Case-based MCQ

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A 65-year-old man comes to the office for evaluation of new-onset pain in the right knee.  The patient first noticed the pain 3 weeks ago after a long hike in the mountains.  The pain has not improved with acetaminophen, and he is now unable to sleep because of it.  He has also had intermittent low-grade fevers.  The patient has Paget disease of bone, which was diagnosed 10 years ago, and type 2 diabetes mellitus.  He drinks alcohol socially but does not use tobacco or illicit drugs.  Temperature is 37.2 C, blood pressure is 120/70 mm Hg, and pulse is 80/min.  BMI is 23 kg/m2.  The right knee has diffuse soft tissue swelling and a small effusion with no surrounding erythema.  The distal aspect of the thigh is tender to palpation.  X-ray of the right knee reveals medullary and cortical bone destruction with lucent areas and a surrounding periosteal elevation along the distal femur.  Which of the following is the most likely cause of this patient’s knee pain? A. Borrelia burgdorferi–induced monoarticular arthritis B. Destruction and inflammation of articular cartilage C. Formation of stress-related microfractures D. Malignant proliferation of osteoid sarcomatous stroma E. Precipitation of calcium pyrophosphate dihydrate crystals

This young woman with constitutional symptoms (fatigue), thrombocytopenia, probable mitral regurgitation (holosystolic murmur to the apex), and a positive antinuclear antibody (ANA), suggestive of systemic lupus erythematosus (SLE), now likely has a cerebrovascular accident (CVA) (sudden-onset unilateral weakness, negative cerebrospinal fluid [CSF] findings).  Given these features, antiphospholipid syndrome (APS) in the setting of SLE is likely. APS presents with a thrombotic event (deep venous thrombus or arterial thrombus) or pregnancy morbidity (fetal loss, severe preeclampsia, placental insufficiency) plus a positive serology for 1 of 3 antiphospholipid antibodies: anticardiolipin antibody, anti-beta2-glycoprotein-I antibody, or lupus anticoagulant.  The biggest risk factor for APS is SLE; APS occurs in up to 40% of these patients. A minority of healthy individuals may develop transient antiphospholipid antibodies, so all positive serology for APS should be repeated at 12 weeks to confirm diagnosis.  Patients with APS often require anticoagulation for life (heparin product or warfarin) as the risk of recurrent thrombus is high.  For those with concomitant SLE, hydroxychloroquine is also added. Multiple sclerosis (MS) presents with neurologic symptoms, but transverse myelitis causing a paraplegia or paraparesis is much more common than hemiparesis.  As MS is inflammatory, symptoms tend to develop over hours or days, not suddenly.  MS also would not explain this patient’s hematologic abnormalities, positive ANA/rapid plasma reagin (RPR), or murmur. Neurosyphilis can occasionally present with an arteritis causing a cerebrovascular event in a young patient, but it is typically accompanied by abnormal CSF cell counts (lymphocytic pleocytosis, 10-100/mm3).  In patients with APS, a false positive RPR test is not uncommon as the syphilis antigen used in RPR testing contains cardiolipin. Polyarteritis nodosa can cause a CVA in a minority of patients, but mononeuritis and polyneuropathy are far more common.  In addition, other findings such as arthralgias, myalgias, and cutaneous, renal, and/or gastrointestinal disease are often also present. Rheumatic fever causes chorea-like movements (Sydenham chorea).  It commonly presents with carditis and migratory arthritis. Antiphospholipid syndrome is characterized by a thrombotic event or pregnancy morbidity in the setting of a positive autoantibody to 1 of 3 phospholipids – anticardiolipin antibody, anti-beta2-glycoprotein-I antibody, or lupus anticoagulant.  It is very common in patients with systemic lupus erythematosus.

A 32-year-old woman is brought to the hospital due to sudden-onset left-sided weakness.  According to the family, she has had fatigue, malaise, and decreased appetite over the last several weeks.  She attributed these symptoms to a “viral infection” and treated herself with over-the-counter acetaminophen.  Her temperature is 37.4 C (99.4 F), blood pressure is 140/86 mm Hg, and pulse is 82/min.  The patient appears confused and agitated.  Lungs are clear to auscultation.  A 2/6 holosystolic murmur is heard at the cardiac apex.  Extremity examination reveals left-sided muscle strength is 1/5 with hyperactive deep tendon reflexes.  Babinski sign is present on the left side.  Laboratory results are as follows: Hemoglobin 10.2 g/dL Platelets 64,000/mm3 Leukocytes 6,800/mm3 Blood urea nitrogen 18 mg/dL Creatinine 70 umol/L Antinuclear antibody (ANA) positive Rapid plasma reagin (RPR) positive Cerebrospinal fluid cell counts are normal, and cultures are negative.  Which of the following is the most likely diagnosis in this patient? A. Antiphospholipid syndrome B. Multiple sclerosis C. Neurosyphilis D. Polyarteritis nodosa E. Rheumatic fever

