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Question 1
Incorrect
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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: Acute rheumatic fever
Correct 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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Question 2
Correct
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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: 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 3
Correct
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Which of the following treatments would you advise for a young, 21 year old student who has ankylosing spondylitis and complains of worsening back pain and morning stiffness?
Your Answer: Oral NSAIDs
Explanation:NSAIDs are considered as the first line of treatment for managing pain and stiffness associated with ankylosing spondylitis. Other useful medications include TNF-alpha inhibitors. Other drugs like paracetamol, colchicine, and steroids are not routinely used. Bilateral total hip replacement might be indicated in advanced disease contrary to complicated spinal surgery.
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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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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: 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 5
Incorrect
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A 28 year old female presents to the rheumatology clinic for review. She has a history of adult onset Still's disease and complains of joint pains and persistent skin rash despite undergoing treatment with methotrexate and etanercept. On examination, an erythematous macular rash and active synovitis is noted. CRP is high (95 mg/dl). Which of the following interventions would be most appropriate?
Your Answer: Rituximab
Correct Answer: Anakinra
Explanation:Adult onset Still’s disease (AOSD) is an uncommon systemic inflammatory disease on the clinical spectrum of autoinflammatory disorders. Its presentation and clinical course may result in several well-differentiated phenotypes: from a systemic and highly symptomatic pattern to a chronic articular pattern. Overproduction of numerous pro-inflammatory cytokines is observed in AOSD. Anakinra, a human interleukin (IL)-1R antagonist, has recently been approved in the treatment of AOSD. Denosumab is a RANK ligand inhibitor used in the treatment of osteoporosis. Mepolizumab is used in the treatment of severe asthma with elevated eosinophils. There is some evidence to support the use of tocilizumab, an anti-IL6 monoclonal antibody in adult onset Still’s, but there is stronger data favouring the use of tocilizumab in the treatment of rheumatoid arthritis. Rituximab is usually reserved for patients who are unresponsive to anakinra.
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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 woman presents with painful genital ulceration. She reports that these attacks have been recurrent over the past 4 years. She has been treated previously with oral acyclovir but this has had little effect on the duration of her symptoms. Over the past year, she has noticed almost weekly attacks of mouth ulcers that heal slowly. Past medical history is significant for treatment of thrombophlebitis two years ago. Which of the following is the most likely diagnosis?
Your Answer: Behcet's syndrome
Explanation:Behçet disease is a rare vasculitic disorder that is characterized by a triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis. The systemic manifestations can be variable. Ocular disease has the greatest morbidity, followed by vascular disease generally from active vasculitis. Cutaneous manifestations can occur in up 75% of patients with Behcet disease and can range from acneiform lesions, to nodules and erythema nodosum. GI manifestations can be severe. Differentiating Behçet disease from active inflammatory bowel disease can be clinically difficult. Herpes would have ideally responded to acyclovir. Sarcoidosis does not have genital and oral ulcerations.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 7
Incorrect
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A 24 year old male, known case of hereditary angioneurotic oedema presents with recurrent fever and arthralgia which is accompanied by a rash on face and upper chest. These attacks have been refractory to treatment and have occurred recurrently requiring adrenaline on several occasions. Lab results reveal persistently reduced C4 levels. Which of the following is most likely causing his current symptoms?
Your Answer: Dermatomyositis
Correct Answer: Systemic lupus erythematosus
Explanation:Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A genetic defect of C1 inhibitor produces hereditary angioedema, which is usually presented with cutaneous painless oedema, but oedema of the genital area, gastrointestinal and laryngeal tracts have also been reported. In lupus patients, angioedema may be the result of an acquired type of C1 inhibitor deficiency, most probably due to antibody formation directed against the C1 inhibitor molecule.
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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 60 year old male presents with thickened patches of skin over his knuckles and extensor surfaces that are consistent with Gottron's papules. Results reveal an elevated creatine kinase. Diagnosis of dermatomyositis is suspected. Which of the following autoantibody is most specific for this condition?
