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Question 1
Incorrect
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A 28 year old male arrives at the clinic complaining of fever, arthralgia and urethritis. On examination, the ankle is swollen and there is a pustular rash on the dorsal foot. What is the most likely diagnosis?
Your Answer: Reactive arthritis
Correct Answer: Disseminated gonorrhoea
Explanation:DGI presents as two syndromes: 1) a bacteremic form that includes a triad of tenosynovitis, dermatitis, and polyarthralgias without purulent arthritis and 2) a septic arthritis form characterized as a purulent arthritis without associated skin lesions. Many patients will have overlapping features of both syndromes. Time from infection to clinical manifestations may range from 1 day to 3 months. There is no travel history and the rash of Lyme disease is not purulent. Reactive arthritis presents with conjunctivitis, urethritis and arthritis usually with a red hot tender and swollen joint.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 2
Incorrect
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A 42 year old obese man complains of a painful swollen ankle. The pain has worsened over the past 2 weeks. He is a diabetic and gives a history of recent alcohol consumption. Joint aspirate shows rhomboid crystals with numerous neutrophils. Radiological examination shows evidence of chondrocalcinosis. Which of the following is the most likely diagnosis?
Your Answer: Gouty arthritis
Correct Answer: Pseudogout
Explanation:Pseudogout is a paroxysmal joint inflammation due to calcium pyrophosphate crystal deposition (calcium pyrophosphate dihydrate). Aetiology includes mostly idiopathic (primary form) and secondary form occurring as a result of joint trauma, familial chondrocalcinosis, hyperparathyroidism, hemochromatosis, gout, hypophosphatemia. Clinical presentation: Often asymptomatic. Acute (pseudogout attack): monoarthritis (rarely oligoarthritis), mostly affecting the knees and other large joints (e.g., hips, wrists, and ankles). It may become chronic (can affect multiple joints). Osteoarthritis with CPPD (most common form of symptomatic CPPD): progressive joint degeneration with episodes of acute inflammatory arthritis typical of pseudogout attacks. Arthrocentesis should be performed, especially in acute cases. Polarized light microscopy: detection of rhomboid-shaped, positively birefringent CPPD crystals. Synovial fluid findings: 10,000-50,000 WBCs/μL with > 90% neutrophils. X-ray findings: cartilage calcification of the affected joint (chondrocalcinosis). Fibrocartilage (meniscus, annulus fibrosus of intervertebral disc) and hyaline cartilage (joint cartilage) may be affected.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 3
Correct
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High titre of which of the following antibodies is not considered diagnostic for the mentioned disease?
Your Answer: Rheumatoid factor in rheumatoid arthritis (RA)
Explanation:Diagnosis of RA is mainly based on clinical features (e.g., morning stiffness, symmetrical joint swelling) and laboratory tests (e.g., anti-CCP). Rheumatoid factor is not very specific for this disease and hence has low reliability. X-ray findings (e.g., soft tissue swelling or joint space narrowing) occur late in the disease and are therefore not typically used for diagnosis.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 4
Correct
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Drug-induced lupus erythematosus most often occurs after taking which of the following drugs?
Your Answer: Procainamide
Explanation:Many drugs are responsible for causing drug induced lupus. However, it is most commonly associated with hydralazine, procainamide and quinidine.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 5
Correct
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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:
- Connective Tissue
- Medicine
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Question 6
Correct
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A 28 year old man presents to the clinic with fatigue, exertional dyspnoea, abdominal discomfort, xerophthalmia and xerostomia. Examination reveals enlargement of the parotid glands bilaterally, hepatomegaly and peripheral motor neuropathy. Lab results are negative for RF, ANA, SS-A and SS-B antibodies. What is the most likely diagnosis?
Your Answer: Diffuse infiltrative lymphocytic syndrome (DILS)
Explanation:The Diffuse Infiltrative Lymphocytosis Syndrome (DILS) is a rare multisystemic syndrome described in HIV-infected patients. It is characterised by CD8(+) T-cell lymphocytosis associated with a CD8(+) T-cell infiltration of multiple organs. DILS is usually seen in uncontrolled or untreated HIV infection but can also manifest itself independently of CD4(+) T-cell counts. The syndrome may present as a Sjögren-like disease that generally associates sicca signs with bilateral parotiditis, lymphadenopathy, and extra glandular organ involvement. The latter may affect the lungs, nervous system, liver, kidneys, and digestive tract. Anomalies of the respiratory system are often identified as lymphocytic interstitial pneumonia. Facial nerve palsy, aseptic meningitis or polyneuropathy are among the more frequent neurological features. Hepatic lymphocytic infiltration, lymphocytic interstitial nephropathy and digestive tract lymphocytic infiltration account for more rarely noted complications. Sicca syndrome, organomegaly and/or organ dysfunction associated with polyclonal CD8(+) T-cell organ-infiltration are greatly suggestive of DILS in people living with HIV.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 7
Correct
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A 46 year old male, known with Rheumatoid Arthritis presents with a swollen and painful right ankle. No drug allergies are reported. On examination, the vitals are as follows: Temp: 38.8BP: 90/60 mmHgPulse: 110/min. Right ankle examination reveals tenderness, warmth, erythema and tense joint effusion. Blood samples including blood cultures are collected and fluid resuscitation is initiated. Which of the following interventions would be most appropriate for this man?