Supracondylar fractures of the humerus are the most common elbow fractures in children.  The mechanism typically involves a fall onto an outstretched hand with a hyperextended elbow.  The supracondylar area is relatively thin and weak due to physiologic remodeling during childhood, and a supracondylar fracture most commonly results in posterior displacement of the distal humerus fragment, as seen in this patient.  The anteriorly displaced proximal fragment can entrap the median nerve and brachial artery, which are located anterior to the humerus. Distal perfusion should be monitored closely because brachial artery injury results in diminished brachial and radial pulses.  Motor and sensory function should also be assessed due to the risk of median nerve injury that can present with a weak “OK” sign (ie, inability to approximate tip of thumb and index finger). Diagnosis is based on x-ray, which reveals a fracture line and often a posterior fat pad indicative of elbow effusion.  Treatment involves analgesia and immobilization.  Neurovascular injury often resolves after orthopedic alignment and immobilization.  Patients who are treated promptly have a good prognosis. The axillary artery and nerve can be injured during proximal humerus fractures but not with supracondylar fractures. Brachial plexus compression is a complication of neonatal clavicle fracture or high-impact trauma to the neck and shoulder area, not the elbow. Ulnar nerve injury is a complication of a rare type of supracondylar fracture that occurs upon impact of a flexed (rather than hyperextended) elbow.  This mechanism of injury causes anterior displacement of the distal humerus fragment, which can impinge on the ulnar nerve located posterior to the medial epicondyle of the humerus. Displaced supracondylar fractures of the humerus most commonly present after a fall onto an outstretched hand with posterior displacement of the distal humerus fragment.  The anteriorly displaced proximal humerus fragment can entrap the brachial artery and median nerve, which pass anterior to the humerus.

A 7-year-old girl is brought to the emergency department due to a right arm injury.  While jumping on a trampoline, she slipped and fell onto her outstretched right hand.  The patient had immediate pain and would not move her arm.  She did not hit her head or lose consciousness.  On examination, the patient is crying in pain, with significant ecchymosis and swelling just above the elbow.  She is holding the right arm in flexion and winces when it is moved.  X-ray reveals a supracondylar fracture with posterior displacement of the distal humerus fragment, as shown in the image below. Which of the following is at greatest risk of injury in this patient? A. Axillary artery B. Axillary nerve C. Brachial artery D. Brachial plexus E. Ulnar nerve

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Correct Answer Is B The risk factors for developing pseudogout include: -Age more than 70 years. -The family history of pseudogout. -Hypothyroidism. -Haemochromatosis. -Hyperparathyroidism. -Hypercalcemia.

Which of the following increases a person’s risk of getting pseudogout? A. Age less than 50 years B. Hypothyroidism C. Hypocalcaemia D. Hypoparathyroidisim E. Hypermagnesemia

Diagnosis of definitive SLE requires four or more criteria, with at least one clinical and one laboratory, with the exception of biopsy-proven LN (which requires fewer criteria). Criteria are cumulative and need not be present concurrently. The antinuclear antibodies (ANA) test is the serological hallmark of SLE. Up to 98% of patients with SLE will have a positive ANA, making it highly sensitive and useful as a screening test. A negative ANA makes SLE very unlikely and other diagnoses should be sought to explain symptoms. Antibodies to double-stranded DNA (dsDNA) are specific for SLE. In some patients, an increase in anti-dsDNA titre may signify onset of disease flare. Other autoantibodies, available on the extractable nuclear antigen-testing panel, can also be associated with SLE or other connective tissue diseases. Antibodies to Sm (anti-Smith), for example, has low sensitivity but high specificity for systemic lupus erythematosus (SLE). It is occasional present in mixed connective-tissue disease (MCTD) While antiphospholipid antibodies are not specific for SLE, they are part of the immunological abnormalities that can be associated with pregnancy morbidities and thrombotic complications. Testing should include anticardiolipin antibodies, lupus anticoagulant and anti-β2 glycoprotein 1.

