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Question 1
Incorrect
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A 23 year old man presents with a painful right elbow and left Achilles tendon. He also gives history of dysuria, fever and conjunctivitis. Examination reveals macules and pustules on his hands. He has returned from a trip to Far East 3 weeks ago and admits to having unprotected sex. Which of the following is the most likely diagnosis?
Your Answer: Gonococcal arthritis
Correct Answer: Reactive arthritis
Explanation:Reactive arthritis, (formerly known as Reiter’s syndrome), is an autoimmune condition that occurs after a bacterial infection of the gastrointestinal or urinary tract. It is categorized as a seronegative spondylarthritis because of its association with HLA-B27. Reactive arthritis primarily affects young men and usually presents with musculoskeletal or extra‑articular symptoms. The characteristic triad consists of arthritis, conjunctivitis, and urethritis. The diagnosis is based on clinical features such as patient history and physical examination; there are no specific tests for reactive arthritis. Treatment is primarily symptomatic and consists of the administration of NSAIDs, as most patients recover spontaneously. extraarticular dermatologic manifestations include skin lesions of the glans resembling psoriasis (balanitis circinata); hyperkeratinisation of the palms and soles (keratoderma blenorrhagicum), oral ulcers. Other STDs including HIV, syphilis have different presentations. Psoriatic arthritis is not commonly associated with urethritis and conjunctivitis.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 2
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 3
Incorrect
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A 72 year old retired fisherman presents with weakness of shoulders and hips over the last four months. Finger flexion is also weak but the extension is normal. There has been some difficulty swallowing liquids. Past medical history is not significant except for sexually transmitted disease that he caught some 40 years ago in South Pacific and got treated with antibiotics. He smokes and drinks one or two tots of rum at the weekend. Creatine kinase level is 125. Which of the following investigations is most significant in establishing a diagnosis?
Your Answer: Anti Jo 1 antibody titres
Correct Answer: Muscle biopsy with electron microscopy
Explanation:Inclusion body myositis (IBM) is a progressive muscle disorder characterized by muscle inflammation, weakness, and atrophy (wasting). It is a type of inflammatory myopathy. IBM develops in adulthood, usually after age 50. The symptoms and rate of progression vary from person to person. The most common symptoms include progressive weakness of the legs, arms, fingers, and wrists. Some people also have weakness of the facial muscles (especially muscles controlling eye closure), or difficulty swallowing (dysphagia). Muscle cramping and pain are uncommon, but have been reported in some people. The underlying cause of IBM is poorly understood and likely involves the interaction of genetic, immune-related, and environmental factors. Some people may have a genetic predisposition to developing IBM, but the condition itself typically is not inherited. Elevated creatine kinase (CK) levels in the blood (at most ,10 times normal) are typical in IBM. Muscle biopsy may display several common findings including; inflammatory cells invading muscle cells, vacuolar degeneration, inclusions or plaques of abnormal proteins.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 4
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 5
Incorrect
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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: Methotrexate
Correct 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 6
Correct
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A 68 year old man presents with acute symptoms of gout on his first metatarsophalangeal joint. Which option best explains the underlying mechanism of gout?
Your Answer: Decreased renal excretion of uric acid
Explanation:Primary gout is related more often to underexcretion of uric acid or overproduction.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 7
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: Brodie’s abscess
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 8
Correct
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A 60 year old male presents with one month history of generalized headache, fever and malaise. He also complains of scalp sensitivity while combing his hair. In order to confirm the diagnosis, which of the following would be the definite test?
Your Answer: Temporal artery biopsy
Explanation:Temporal arteritis is a chronic large- and medium-sized vessel vasculitis that typically involves the temporal arteries. Classical symptoms include temporal headaches, jaw claudication, amaurosis fugax. Physical exam shows scalp tenderness, palpation of the temporal area may demonstrate an absent pulse, knot-like swelling, and vision loss. Lab results reveal an increased erythematous sedimentation rate and C-reactive protein. Temporal artery biopsy confirms the diagnosis. Management approach: high-dose systemic corticosteroids should be promptly administered even before the diagnosis is established. Temporal artery biopsy confirms the diagnosis. Inability to manage this or administer glucocorticoids might lead to development of blindness.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 9
Correct
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A 34 year old female presents to the clinic with skin tightness. On examination she has sclerodactyly, thickened skin of the shoulders and bi-basal crepitations. Her HRCT chest shows ground glass changes. Raynaud phenomenon is suspected and she is started on a monthly dose of IV cyclophosphamide (1 gm/month) for 6 months and a daily dose of 10 mg of oral prednisolone. However, she returned over a period of few weeks after developing exertional dyspnoea, pedal oedema and feeling unwell. On examination, JVP is raised, there is marked pedal oedema and bi basal crepitations on chest auscultation. Urine dipstick shows haematuria (++) and proteinuria (++). What in your opinion is the most likely cause of her deteriorating renal function?