Your Answer: Anti-scl-70 antibodies
Correct Answer: Anti-Mi-2 antibodies
Explanation:Anti–Mi-2 antibodies are highly specific for dermatomyositis, but sensitivity is low; only 25% of patients with dermatomyositis demonstrate these antibodies. A positive antinuclear antibody (ANA) finding is common in patients with dermatomyositis, but is not necessary for diagnosis. Anti-Jo-1 antibodies are mostly associated with polymyositis. Anti Scl-70 antibodies and anti centromere antibodies are most commonly found in systemic scleroderma.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 9
Incorrect
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A 42 year old female with a history of SLE presents with an exacerbation of wrist pain. Which of the following markers would be the most suitable for monitoring disease activity?
Your Answer: C-reactive protein
Correct Answer: Anti-dsDNA titres
Explanation:A high level of anti-dsDNA in the blood is strongly associated with lupus and is often significantly increased during or just prior to a flare-up. When the anti-dsDNA is positive and the person tested has other clinical signs and symptoms associated with lupus, it means that the person tested likely has lupus. This is especially true if an anti-Sm test is also positive.In the evaluation of someone with lupus nephritis, a high level (titre) of anti-dsDNA is generally associated with ongoing inflammation and damage to the kidneys.A very low level of anti-dsDNA is considered negative but does not exclude a diagnosis of lupus. Only about 65-85% of those with lupus will have anti-dsDNA.Low to moderate levels of the autoantibody may be seen with other autoimmune disorders, such as Sjögren syndrome and mixed connective tissue disease (MCTD).
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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 72 year old retired fisherman presents with weakness of shoulders and hips over the last four months. Finger flexion is also weak but the extension is normal. There has been some difficulty swallowing liquids. Past medical history is not significant except for sexually transmitted disease that he caught some 40 years ago in South Pacific and got treated with antibiotics. He smokes and drinks one or two tots of rum at the weekend. Creatine kinase level is 125. Which of the following investigations is most significant in establishing a diagnosis?
Your Answer:
Correct Answer: Muscle biopsy with electron microscopy
Explanation:Inclusion body myositis (IBM) is a progressive muscle disorder characterized by muscle inflammation, weakness, and atrophy (wasting). It is a type of inflammatory myopathy. IBM develops in adulthood, usually after age 50. The symptoms and rate of progression vary from person to person. The most common symptoms include progressive weakness of the legs, arms, fingers, and wrists. Some people also have weakness of the facial muscles (especially muscles controlling eye closure), or difficulty swallowing (dysphagia). Muscle cramping and pain are uncommon, but have been reported in some people. The underlying cause of IBM is poorly understood and likely involves the interaction of genetic, immune-related, and environmental factors. Some people may have a genetic predisposition to developing IBM, but the condition itself typically is not inherited. Elevated creatine kinase (CK) levels in the blood (at most ,10 times normal) are typical in IBM. Muscle biopsy may display several common findings including; inflammatory cells invading muscle cells, vacuolar degeneration, inclusions or plaques of abnormal proteins.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 11
Incorrect
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A 34 year old female presents to the clinic with skin tightness. On examination she has sclerodactyly, thickened skin of the shoulders and bi-basal crepitations. Her HRCT chest shows ground glass changes. Raynaud phenomenon is suspected and she is started on a monthly dose of IV cyclophosphamide (1 gm/month) for 6 months and a daily dose of 10 mg of oral prednisolone. However, she returned over a period of few weeks after developing exertional dyspnoea, pedal oedema and feeling unwell. On examination, JVP is raised, there is marked pedal oedema and bi basal crepitations on chest auscultation. Urine dipstick shows haematuria (++) and proteinuria (++). What in your opinion is the most likely cause of her deteriorating renal function?
Your Answer:
Correct Answer: Scleroderma renal crisis
Explanation:Scleroderma renal crisis (SRC) is a rare but severe complication in patients with systemic sclerosis (SSc). It is characterized by malignant hypertension, microangiopathic haemolytic anaemia with schistocytes and oligo/anuric acute renal failure. SRC occurs in 5% of patients with systemic scleroderma, particularly in the first years of disease evolution and in the diffuse form. Patients may develop symptoms of fluid overload.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 12
Incorrect
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An 82 year old man arrives at the clinic with painful right upper arm that he has been experiencing for the last few months. The pain is gradually worsening and often wakes him up at night. He is a known case of Paget's disease involving his lumbar spine and pelvis and is currently taking oral bisphosphonates. On examination, shoulder movements are intact. Which of the following is the most likely cause of his arm pain?
