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Question 1
Incorrect
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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: Arthritis mutilans
Correct 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 2
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 3
Correct
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An 18 year old girl presents with short history of marked, right hip pain and an associated limp. She has a history of acute lymphoblastic leukaemia for which she completed treatment for last six months.
Your Answer: Avascular necrosis of the femoral head
Explanation:Avascular necrosis (AVN) of the femoral head is a pathological process that results from disruption of the blood supply to the bone and occurs most commonly in the femoral epiphysis. Patients usually present with pain and limited joint motion. The mechanism involves impaired circulation to a specific area that ultimately becomes necrotic. AVN is most frequently associated with high doses of oral and intravenous corticosteroids and prolonged duration of therapy. Cancer patients receiving chemotherapy are usually also treated with corticosteroids.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 4
Correct
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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: 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 5
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 6
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: Lateral epicondylitis
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 7
Correct
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A 42 year old male arrives at the clinic due to cough and haemoptysis. Examination shows nasal mucosal ulceration. The doctor suspects Wegener's granulomatosis. Which anatomical area would be most commonly involved in this condition?
Your Answer: Lungs
Explanation:Granulomatosis with polyangiitis (GPA, previously known as Wegener’s granulomatosis) is a systemic vasculitis that affects both small and medium-sized vessels. Patients typically initially suffer from a limited form that may consist of constitutional symptoms and localized manifestations such as chronic sinusitis, rhinitis, otitis media, ocular conditions. In later stages, more serious manifestations may arise, including pulmonary complications and glomerulonephritis, although the skin, eyes, and heart may also be involved but these lesions are less common. Diagnosis is based on laboratory testing (positive for PR3-ANCA/c-ANCA), imaging, and biopsy of affected organs, which demonstrate necrotizing granulomatous inflammation. GPA is treated with immunosuppressive drugs, typically consisting of glucocorticoids combined with methotrexate, cyclophosphamide, or rituximab. Relapses are common and the following systems are affected: Lower respiratory tract (95% of cases), renal involvement (80% of cases), skin lesions (45% of cases), ocular involvement (45% of cases) and cardiac involvement (33% of cases).
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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 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: Ankylosing spondylitis
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 9
Correct
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A 16 year old previously well male presents with a 4 day history of fever, lethargy and a generalized macular rash. There is no significant previous medical history and the patient has not travelled abroad either. Vitals are as follows: Temp: 38.5BP: 125/75mmHgPulse: 100/min On auscultation the chest was clear and no heart murmur was heard. Examination also reveals a non blanching widespread macular rash over the chest and abdomen. There is swelling of interphalangeal joints of both hands and feet associated with mild tenderness. Lymph nodes are palpable over the supraclavicular, axillary and inguinal areas. Abdominal examination reveals palpable mass on both right and left hypochondrium. Lab results are given below:Haemoglobin (Hb) 13.5 g/dlWhite cell count (WCC) 14.0 × 109/lPlatelets 380 × 109/lSodium 145 mmol/lPotassium 4.8 mmol/lCreatinine 89 μmol/lRheumatoid factor NegativeAntinuclear antibody NegativeAnti-dsDNA NegativeASO titre Not detectedElectrocardiogram (ECG) Sinus rhythmWhat is the most likely underlying diagnosis?
Your Answer: Systemic Still’s disease
Explanation:People with Systemic Juvenile Idiopathic Arthritis (also known as Stills disease) can have recurrent fevers, a macular rash, joint pain, joint deformities, an enlarged liver and/or spleen, and can occasionally have polyserositis, lung involvement or pericardial effusions. Rheumatoid factor and antinuclear antibodies are usually negative. Treatment is with non-steroidal anti-inflammatory drugs (NSAIDs) and the prognosis is better than for adult rheumatoid arthritis.In pauciarticular Still’s disease, antinuclear antibodies are present. Large joints are affected and most patients develop classic features of seronegative spondylarthritis.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 10
Correct
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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 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 11
Incorrect
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A 43 year old man with hemochromatosis presents with a painful and swollen right knee. X-ray shows extensive chondrocalcinosis but no fracture. Given the most likely diagnosis, which of the following would be present in the joint fluid aspirate?
Your Answer: Negatively birefringent calcium carbonate crystals
Correct Answer: Positively birefringent rhomboid-shaped crystals
Explanation:Pseudogout is a paroxysmal joint inflammation due to calcium pyrophosphate crystal deposition (calcium pyrophosphate dihydrate). Arthrocentesis should be performed, especially in acute cases. Polarized light microscopy: detection of rhomboid-shaped, positively birefringent CPPD crystals. Synovial fluid findings: 10,000-50,000 WBCs/μL with > 90% neutrophils. X-ray findings: cartilage calcification of the affected joint (chondrocalcinosis). Fibrocartilage (meniscus, annulus fibrosus of intervertebral disc) and hyaline cartilage (joint cartilage) may be affected.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 12
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: HLA-DR2 tissue type
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 13
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: Cyclophosphamide
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 14
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 15
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 16
Correct
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A 46 year old male, known with Rheumatoid Arthritis presents with a swollen and painful right ankle. No drug allergies are reported. On examination, the vitals are as follows: Temp: 38.8BP: 90/60 mmHgPulse: 110/min. Right ankle examination reveals tenderness, warmth, erythema and tense joint effusion. Blood samples including blood cultures are collected and fluid resuscitation is initiated. Which of the following interventions would be most appropriate for this man?
Your Answer: Administer i.v. flucloxacillin, and arrange joint aspiration urgently
Explanation:Although microscopic analysis of joint fluid aspirate and culture is the basis of septic arthritis diagnosis and should usually be collected before the administration of antibiotics, the patient is showing signs of septic shock. A delay in antibiotics might lead to worsening of symptoms so they are administered first.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 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: keratitis
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
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: Methotrexate
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 19
Incorrect
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A 64 year old woman who is of Asian descent and is diabetic (controlled by diet) presents with generalized body aches and difficulty rising from sitting for the last few months. Her blood glucose levels are in the normal range. Lab examination reveals normal blood cell count, low serum phosphate, calcium at the lower range, and raised alkaline phosphatase levels. Radiological examination shows which of the following?
Your Answer: Osteolytic areas with bone destruction
Correct Answer: Linear areas of low density
Explanation:Osteomalacia is a condition due to defective mineralization of osteoid. Occurs as a result of Vitamin D deficiency secondary to poor dietary intake and sun exposure, malabsorption e.g., inflammatory bowel disease and gastrointestinal bypass surgery. Radiological findings include reduced bone mineral density (a non specific finding), inability to radiologically distinguish vertebral body trabeculae (the film appears poor quality), looser pseudo fractures, fissures, or narrow radiolucent lines (these are the characteristic findings). Osteolytic or punched out lesions may be seen with multiple myeloma and bony metastases. Areas of sclerosis may be observed with conditions like osteosclerosis and Paget disease. A Brodie abscess is a subacute osteomyelitis, which may persist for years before progressing to a chronic, frank osteomyelitis.
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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 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: Fast acetylator status
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 21
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 22
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: Positive serology for Borrelia burgdorferi
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 23
Incorrect
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Presence of which of the following indicates a worse prognosis in rheumatoid arthritis?
Your Answer: Female sex
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 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: Ventricular 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 25
Incorrect
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A 45 year old man, known case of hypothyroidism, presents with a 3 month history of pain and stiffness in the joints of his hands and feet. He also complains of dyspnoea occurring for the past 6 weeks. On examination there is a right pleural effusion and swollen hand joints. The clinician makes a diagnosis of rheumatoid arthritis. Which of the following is most characteristic of a rheumatoid pleural effusion?
Your Answer: It should be treated by decortication
Correct Answer: It has a low glucose level
Explanation:The typical “rheumatoid effusion” is a sterile exudative fluid with low pH (<7.3), low glucose (700 IU). It should initially be treated with NSAIDs. Decortication should be reserved in patients with thickened pleura who have symptomatic dyspnoea.
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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 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: Decrease in the production of 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 27
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: Tropoelastin
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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Question 28
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: Pustular lesions on the soles of the feet
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 29
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: Osteoarthritis
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 30
Incorrect
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A 32 year old male who has recently returned from a trip to Thailand presents with congestion of eyes and swelling of the knee joint. He completed a course of antibiotics for dysentery 4 weeks back. He experiences no dysuria and urine examination is normal. Which further information would be most useful in establishing a diagnosis?
Your Answer: Auscultation of the chest
Correct Answer: History and physical examination
Explanation:The patient most likely has reactive arthritis which is usually diagnosed on history and clinical examination. The classic triad of symptoms include conjunctivitis, urethritis and arthritis. Arthritis and conjunctivitis may occur 4-6 weeks after a gastrointestinal or genitourinary infection. Arthritis usually occurs acutely, mostly involving the lower limb and is asymmetrical. Blood culture, urine culture and arthrocentesis (joint aspiration) will not yield positive results.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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SESSION STATS - PERFORMANCE PER SPECIALTY