Your Answer: Scleroderma renal crisis
Explanation:Scleroderma renal crisis (SRC) is a rare but severe complication in patients with systemic sclerosis (SSc). It is characterized by malignant hypertension, microangiopathic haemolytic anaemia with schistocytes and oligo/anuric acute renal failure. SRC occurs in 5% of patients with systemic scleroderma, particularly in the first years of disease evolution and in the diffuse form. Patients may develop symptoms of fluid overload.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 10
Incorrect
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A 78 year old woman presents to the clinic complaining of left sided temporal headaches and jaw claudication that has been going on for a month. A left temporal artery biopsy is negative. Results show:Hb: 130g/lPlatelets: 359*109/lWBC: 10*109/lCRP: 89mg/lWhich of the following options would be the next best step in the management of this patient?
Your Answer: Observation
Correct Answer: Commence prednisolone
Explanation:Temporal arteritis is a chronic large- and medium-sized vessel vasculitis that typically involves the temporal arteries. Classical symptoms include temporal headaches, jaw claudication, amaurosis fugax. Physical exam shows scalp tenderness, palpation of the temporal area may demonstrate an absent pulse, knot-like swelling and vision loss. Lab results reveal an increased erythematous sedimentation rate and C-reactive protein. Temporal artery biopsy confirms the diagnosis. Management approach: high-dose systemic corticosteroids should be promptly administered even before the diagnosis is established. Temporal artery biopsy confirms the diagnosis. Inability to manage this or administer glucocorticoids might lead to development of blindness.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 11
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 12
Correct
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A 23 year old male presents with a history of lower back pain for the last one year. Presence of which of the following features most likely points towards ankylosing spondylitis?
Your Answer: Bilateral erosion of sacroiliac joints on X-ray
Explanation:Bilateral erosions of the sacroiliac joints on pelvic radiographs of patients with ankylosing spondylitis are an important feature of the modified New York classification criteria. Although HLA-B27 is commonly associated with AS, it can also be found in normal individuals. Back stiffness is worse in the morning and gets better as the day progresses. Tenderness and limited lumbar motion can be associated with other spine problems as well and is not characteristic of rheumatoid arthritis.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 13
Incorrect
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A 32 year old female is diagnosed with SLE based on her complaints of polyarthralgia, mouth ulcers and ANA positivity. Labs reveal normal urinalysis, urea and electrolytes. ESR is 90mm in the first hour. How will you manage this patient?
Your Answer: Six monthly pulses of cyclophosphamide (2 mg/kg)
Correct Answer: Hydroxychloroquine 200 mg/day
Explanation:Hydroxychloroquine is used in the management of SLE as it prevents disease progression and has relatively mild side effects, for instance headache, nausea etc. Its use reduces the usage of corticosteroids. It is particularly effective when the disease is less severe and there is no organ involvement. Cyclophosphamide and prednisolone are indicated in cases of renal, neurological and lung involvement.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 14
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 15
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: Elastin
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 16
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 17
Correct
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A 43 year old female presents with an array of symptoms including weakness, lethargy, dysphagia, dry mouth, gritty sensation in her eyes and increased photosensitivity. In order to confirm the suspected diagnosis, which of the following tests should be performed?
Your Answer: Labial gland biopsy
Explanation:To confirm the diagnosis, especially in patients with negative anti-Ro or anti-La antibodies, labial gland biopsy is done. In performing a labial biopsy, the surgeon typically makes a shallow 1/2 inch wide incision on either side of the inner lip after numbing the area with a local anaesthetic. Schirmer’s test determines whether the eye produces enough tears to keep it moist. This test can be done for ocular symptoms of Sjogren syndrome but is of no diagnostic importance as it can be positive with many other diseases.
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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 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 19
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: Joint aspiration and culture
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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Question 20
Incorrect
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A 49 year old female presents to the clinic complaining of pain in her left elbow that is localized to the left lateral epicondyle. She has spent the weekend painting her house. A diagnosis of lateral epicondylitis is suspected. The pain would characteristically worsen on which of the following movements?
Your Answer: Pronation of the forearm with the elbow flexed
Correct Answer: Resisted wrist extension with the elbow extended
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, 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 results 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 21
Correct
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A 53 year old female, longstanding case of rheumatoid arthritis comes for a review. Which of the following features are commonly associated with her condition?
Your Answer: Proximal interphalangeal joint involvement in the hands
Explanation:Rheumatoid arthritis is a polyarthritis that results in symmetrical pain and swelling of the affected joints (also at rest). It particularly affects the metacarpophalangeal joints (MCPJs) and proximal interphalangeal joints (PIPJs), not the distal interphalangeal joints (DIPs). