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Question 1
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An 18 year old girl presents with short history of marked, right hip pain and an associated limp. She has a history of acute lymphoblastic leukaemia for which she completed treatment for last six months.
Your Answer: Avascular necrosis of the femoral head
Explanation:Avascular necrosis (AVN) of the femoral head is a pathological process that results from disruption of the blood supply to the bone and occurs most commonly in the femoral epiphysis. Patients usually present with pain and limited joint motion. The mechanism involves impaired circulation to a specific area that ultimately becomes necrotic. AVN is most frequently associated with high doses of oral and intravenous corticosteroids and prolonged duration of therapy. Cancer patients receiving chemotherapy are usually also treated with corticosteroids.
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This question is part of the following fields:
- Rheumatology
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Question 2
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An 18 year old boy, thin with a tall stature, and a high arched palate arrives at the hospital with a spontaneous pneumothorax. He is accompanied by his brother who has a similar appearance. You suspect Marfan's Syndrome. The gene encoding which of the following proteins is defective in this condition?
Your Answer: Fibrillin-1
Explanation:A variety of proteins compose the structure of microfibrils, the most prominent of which are the two fibrillins. Fibrillin-1 a scaffolding protein is encoded by FBN1 on human chromosome 15q21 and fibrillin-2 is encoded by FBN2 on 5q23. Mutations in FBN1 produce Marfan syndrome, a pleiotropic autosomal dominant connective tissue disorder with prominent manifestations in the skeleton, eye and cardiovascular system. A number of conditions related to Marfan syndrome are also due to FBN1 mutations.
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This question is part of the following fields:
- Rheumatology
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Question 3
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A 22 year old nulliparous female presents with shortness of breath. She has a history of recurrent deep vein thrombosis. Complete blood count and clotting screen reveals the following results: Hb: 12.4 g/dl Plt: 137 WBC: 7.5*109/l PT: 14 secs APTT: 46 secs Which of the following would be the most likely diagnosis?
Your Answer: Antiphospholipid syndrome
Explanation:The combination of APTT and low platelets with recurrent DVTs make antiphospholipid syndrome the most likely diagnosis.
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This question is part of the following fields:
- Rheumatology
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Question 4
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A 28 year old man was rushed to the hospital after experiencing sudden onset chest pain while playing football. He has never felt such pain in the past. However, one of his uncles had a similar sudden discomfort at a young age and he passed away following a heart problem. The following vitals are recorded on examination: BP: 101/74 mmHg RR: 22 breaths/min PR: 87 beats/min Physical examination reveals abnormally long fingers and on asking the man to hold the opposite wrist, the thumb and little finger overlap each other. He is not taking any medication regularly and past medical/surgical history is not significant. He admits to smoking half pack of cigarettes/day for the last 10 years but denies abusing any illicit drugs. Which of the following explanation most likely explains the disease process in this man?
Your Answer: A defect of the glycoprotein structure which usually wraps around elastin
Explanation:Aortic dissection is defined as separation of the layers within the aortic wall. Tears in the intimal layer result in the propagation of dissection (proximally or distally) secondary to blood entering the intima-media space. This can be caused as a result of both congenital or acquired factors like chronic uncontrolled hypertension. This patient shows no sign of hypertension but his physical examination hints towards Marfan Syndrome. Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder affecting the microfibrils and elastin in connective tissue throughout the body. MFS is associated with pathological manifestations in the cardiovascular system (e.g., mitral valve prolapse, aortic aneurysm, and dissection), the musculoskeletal system (e.g., tall stature with disproportionately long extremities, joint hypermobility), and the eyes (e.g., subluxation of the lens of the eye). Decreased collagen production occurs in ageing, hydroxylation defects are present in vitamin C deficiency, copper deficiency affecting lysyl oxidase enzyme occurs in Menke’s disease.