Your Answer: Administer i.v. flucloxacillin, and arrange joint aspiration urgently
Explanation:Although microscopic analysis of joint fluid aspirate and culture is the basis of septic arthritis diagnosis and should usually be collected before the administration of antibiotics, the patient is showing signs of septic shock. A delay in antibiotics might lead to worsening of symptoms so they are administered first.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 8
Incorrect
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A 45 year old man presents with fever, malaise, weight loss and myalgias that have been occurring for a month. You suspect polyarteritis nodosa and arrange for some lab investigations. Which of the following abnormality would most likely be present?
Your Answer: Positive ANCA
Correct Answer: Elevated creatinine
Explanation:People with polyarteritis nodosa often exhibit anaemia of chronic disease. Leucocytosis and eosinophilia may also be present. ANCA is only rarely positive. As polyarteritis nodosa affects the kidneys as well, the creatinine is elevated in most cases.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 9
Correct
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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: normalCXR: patchy airspace shadows bilaterallyWhat 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:
- Connective Tissue
- Medicine
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Question 10
Incorrect
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A 22 year old man is being evaluated for chronic lower backache. Which of the following would most strongly point towards the diagnosis of ankylosing spondylitis?
Your Answer: Accentuated lumbar lordosis
Correct Answer: Reduced lateral flexion of the lumbar spine
Explanation:Ankylosing spondylitis (spondylarthritis) is a chronic inflammatory disease of the axial skeleton that leads to partial or even complete fusion and rigidity of the spine. Males are disproportionately affected and upwards of 90% of patients are positive for the HLA-B27 genotype, which predisposes to the disease. The most characteristic early finding is pain and stiffness in the neck and lower back, caused by inflammation of the vertebral column and the sacroiliac joints. The pain typically improves with activity and is especially prominent at night. Other articular findings include tenderness to percussion and displacement of the sacroiliac joints (Mennell’s sign), as well as limited spine mobility, which can progress to restrictive pulmonary disease. The most common extra-articular manifestation is acute, unilateral anterior uveitis. Diagnosis is primarily based on symptoms and x-ray of the sacroiliac joints, with HLA-B27 testing and MRI reserved for inconclusive cases. There is no curative treatment, but regular physiotherapy can slow progression of the disease. Additionally, NSAIDs and/or tumour necrosis factor-α inhibitors may improve symptoms. In severe cases, surgery may be considered to improve quality of life. The spine adopts a bamboo shape, not lordosis. The pain usually improves as the day progresses. leg raise test causes pain in cases of meningitis etc not in this case.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 11
Correct
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A 68 year old man presents with acute symptoms of gout on his first metatarsophalangeal joint. Which option best explains the underlying mechanism of gout?
Your Answer: Decreased renal excretion of uric acid
Explanation:Primary gout is related more often to underexcretion of uric acid or overproduction.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 12
Correct
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A 32 year old complains of pain in her hands bilaterally. Which of the following symptoms would point towards an inflammatory joint disease such as rheumatoid arthritis?
Your Answer: Marked stiffness for more than an hour in the mornings
Explanation:In rheumatoid arthritis (RA), clinical symptoms of joint stiffness, pain, and functional disability are commonly most severe in the early morning. These symptoms closely follow the circadian rhythm of the pro-inflammatory cytokine, interleukin (IL)-6. In RA, the increase in nocturnal anti-inflammatory cortisol secretion is insufficient to suppress ongoing inflammation, resulting in the morning symptoms characteristic of RA. Established diagnostic criteria for RA include prolonged morning stiffness that could last up to an hour. Loss of joint mobility, pain, malaise and swelling of finger joints are features that are not specific to rheumatoid arthritis, and are found in many other conditions.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 13
Incorrect
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A 23 year old male presents with a history of lower back pain for the last one year. Presence of which of the following features most likely points towards ankylosing spondylitis?
