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Question 1
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 2
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 3
Correct
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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: 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 4
Correct
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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: 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 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
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: Alcohol excess
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 7
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 8
Correct
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A 33 year old female presents with dyspnoea, myalgia, arthralgia and a skin rash. The presence of which of the following antibodies would be the most specific for SLE?
Your Answer: Anti-Sm
Explanation:Anti-Sm antibodies are essential for diagnosis of SLE, especially in anti-dsDNA-negative patients. ANA are also found in 95% of the patients with SLE but they may also occur with other conditions like Juvenile inflammatory arthritis, chronic activity hepatitis, and Sjogren’s syndrome. Anti-Ro, although also found with SLE are more characteristic of Sjogren Syndrome. RF is usually associated with rheumatoid arthritis and cANCA with Wegener’s granulomatosis, Churg Strauss, and microscopic polyangiitis.
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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 42 year old female presents with morning stiffness that usually takes an hour to settle and a one year history of intermittent pain and swelling of the small joints of her hands. Examination reveals symmetric soft tissue swelling over the PIP and MCP joints and rheumatoid nodules on the elbows. There is also an effusion of both wrists. Lab results are positive for rheumatoid factor. X-ray of the wrists and hands shows erosions and bony decalcification. NSAIDs are started and the patient is referred to a rheumatologist for consideration of DMARD. Previous history is significant for TB. Which of the following should be avoided?
Your Answer: Infliximab
Explanation:Anti-TNF-α therapy is effective for patients with arthritis but it can oftentimes lead to the reactivation of latent TB. Hence it should be used with great caution in patients with a past history of TB or current infection.
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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 31 year old female with systemic lupus erythematosus wants to know if she has any predisposing factors for the disease. Which of the following carries the greatest risk of developing SLE?
Your Answer: Positive HLA–DR2
Correct Answer: Monozygotic twin
Explanation:An overall concordance rate in monozygotic twins was documented to be 25% as compared to dizygotic twins with 3%. First degree relatives have a chance of around 3% of developing the disease. Caucasians show an increase frequency of HLA-B8. The Japanese lupus patients had a stronger association with HLA-DR2.
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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 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: 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 12
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: Plain X-rays
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 13
Incorrect
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High titre of which of the following antibodies is not considered diagnostic for the mentioned disease?
Your Answer: Anti-centromere antibodies in limited systemic sclerosis
Correct 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 14
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:
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 15
Incorrect
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A 23 year old male patient presents with urethritis for the last 2 weeks that has not responded to antibiotics. Lately he has developed an onset of new range of symptoms that are linked to his HLA B27 positivity. Which of the following signs is not related to Reiter's syndrome?
Your Answer:
Correct Answer: A mild fever with a generalised macular rash
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. Dermatologic manifestations include skin lesions of the glans resembling psoriasis (balanitis circinata); hyperkeratinisation of the palms and soles (keratoderma blenorrhagicum)
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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 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:
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 17
Incorrect
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A 32 year old woman presents with a painful elbow which she has been feeling for the past two weeks. Which of the following will be consistent with a diagnosis of tennis elbow?
Your Answer:
Correct Answer: Pain on wrist extension against resistance
Explanation:Lateral epicondylitis (tennis elbow) is an overuse injury of the hand and finger extensor tendons that originate in the lateral humeral epicondyle that occurs following repeated or excessive pronation/supination and extension of the wrist (e.g., in racquet sports). Clinical features include pain and tenderness over the lateral epicondyle and along extensor muscles and thickening of the tendons. The examiner holds the patient’s hand with the thumb placed over the lateral epicondyle – The patient makes a fist, supinates the forearm, deviates radially, and extends the fist against the examiner’s resistance which will result in pain over the lateral epicondyle. Conservative treatment includes rest, physiotherapy and orthotic braces. If this fails corticosteroids and lidocaine injections are employed. Surgery is indicated in patients with persistent symptoms despite 6 months of conservative treatment. Excision of abnormal tendon tissue; longitudinal incisions (tenotomies) in scarred and fibrotic areas to promote healing.
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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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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:
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 19
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 20
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 21
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 22
Incorrect
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Which of the following would be the most appropriate treatment (leaving out the current NICE guidelines) for a 29 year old man with erythrodermic psoriasis and arthritis mutilans involving several digits of both hands?