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This question is part of the following fields:
- Rheumatology
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Question 5
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A 36 year old man arrives at the clinic complaining of pain and swelling of the left knee, ankles and right hallux. He has acute conjunctivitis, and dysuria. He suffered from an episode of gastroenteritis two weeks back. Clinical examination shows left Achilles tendonitis and right plantar fasciitis. Radiological examination reveals left sacroiliitis, with evidence of enthesitis, joint erosions and periostitis. HLA-B27 is positive. Which of the following is the most likely diagnosis?
Your Answer: Reiter’s syndrome
Explanation:Reactive arthritis, (formerly known as Reiter’s syndrome), is an autoimmune condition that occurs after a bacterial infection of the gastrointestinal or urinary tract. It is categorized as a seronegative spondylarthritis because of its association with HLA-B27. Reactive arthritis primarily affects young men and usually presents with musculoskeletal or extra‑articular symptoms. The characteristic triad consists of arthritis, conjunctivitis, and urethritis. The diagnosis is based on clinical features such as patient history and physical examination; there are no specific tests for reactive arthritis. Treatment is primarily symptomatic and consists of the administration of NSAIDs, as most patients recover spontaneously. extraarticular dermatologic manifestations include skin lesions of the glans resembling psoriasis (balanitis circinata); hyperkeratinisation of the palms and soles (keratoderma blenorrhagicum), oral ulcers.
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This question is part of the following fields:
- Rheumatology
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Question 6
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A 74 year old man presents with 12 kg weight loss and persistent back pain that is unrelated to activity for the past several months. Laboratory findings show : WCC: 6.7 x 109/l (5.4 neutrophils, 1.2 lymphocytes and 0.2 monocytes), Haemoglobin: 11.2 g/dL, Haematocrit: 33.3%, MCV: 88 fl, Platelet count: 89 x 109/l. The biochemistry shows: sodium 144 mmol/L, potassium 4.5 mmol/L, chloride 100 mmol/L, bicarbonate 26 mmol/L, urea 14 mmol/L, creatinine 90 μmol/L, glucose of 5.4 mmol/l. A CT scan of the spine reveals scattered 0.4 to 1.2 cm bright lesions in the vertebral bodies. Which of the following additional laboratory test findings is he most likely to have?
Your Answer: Serum prostate specific antigen of 35 microgram/l
Explanation:Old age, persistent backache, weight loss, and osteosclerotic lesions make prostatic adenocarcinoma the most likely diagnosis. The sequelae include severe pain, pathological fractures, hypercalcemia and cord compression. Prostatic adenocarcinoma is detected by elevated levels of prostate specific antigen. Positive serology for borrelia burgdorferi would hint at Lyme disease which does not cause osteosclerotic bone lesions, neither would Neisseria gonorrhoeae have such a presentation.
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This question is part of the following fields:
- Rheumatology
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Question 7
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A 55 year old female presents with progressive dyspnoea, dry cough and fever. She started methotrexate therapy six weeks ago. The current regimen includes methotrexate 15 mg/ week, folic acid 5 mg/day and aspirin 75 mg/day. There is no history of any other chronic illness. Vitals are as follows: Temp: 37.8C, pulse: 100 beats/min, BP: 110/80mmHg and SaO2: 90% on air. Examination reveals synovitis in both wrists, and metacarpophalangeal joints. On auscultation, there are scattered crepitations. Blood test reports are given below: Haemoglobin: 13.1g/dl (13.0 – 18.0 g/dL), WBC: 8.2 x109/l (4 – 11 x 109/l), Neutrophils: 5.1×109/l (1.5 – 7 x 109/l), Platelets: 280 x109/l (150 – 400 x 109/L), ESR: 48 mm/hr (0 – 30 mm/1st hr), Urea, electrolytes and creatinine: normal. CXR: patchy airspace shadows bilaterally What is the most likely diagnosis?