Your Answer: Presence of HLA-B27 antigen on tissue typing
Correct Answer: Bilateral erosion of sacroiliac joints on X-ray
Explanation:Bilateral erosions of the sacroiliac joints on pelvic radiographs of patients with ankylosing spondylitis are an important feature of the modified New York classification criteria. Although HLA-B27 is commonly associated with AS, it can also be found in normal individuals. Back stiffness is worse in the morning and gets better as the day progresses. Tenderness and limited lumbar motion can be associated with other spine problems as well and is not characteristic of rheumatoid arthritis.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 14
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: The symptoms may be alleviated with long-term steroids
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:
- Connective Tissue
- Medicine
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Question 15
Correct
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A 28 year old female with a history of psoriatic arthritis would most likely have which of the following hand conditions?
Your Answer: Nail dystrophy
Explanation:Nail dystrophy (pitting of nails, onycholysis, subungual hyperkeratosis), dactylitis, sausage shaped fingers are most commonly seen with psoriatic arthropathy. There is asymmetric joint involvement most commonly distal interphalangeal joints. Uveitis and sacroiliitis may also occur. Arthritis mutilans may occur but is very rare. Cutaneous lesions may or may not develop. When they do, its usually much after the symptoms of arthritis.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 16
Incorrect
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A 30 year old female has been started on azathioprine after she was found to be intolerant of methotrexate. Routine blood monitoring shows the following values:Hb 7.9 g/dlPlt 97*109/l WBC 2.7*109/l Azathioprine toxicity will most likely to occur in the presence of which of the following?
Your Answer: Rifampicin
Correct Answer: Thiopurine methyltransferase deficiency
Explanation:Azathioprine therapy can cause acute myelosuppression. Toxicity is in part caused by the incorporation of azathioprine-derived 6-thioguanine nucleotides (6-TGN) into deoxyribonucleic acid (DNA). The enzyme thiopurine methyltransferase (TPMT) plays an important role in azathioprine catabolism.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 17
Correct
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A 35 year old female, known case of anti phospholipid syndrome, arrives at the clinic due to a swollen and painful left leg. Doppler ultrasonography confirms the diagnosis of a deep vein thrombosis. She was previously diagnosed with DVT 4 months back and was on warfarin therapy (target INR 2-3) when it occurred. How should her anticoagulation be managed?
Your Answer: Life-long warfarin, increase target INR to 3 - 4
Explanation:If the INR in the range of 2-3 has still resulted in thrombosis, the target INR is increased to 3-4. However, because the risk of bleeding increases as the INR rises, the INR is closely monitored and adjustments are made as needed to maintain the INR within the target range.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 18
Incorrect
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A 43 year old man with hemochromatosis presents with a painful and swollen right knee. X-ray shows extensive chondrocalcinosis but no fracture. Given the most likely diagnosis, which of the following would be present in the joint fluid aspirate?
Your Answer: Negatively birefringent calcium carbonate crystals
Correct Answer: Positively birefringent rhomboid-shaped crystals
Explanation:Pseudogout is a paroxysmal joint inflammation due to calcium pyrophosphate crystal deposition (calcium pyrophosphate dihydrate). Arthrocentesis should be performed, especially in acute cases. Polarized light microscopy: detection of rhomboid-shaped, positively birefringent CPPD crystals. Synovial fluid findings: 10,000-50,000 WBCs/μL with > 90% neutrophils. X-ray findings: cartilage calcification of the affected joint (chondrocalcinosis). Fibrocartilage (meniscus, annulus fibrosus of intervertebral disc) and hyaline cartilage (joint cartilage) may be affected.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 19
Incorrect
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An 80 year old female, previously diagnosed with polymyalgia rheumatica 18 months back, presents to the outpatient clinic with bilateral shoulder stiffness and generalized myalgia. The ESR was found to be 60mm/1st hour at the time of presentation. Prednisolone therapy was initiated at a daily dose of 15 mg along with Calcium and Vitamin supplementation. She reported resolution of her symptoms in one week. However the symptoms relapsed when the prednisolone dose was reduced below the current dose of 12.5 mg daily. How should she be ideally managed?
