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Question 1
Correct
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Osteopetrosis occurs as a result of a defect in:
Your 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 2
Correct
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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: 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 3
Incorrect
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A 67 year old man reports weight loss. Labs show a raised alkaline phosphatase at 290 U/L (normal range 35-120). Plain radiographs reveal sclerotic lesions of the bone. Which of the following is the most likely cause of these findings?
Your Answer: Multiple myeloma
Correct Answer: Prostate cancer
Explanation:Osteoblastic (or sclerotic) bony metastases, characterized by deposition of new bone, present in prostate cancer, carcinoid, small cell lung cancer, Hodgkin lymphoma or medulloblastoma. The other cancers listed in the options are osteolytic.
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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 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: 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 5
Incorrect
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A 62 year old man arrives at the clinic with a history of cough and intermittent haemoptysis for the last 3 months. He has a 50 pack year smoking history and is currently waiting for bronchoscopy to assess a left lower lobe collapse. The patient also has a marked muscle weakness and wasting of proximal muscles of his shoulders and pelvic girdle. His wife states that lately he has been unable to eat solids. Which of the following statements would be true regarding this scenario?
Your Answer: He almost certainly has adenocarcinoma of the lung
Correct Answer: He may have a photosensitive facial rash
Explanation:The patient has presented with signs of small cell lung cancer. The associated proximal muscle weakness is most probably due to dermatomyositis which occurs as a paraneoplastic syndrome associated with lung carcinoma. In most cases, the first symptom is a distinctive skin rash on the face, eyelids, chest, nail cuticle areas, knuckles, knees or elbows. The rash is patchy and usually a bluish-purple colour. Corticosteroids are helpful in the management of the cutaneous changes and muscle weakness.
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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 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-Jo-1 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 7
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 8
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 9
Incorrect
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Which of the following statements is the most characteristic of primary Raynaud's phenomena?
Your Answer: Digital gangrene is a frequent complication
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 10
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: Impaired hydroxylation of lysine and proline amino acids on collagen
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 11
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 12
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 13
Correct
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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: 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 14
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: Atrial septal defect
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 15
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 16
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: Pain on extension of the elbow
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 17
Incorrect
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A 72 year old female, known with rheumatoid arthritis for last 17 years, presents with recurrent attacks of red eyes with a sensation of grittiness. Which of the following is most likely cause of the red eyes?
Your Answer: episcleritis
Correct Answer: keratoconjunctivitis sicca
Explanation:Rheumatoid arthritis is an inflammatory systemic disease associated with some extraarticular manifestations. Keratoconjunctivitis sicca, episcleritis, scleritis, corneal changes, and retinal vasculitis are the most common ocular complications among extraarticular manifestations of RA. The overall prevalence of keratoconjunctivitis sicca also known as dry eye syndrome among patients of RA is 21.2% and is the most common with sense of grittiness in the eyes.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 18
Correct
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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: 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 19
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 20
Correct
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A 56 year old obese female presents due to moderate pain in her finger joints which occurs mostly at the end of the day which gets better with rest. There is also some associated swelling. On examination, there is enlargement of her distal interphalangeal joints and tenderness to palpation. Which of the following is the most likely diagnosis?
Your Answer: Osteoarthritis
Explanation:Osteoarthritis (OA) is a disabling joint disease characterized by a noninflammatory degeneration of the joint complex (articular cartilage, subchondral bone, and synovium) that occurs with old age or from overuse. It mainly affects the weight-bearing and high-use joints, such as the hip, knee, hands, and vertebrae. Despite the widespread view that OA is a condition caused exclusively by degenerative “wear and tear” of the joints, newer research indicates a significant heterogeneity of causation, including pre-existing peculiarities of joint anatomy, genetics, local inflammation, mechanical forces, and biochemical processes that are affected by proinflammatory mediators and proteases. Major risk factors include advanced age, obesity, previous injuries, and asymmetrically stressed joints. In early-stage osteoarthritis, patients may complain of reduced range of motion, joint-stiffness, and pain that is aggravated with heavy use. As the disease advances, nagging pain may also occur during the night or at rest. Diagnosis is predominantly based on clinical and radiological findings. Classic radiographic features of OA do not necessarily correlate with clinical symptoms and appearance. RA involves proximal interphalangeal joints not distal. Gout occurs as a result of overproduction or underexcretion of uric acid and frequently involves first toe. Fibromyalgia is a disorder characterized by widespread musculoskeletal pain accompanied by fatigue, sleep, memory and mood issues.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 21
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 22
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: Continue the current dose of prednisolone
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 23
Incorrect
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Presence of which of the following indicates a worse prognosis in rheumatoid arthritis?
Your Answer: HLA DR2 allele
Correct Answer: Anti-CCP antibodies
Explanation:Rheumatoid arthritis is both common and chronic, with significant consequences for multiple organ systems. Anti-cyclic citrullinated peptide (anti-CCP) antibody testing is particularly useful in the diagnosis of rheumatoid arthritis, with high specificity, presence early in the disease process, and ability to identify patients who are likely to have severe disease and irreversible damage. However, its sensitivity is low, and a negative result does not exclude disease. Anti-CCP antibodies have not been found at a significant frequency in other diseases to date, and are more specific than rheumatoid factor for detecting rheumatoid arthritis. The other factors that are mentioned do not play a key prognostic role.
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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 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: Advise dietary restriction of rich foods
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 25
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: Pericarditis
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 26
Incorrect
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High titre of which of the following antibodies is not considered diagnostic for the mentioned disease?
Your Answer: Ads-DNA antibodies in systemic lupus erythematosus (SLE)
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 27
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: Osteonecrosis
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 28
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: Blood glucose
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 29
Correct
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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: 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 30
Incorrect
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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: Lymphoma
Correct 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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SESSION STATS - PERFORMANCE PER SPECIALTY