Your Answer:
Correct Answer: Etanercept
Explanation:TNF-alpha inhibitors are known to ameliorate the symptoms and disease activity of Arthritis mutilans (a rare and severe form of psoriatic arthritis), by disabling the cytokines that are involved in inflammation and joint destruction. From the mentioned choices, this would be the most effective option. Methotrexate is the most commonly used DMARD, followed by sulfasalazine used in mild to moderate forms of psoriatic arthritis but has not shown much efficacy in arthritis mutilans. Phototherapy, narrowband UVB light therapy can be very effective in clearing skin lesions.
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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 41 year old woman who has a history of SLE presents with a dry cough, dyspnoea and fever. She is being treated with a monthly dose of IV cyclophosphamide for Grade IV nephropathy. The last cyclophosphamide dose was 10 years ago. Lab investigations are as follows: WCC: 2.3 (lymphocyte count 0.7)Platelets: 81Hb: 10.5ESR: 56CRP: 43PO2: 7.2 kPa, PCO2: 3.6 kPa after walking out to the toilet.Chest X ray was unremarkable apart from some patchy pulmonary infiltration.What is the likely diagnosis?
Your Answer:
Correct Answer: Pneumocystis carinii pneumonia (PCP)
Explanation:Pneumocystis carinii pneumonia, is an opportunistic fungal lung infection occurring almost exclusively in immunocompromised individuals. In 50% of cases, PCP is the first manifestation of AIDS (acquired immune deficiency syndrome), but it may be caused by other immunodeficiency disorders. PCP should be suspected in patients with a history of progressive dyspnoea and a dry cough with resistance to standard antibiotic treatment. Signs that support this diagnosis include a CD4 count < 200/μL, an increased beta-D-glucan level, and diffuse bilateral infiltrates on chest x-ray. Management of PCP includes high-dose trimethoprim/sulfamethoxazole (TMP/SMX), treatment of the underlying immunodeficiency disorder, and steroids in the case of severe respiratory insufficiency. TB is less likely to be present in this case as ESR is relatively low and chest x-ray appeared normal.
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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 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:
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 25
Incorrect
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Osteopetrosis occurs as a result of a defect in:
Your Answer:
Correct Answer: Osteoclast function
Explanation:It is a metabolic bone disease caused by defective osteoclastic resorption of immature bone. Osteopetrosis is also known as marble bone disease. Osteoclasts are unable to adequately acidify bone matrix. Impaired bone resorption leads to overly dense bone that is more likely to fracture. It is usually treated with bone marrow transplant and high dose calcitriol.
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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 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 27
Incorrect
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Which of the following statements is the most characteristic of primary Raynaud's phenomena?
Your Answer:
Correct Answer: Fingers are symmetrically involved during an attack
Explanation:A typical attack may last less than an hour but can also persist for longer. In primary Raynaud’s, attacks are more likely symmetric, episodic, and without evidence of peripheral vascular disease. Patients more commonly have a negative ANA and normal inflammatory markers. There should be no evidence of tissue gangrene, digital pitting, or tissue injury in primary Raynaud’s. In contrast, patients with secondary Raynaud’s will describe attacks that are more frequent, painful, often asymmetric and may lead to digital ulcerations.
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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 31 year old female complains of bilateral proximal muscle weakness. There is marked blanching of fingers especially in response to cold weather. Lab results are as follows: Anti Jo-1: positive ANA: positiveCK: 2000 U/LESR: 60mm/hrEMG: myopathic changesPresence of which of the following signifies the worst prognosis?
Your Answer:
Correct Answer: Interstitial lung disease
Explanation:Polymyositis is an inflammatory disorder causing symmetrical, proximal muscle weakness. It is thought to be a T-cell mediated cytotoxic process directed against muscle fibres. It may be idiopathic or associated with connective tissue disorders. It may also be associated with malignancy for example small cell lung carcinoma.Dermatomyositis is a variant of the disease where skin manifestations are prominent, for example a purple (heliotrope) rash on the cheeks and eyelids.It typically affects middle-aged, female: male 3:1. Features include proximal muscle weakness +/- tenderness, Raynaud’s phenomenon, respiratory muscle weakness, interstitial lung disease: e.g. fibrosing alveolitis or organising pneumonia, dysphagia, dysphonia. Investigations: elevated creatine kinase, other muscle enzymes (lactate dehydrogenase (LD), aldolase, AST and ALT) are also elevated in 85-95% of patients, EMG, muscle biopsy. Anti-Jo-1 antibodies are seen in pattern of disease associated with lung involvement, Raynaud’s and fever. Interstitial lung disease plays a major role in morbidity and mortality in patients with polymyositis and is considered a major risk factor for premature death in patients with myositis.
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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 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:
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 30
Incorrect
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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:
Correct 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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SESSION STATS - PERFORMANCE PER SPECIALTY