Your Answer: Increase the dose of prednisolone and add a bisphosphonate
Correct Answer: Continue the current dose of prednisolone and start methotrexate
Explanation:Polymyalgia rheumatica, a syndrome characterized by proximal muscle pain and stiffness in older persons, generally is treated with prednisone. Dosages of 15 to 25 mg of prednisone per day can reduce inflammation considerably, although many patients relapse when therapy is tapered. Long-term (18 to 36 months) steroid treatment has been recommended by several studies, but this can result in multiple side effects, including osteoporosis, hypertension, cataracts, and hyperglycaemia. Methotrexate has been used to reduce inflammation in rheumatoid arthritis, systemic vasculitis, and giant cell arteritis, and in some studies has been combined with prednisone to treat polymyalgia rheumatica, decreasing the duration of treatment.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 20
Incorrect
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A 78 year old woman presents to the clinic complaining of left sided temporal headaches and jaw claudication that has been going on for a month. A left temporal artery biopsy is negative. Results show:Hb: 130g/lPlatelets: 359*109/lWBC: 10*109/lCRP: 89mg/lWhich of the following options would be the next best step in the management of this patient?
Your Answer: CT brain
Correct Answer: Commence prednisolone
Explanation:Temporal arteritis is a chronic large- and medium-sized vessel vasculitis that typically involves the temporal arteries. Classical symptoms include temporal headaches, jaw claudication, amaurosis fugax. Physical exam shows scalp tenderness, palpation of the temporal area may demonstrate an absent pulse, knot-like swelling and vision loss. Lab results reveal an increased erythematous sedimentation rate and C-reactive protein. Temporal artery biopsy confirms the diagnosis. Management approach: high-dose systemic corticosteroids should be promptly administered even before the diagnosis is established. Temporal artery biopsy confirms the diagnosis. Inability to manage this or administer glucocorticoids might lead to development of blindness.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 21
Correct
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A 60 year old male presents with one month history of generalized headache, fever and malaise. He also complains of scalp sensitivity while combing his hair. In order to confirm the diagnosis, which of the following would be the definite test?
Your Answer: Temporal artery biopsy
Explanation:Temporal arteritis is a chronic large- and medium-sized vessel vasculitis that typically involves the temporal arteries. Classical symptoms include temporal headaches, jaw claudication, amaurosis fugax. Physical exam shows scalp tenderness, palpation of the temporal area may demonstrate an absent pulse, knot-like swelling, and vision loss. Lab results reveal an increased erythematous sedimentation rate and C-reactive protein. Temporal artery biopsy confirms the diagnosis. Management approach: high-dose systemic corticosteroids should be promptly administered even before the diagnosis is established. Temporal artery biopsy confirms the diagnosis. Inability to manage this or administer glucocorticoids might lead to development of blindness.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 22
Incorrect
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Which of the following options best describes rheumatoid factor?
Your Answer: IgM against the Fc portion of IgA
Correct Answer: IgM against the Fc portion of IgG
Explanation:Rheumatoid factor is an IgM antibody that targets the Fc portion of IgG.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 23
Incorrect
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Which of the following features occurs very less frequently with drug induced lupus?
Your Answer: Pleurisy
Correct Answer: Glomerulonephritis
Explanation:Drug induced lupus typically presents with pulmonary involvement and no renal or neurological involvement. Hence glomerulonephritis would be highly unlikely in this case. Rash and arthralgias are classic presentations. Pleurisy can be present as pulmonary involvement may occur with DILE.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 24
Incorrect
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A 39 year old woman with a history of rheumatoid arthritis has recently been switched from methotrexate to leflunomide. Monitoring of full blood count and LFTs has been carried out. Which of the following parameters should also be monitored in this case?
Your Answer: QT interval on ECG
Correct Answer: Blood pressure
Explanation:Blood pressure should be routinely measured as leflunomide may cause hypertension and thus an increase in BP. It doesn’t cause changes in blood sugar levels, peak expiratory flow rate or haematuria.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 25
Correct
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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:
- Connective Tissue
- Medicine
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Question 26
Incorrect
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A 56 year old woman presents to the clinic complaining of shoulder pain that she has been experiencing for the last 4 weeks. She does not remember getting injured previously. The pain worsens on movement especially when she is moving the arm quickly. At night, lying on the affected side is painful. Examination reveals no erythema or swelling. However, pain is felt on passive abduction between 60 to 120 degrees and she is unable to abduct the arm past 70-80 degrees. Flexion and extension are intact. What is the most likely diagnosis?
