection. Nucleated cell counts may exceed 100,000/mm3
and are predominantly neutrophils. Iatrogenic septic joint after intra-
articular injection has been estimated to occur once in 2,000 to once in
15,000 injections. Some cases of crystal-proven pseudogout have been
reported to occur after injection of hylan G-F 20. However, this patient’s
synovial fluid did not contain any crystals. Osteonecrosis is not a
complication of hyaluronate injection and is not associated with highly
inflammatory synovial fluid. This patient does not meet criteria for a
diagnosis of RA.
Question Number: 60
Case
A 14-year-old girl has a 6-week history of unexplained low-grade fever,
fatigue, and joint pain. Family history reveals that her 4-year-old brother
has a history of perirectal abcesses, two hospitalizations for staphylococcal
pneumonia, and a chronic cervical lymphadenitis for which culture grew
Serratia species.
Question
Which of the following is the most likely diagnosis in the sister?
A. Inflammatory bowel disease
C. Endocardial elastosis
Correct Answer
D
Answer Rationale
The x-linked form of CGD usually presents with perirectal abcess and
difficulties in clearing staph infection and infection caused by other catylase-
positive organisms. Having a brother with these symptoms should raise the
index of suspicion of SLE. More commonly, this is discoid lupus, but it can
also be systemic. In the absence of that diagnosis, IBD and CVID could also
cause similar symptoms and appropriate evaluation should be undertaken.
Inflammatory bowel disease may present with fever, fatigue, and arthralgia,
but the family history points more toward SLE. Common variable immune
deficiency (CVID) may also have similar features, but the age of onset and
types of infections in the brother are not those usually found with CVID. The
carriers of CGD are also at higher risk for endocardial fibroelastosis, but the
symptoms would more likely be shortness of breath and cardiac failure. The
patient had no adenopathy or rash suggestive of EVB, and duration is too
long for this to be likely.