Your Answer:
Correct Answer: Osteosarcoma
Explanation:Paget’s accelerates the remodelling process with old bone breaking down more quickly which disrupts the normal growth process. New bone development eventually adjusts to the faster pace and speeds up on its own. The pace is too fast for healthy bone growth, and the regrowth ends up softer and weaker than normal. The effect of this accelerated process causes bone pain, fractures, and deformities. Since osteosarcoma is a type of bone cancer linked to abnormal bone growth, this makes it a rare but possible complication of Paget’s disease although the chances of developing osteosarcoma are minimal. Fractures are painful and restrict movement whereas this patients pain is gradually developing. Pain associated with osteoarthritis is usually mild to moderate and worsens as the day progresses.
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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 68 year old male, previously diagnosed with osteoarthritis presents to your clinic with acute on chronic pain in his big toe. Past history is significant for hypertension for which he takes Bendroflumethiazide 5mg daily. Examination reveals an erythematous, red hot metatarsophalangeal joint that has a knobbly appearance. X-ray shows punched out bony cysts. Which of the following would be the most appropriate long term management for this patient?
Your Answer:
Correct Answer: Stop Bendroflumethiazide and substitute a calcium antagonist
Explanation:The most likely diagnosis is chronic tophaceous gout, which is classically associated with chronic renal impairment or long term diuretic therapy. There is tophus formation due to urate crystal deposition in and around the joint. These tophi can also form in the bones and soft tissues. Renal manifestations with uric acid include nephrolithiasis and uric acid nephropathy. Whenever there is an acute on chronic attack of gout, the inciting cause must be established and in case of diuretic use, they should be immediately replaced with another medication. Allopurinol is never started during an acute episode. it is first allowed to settle before administration of allopurinol. Although dietary restriction must be observed in people with a propensity of gout, this scenario clearly presents diuretics as the cause.
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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 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:
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 15
Incorrect
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A 52 year old shopkeeper presents with pain in her hands. Examination reveals plaques on the extensor surfaces of her upper limbs and a telescoping deformity of both index fingers. Nails show pitting and horizontal ridging. The patient is most likely suffering from which of the following?
Your Answer:
Correct Answer: Arthritis mutilans
Explanation:Arthritis mutilans is a rare (occurs in only 5% of the patients) and extremely severe form psoriatic arthritis characterized by resorption of bones and the consequent collapse of soft tissue. When this affects the hands, it can cause a phenomenon sometimes referred to as ‘telescoping fingers.’ The associated nail changes are also characteristic 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 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:
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 17
Incorrect
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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/dlhaematocrit: 33.3%MCV: 88 flPlatelet count: 89 x 109/l.The biochemistry shows:sodium 144 mmol/lpotassium 4.5 mmol/lchloride 100 mmol/lbicarbonate 26 mmol/lurea 14 mmol/lcreatinine 90 μmol/la 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:
Correct 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:
- Connective Tissue
- Medicine
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Question 18
Incorrect
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A patient who has recently been diagnosed with SLE undergoes serum testing. A rise in which of the following antibodies would indicate severe systemic involvement?
Your Answer:
Correct Answer: Anti double-stranded DNA antibodies
Explanation:Anti ds-DNA antibodies are very specific for SLE and their presence most often indicates systemic spread of the disease. These antibodies are present in about 30 percent of the total cases of SLE.
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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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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:
Correct 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 20
Incorrect
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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:
Correct 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 21
Incorrect
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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:
Correct 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 22
Incorrect
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A 42 year old female presents with a funny bone sensation in her right elbow that is accompanied by numbness and tingling in the 4th and 5th digits. The symptoms are aggravated when the elbow is bent for a prolonged period. Which of the following explains the most likely diagnosis?
Your Answer:
Correct Answer: Cubital tunnel syndrome
Explanation:Cubital Tunnel Syndrome is a condition that involves pressure or stretching of the ulnar nerve (also known as the “funny bone” nerve), which can cause numbness or tingling in the 4th and 5th digit, pain in the forearm, and/or weakness in the hand. Epicondylitis of the elbow is a condition associated with repetitive forearm and elbow activities. Both lateral epicondylitis (commonly known as tennis elbow) and medial epicondylitis (commonly known as golfer’s elbow) are characterized by elbow pain during or following elbow flexion and extension. Radial tunnel syndrome is a set of symptoms that include fatigue or a dull, aching pain at the top of the forearm with use. Although less common, symptoms can also occur at the back of the hand or wrist.