Your Answer: Adhesive capsulitis (frozen shoulder)
Correct Answer: Supraspinatus tendonitis
Explanation:Supraspinatus tendinopathy is a common and disabling condition that becomes more prevalent after middle age and is a common cause of pain in the shoulder. A predisposing factor is resistive overuse. This patient has the classic painful arc that is a sign of shoulder impingement characteristic of supraspinatus tendonitis.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 27
Incorrect
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A 26 year old male presents with right sided elbow and wrist pain and left sided knee and ankle pain that has persisted for about two weeks. He recently returned from a trip to Thailand that last for two weeks. The patient admits to having unprotected sex while on holiday. Examination reveals swelling and tenderness of tendons around joints but no inflammation of the joints. A vesiculopustular skin rash is also observed. What is the most likely cause?
Your Answer: Reactive arthritis
Correct Answer: Gonococcal arthritis
Explanation:Patients with disseminated gonococcal arthritis may present with dermatitis-arthritis syndrome (60%) of with localized septic arthritis. (40%). Arthritis-dermatitis syndrome includes the classic triad of dermatitis, tenosynovitis, and migratory polyarthritis. Gout usually involves a singe joint and does not cause vesicopustular skin rash. Reactive arthritis has ocular symptoms (conjunctivitis), urethritis, and arthritis. Fungal arthritis occurs rarely and it may occur after a surgical infection or fungal spread hematogenously. it presents with tender, red, hot and swollen joint with loss of range of motion.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 28
Correct
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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:
- Connective Tissue
- Medicine
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Question 29
Correct
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A 35 year old sales representative presents with severe pain going down her neck and right arm. She admits that this pain is worse on sitting and driving for long periods. Past history is significant for two previous road traffic accidents. Examination reveals weakness and sensory loss over C5/C6 nerve distribution. There is pain with neck movement and particularly extension. Which of the following investigations would be the most helpful in this case?
Your Answer: MRI scan of the cervical spinal cord
Explanation:Cervical radiculopathy is usually due to compression or injury to a nerve root by a herniated disc or degenerative changes. Levels C5 to T1 are the most commonly affected. It is usually, but not always, accompanied by cervical radicular pain, a sharp and shooting pain that travels from the neck and down the upper limb and may be severe. This needs to be differentiated from pain referred from the musculoskeletal (somatic) structures in the neck, which may be aching rather than sharp, and is more severe in the neck than in the upper limb. The neurological signs of cervical radiculopathy depend on the site of the lesion. The patient may have motor dysfunction, sensory deficits or alteration in tendon reflexes. While pain is a common presenting symptom, not all radiculopathies are painful (i.e. only motor deficits may be obvious). CT scanning cannot accurately demonstrate the commonest cause for cervical radiculopathy (disc herniation) without myelography, which requires hospital admission, lumbar puncture and the use of contrast. In patients with cervical radiculopathy, MRI is the imaging technique of choice for the detection of root compression by disc herniation and osteophytes. MRI allows the nerve roots to be directly visualised. Nerve conduction studies are also useful in determining the nerve roots that are involved.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 30
Correct
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A 16 year old previously well male presents with a 4 day history of fever, lethargy and a generalized macular rash. There is no significant previous medical history and the patient has not travelled abroad either. Vitals are as follows: Temp: 38.5BP: 125/75mmHgPulse: 100/min On auscultation the chest was clear and no heart murmur was heard. Examination also reveals a non blanching widespread macular rash over the chest and abdomen. There is swelling of interphalangeal joints of both hands and feet associated with mild tenderness. Lymph nodes are palpable over the supraclavicular, axillary and inguinal areas. Abdominal examination reveals palpable mass on both right and left hypochondrium. Lab results are given below:Haemoglobin (Hb) 13.5 g/dlWhite cell count (WCC) 14.0 × 109/lPlatelets 380 × 109/lSodium 145 mmol/lPotassium 4.8 mmol/lCreatinine 89 μmol/lRheumatoid factor NegativeAntinuclear antibody NegativeAnti-dsDNA NegativeASO titre Not detectedElectrocardiogram (ECG) Sinus rhythmWhat is the most likely underlying diagnosis?
Your Answer: Systemic Still’s disease
Explanation:People with Systemic Juvenile Idiopathic Arthritis (also known as Stills disease) can have recurrent fevers, a macular rash, joint pain, joint deformities, an enlarged liver and/or spleen, and can occasionally have polyserositis, lung involvement or pericardial effusions. Rheumatoid factor and antinuclear antibodies are usually negative. Treatment is with non-steroidal anti-inflammatory drugs (NSAIDs) and the prognosis is better than for adult rheumatoid arthritis.In pauciarticular Still’s disease, antinuclear antibodies are present. Large joints are affected and most patients develop classic features of seronegative spondylarthritis.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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SESSION STATS - PERFORMANCE PER SPECIALTY