Your Answer: Methotrexate pneumonitis
Explanation:Pneumonitis is a serious and unpredictable side-effect of treatment with methotrexate (MTX) that may become life-threatening. The typical clinical symptoms include progressive shortness of breath and cough, often associated with fever. Hypoxaemia and tachypnoea are always present and crackles are frequently audible. Chest radiography reveals a diffuse interstitial or mixed interstitial and alveolar infiltrate, with a predilection for the lower lung fields. Pulmonary function tests show a restrictive pattern with diminished diffusion capacity. Lung biopsy reveals cellular interstitial infiltrates, granulomas or a diffuse alveolar damage pattern accompanied by perivascular inflammation. Most patients present in the first few months of starting methotrexate. It is important that all patients receiving methotrexate be educated concerning this potential adverse reaction and instructed to contact their physicians should significant new pulmonary symptoms develop while undergoing therapy. If methotrexate pneumonitis is suspected, methotrexate should be discontinued, supportive measures instituted and careful examination for different causes of respiratory distress conducted. This may be treated with corticosteroids once underlying infection has been excluded.
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This question is part of the following fields:
- Rheumatology
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Question 8
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A 64 year old female presents with sudden onset pain, swelling and stiffness in her right knee. Her medical history is significant for osteoarthritis affecting her hand joints and diet controlled diabetes mellitus. On examination, the right knee is swollen, erythematous and tender. Which of the following tests would be most useful in the diagnosis of this case?
Your Answer: Aspiration and examination of the synovial fluid
Explanation:Arthrocentesis should usually be done when there is a suspicion of pseudogout or septic arthritis as in this case which leads to an early diagnosis and prompt treatment. Polarized microscopy demonstrates weakly positively birefringent rhomboid crystals which are blue when parallel to light and yellow when perpendicular to light. Elevated serum uric acid levels that cause gout are usually found after large consumption of alcohol or meat, or post surgery. Autoimmune diseases like SLE, RA etc require an autoimmune screen.
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This question is part of the following fields:
- Rheumatology
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Question 9
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A 56 year old obese female presents due to moderate pain in her finger joints which occurs mostly at the end of the day which gets better with rest. There is also some associated swelling. On examination, there is enlargement of her distal interphalangeal joints and tenderness to palpation. Which of the following is the most likely diagnosis?
Your Answer: Osteoarthritis
Explanation:Osteoarthritis (OA) is a disabling joint disease characterized by a noninflammatory degeneration of the joint complex (articular cartilage, subchondral bone, and synovium) that occurs with old age or from overuse. It mainly affects the weight-bearing and high-use joints, such as the hip, knee, hands, and vertebrae. Despite the widespread view that OA is a condition caused exclusively by degenerative “wear and tear” of the joints, newer research indicates a significant heterogeneity of causation, including pre-existing peculiarities of joint anatomy, genetics, local inflammation, mechanical forces, and biochemical processes that are affected by proinflammatory mediators and proteases. Major risk factors include advanced age, obesity, previous injuries, and asymmetrically stressed joints. In early-stage osteoarthritis, patients may complain of reduced range of motion, joint-stiffness, and pain that is aggravated with heavy use. As the disease advances, nagging pain may also occur during the night or at rest. Diagnosis is predominantly based on clinical and radiological findings. Classic radiographic features of OA do not necessarily correlate with clinical symptoms and appearance. RA involves proximal interphalangeal joints not distal. Gout occurs as a result of overproduction or underexcretion of uric acid and frequently involves first toe. Fibromyalgia is a disorder characterized by widespread musculoskeletal pain accompanied by fatigue, sleep, memory and mood issues.
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This question is part of the following fields:
- Rheumatology
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Question 10
Incorrect
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A 56 year old woman taking procainamide develops drug induced erythematosus. Which of the following features is the most characteristic of this condition?
Your Answer: It does not occur with isoniazid
Correct Answer: It may occur with chlorpromazine
Explanation:Drug induced lupus is usually positive for antinuclear and antihistone antibodies, typically without renal or neurologic involvement. However, pulmonary involvement is common. Drugs that can induce lupus include isoniazid, hydralazine, procainamide, chlorpromazine, and other anticonvulsants.
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This question is part of the following fields:
- Rheumatology
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