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A 22-year-old lady presented to you with frequent fatigue, joint pain and weight loss. On physical examination noted that she has malar rash and oral ulcers. A diagnosis of systemic lupus erythematosus Which of the following statement is INCORRECT regarding SLE? A. It mainly affects women in high estrogen period B. It is more common in female C. Polyarthritis is the commonest symptom of SLE D. Antinuclear antibody is highly sensitive towards SLE E. Anti-Smith antibody is highly sensitive towards SLE

A 22-year-old lady presented to you with frequent fatigue, joint pain and weight loss. On physical examination noted that she has malar rash and oral ulcers. A diagnosis of systemic lupus erythematosus Which of the following statement is INCORRECT regarding SLE? A. It mainly affects women in high estrogen period B. It is more common in female C. Polyarthritis is the commonest symptom of SLE D. Antinuclear antibody is highly sensitive towards SLE E. Anti-Smith antibody is highly sensitive towards SLE

Correct Answer Is C This patient has presented with signs and symptoms of rheumatoid arthritis. The criteria to diagnose rheumatoid arthritis include the following: – Symptoms duration more than 6 weeks. – Early morning stiffness for more than 1 hour. – Arthritis in three or more joints. – Bilateral compression tenderness of the metatarsophalangeal joints. – Symmetry of the areas affected. – Rheumatoid factor, Anti-CCP antibody positivity. The role of non-steroidal anti-inflammatory drugs to reduce the pain and inflammation is well known in patients with rheumatoid arthritis. However, they do not prevent the joint damage and are associated with many side effects. Standard initial therapy with disease-modifying anti-rheumatic drugs helps to prevent damage to the joint in any patient with the rheumatoid arthritis. Methotrexate is the drug of choice as initial monotherapy. If disease remission is not achieved, increase the dose of methotrexate or consider combination therapy with other agents like sulfasalazine and hydroxychloroquine.

A 32-year-old woman presented with 3 month history of joint pain in both hands and stiffness lasting for hours once she wakes up in the morning every day. She also reported disturbed sleep due to the nocturnal pain. On examination, you notice swelling around metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joint. Which ONE of the following is the drug of choice to reduce the synovial inflammation and prevent joint damage? A. Fish oil B. Naproxen C. Methotrexate D. Sulfasalazine E. Hydroxychloroquine

Correct Answer Is A She was diagnosed as systemic lupus erythematous (SLE) and treated with topical ointments, sunscreen lotion, oral prednisolone (for autoimmune hemolytic anemia) and hydroxychloroquine. Lupus (Latin for wolf) derives its name from the classical butterfly rash which mimics ‘the bite or scratch of a wolf’. Malar rash (butterfly rash), seen in 46–65% of lupus patients, is highly specific for the diagnosis of SLE. The rash may be raised or flat, scaly, non-pruritic and characteristically spares the nasolabial fold (differentiates from dermatomyositis). Common differential diagnoses include melasma (gray brown patches, common in pregnancy), dermatomyositis, lupus pernio and lupus vulgaris (indurated and nodular lesions), pellagra (symmetric keratotic areas on face which always affects body also), seborrheic dermatitis (also on other areas of face), rosacea (erythema, papule or pustules and telengiectasias on nose and medial cheeks, associated ocular features) and sunburn (significant pruritus and temporal association with sun exposure.

A 25-year-old married lady presented with increased hair loss, painless oral ulcers and rash over the face. She also had arthralgias involving small and large joints with early morning stiffness for 8 months. Physical examination revealed nonscarring alopecia, fixed erythematous maculopapular rash with hyperpigmen- tation over the cheeks and nasal bridge sparing nasolabial fold (Figure 1A). Multiple oral ulcers were also noted over the hard palate (Figure 1B). Investigations were notable for autoimmune hemolytic anemia (direct coombs test positive), leucopenia, anti- nuclear antibody positivity by indirect immunofluorescence method (dilution 1:160, 2þ intensity, pattern homogenous) and low complement (C3 and C4). Discuss and what is your diagnosis? Figure 1. (A) Maculopapular rash over the malar area in the shape of a butterfly, which is sparing the nasolabial folds (marked by black lines) and (B) multiple oral ulcers on the hard palate (black arrows). A. Systemic lupus erythematosus B. Pellagra C. Lupus Pernio D. Dermatomyositis E. Melasma