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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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A 20 year old male student presents to the clinic with swelling of his face, hands and feet along with diffuse abdominal pain. He has a history of similar recurrent episodes since he was 10 years old, with each episode lasting 2-3 days. Examination reveals swelling on face, hands, feet but no sign of urticaria. Family history is significant for similar episodes in the mother who experienced these since childhood, and a brother who passed away following respiratory distress at age of 8 during one such episode. Which of the following tests would be the most helpful in reaching the diagnosis?
Your Answer:
Correct Answer: C1 esterase inhibitor
Explanation:Hereditary C1 inhibitor deficiency leads to recurrent angioedema without urticaria or pruritus. Physical triggers include dental work, surgery or intubation. Medical triggers include angiotensin-converting enzyme (ACE) inhibitor, tamoxifen, oestrogen-containing medications (e.g., hormone replacement therapy and oral contraceptives). It is diagnosed on the basis of low levels of C1 esterase inhibitor or elevated levels of dysfunctional C1 esterase inhibitor. C4 levels are low between attacks. IgE levels, eosinophils, skin prick tests and RASTs are helpful in other allergic conditions and asthma but not of use in this case.
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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 34 year old woman presents to the emergency department due to right sided weakness. Past history reveals a DVT following the birth of her daughter, and two miscarriages. Head CT confirms an ischaemic stroke in the territory of left middle cerebral artery. What would be the most likely finding on echocardiography?
Your Answer:
Correct Answer: Normal
Explanation:The patient most likely suffers from antiphospholipid syndrome. The clinical criteria consist of vascular thrombosis and pregnancy morbidity. Vascular thrombosis is defined as one or more clinical episodes of arterial, venous, or small-vessel thrombosis in any tissue or organ confirmed by findings from imaging studies, Doppler studies, or histopathology. ASD, VSDs would cause paradoxical emboli and stroke, however the recurrent pregnancy loss in this case is strongly suggestive of antiphospholipid syndrome. The ECG would be normal in most cases associated with anti phospholipid syndrome.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 25
Incorrect
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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 mmHgRR: 22 breaths/minPR: 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:
Correct 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:
- Connective Tissue
- Medicine
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Question 26
Incorrect
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A 47 year old woman presents with joint pains and a history of recurrent infections over the past few months. Labs reveal a positive rheumatoid factor and low white cell count. Given the likely diagnosis, which of the following features would be present in her case?
Your Answer:
Correct Answer: Splenomegaly
Explanation:Felty syndrome is a severe subtype of seropositive Rheumatoid arthritis. Clinical triad consists of arthritis, splenomegaly, and neutropenia (leads to an increased risk of recurrent bacterial infections). Other symptoms include skin ulcers of the lower limbs (indicating vasculitis), hepatomegaly, fever, and chest pain (indicating pleuritis or pericarditis). It is associated with increased risk of developing non-Hodgkin lymphoma.
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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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Which of the following drugs would be the safest to prescribe in a 22 year old man with seropositive rheumatoid arthritis who is planning to start a family?
Your Answer:
Correct Answer: Prednisolone
Explanation:Prednisolone although has many undesirable side effects it may be considered relatively safe compared to the drugs that are provided here. Prolonged treatment with sulphasalazine may depress semen quality and cause irreversible infertility. Methotrexate and leflunomide both inhibit purine/pyrimidine synthesis (the former by inhibiting folate metabolism) and are contraindicated in pregnancy or while trying to conceive. In males, a temporary or permanent decrease in sperm count may occur with cyclophosphamide. Because the recovery of fertility after cyclophosphamide therapy is variable, sperm banking should be considered before treatment is begun.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 28
Incorrect
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A 23 year old man presents with a painful right elbow and left Achilles tendon. He also gives history of dysuria, fever and conjunctivitis. Examination reveals macules and pustules on his hands. He has returned from a trip to Far East 3 weeks ago and admits to having unprotected sex. Which of the following is the most likely diagnosis?
Your Answer:
Correct Answer: Reactive arthritis
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. Other STDs including HIV, syphilis have different presentations. Psoriatic arthritis is not commonly associated with urethritis and conjunctivitis.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 29
Incorrect
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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:
Correct 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
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:
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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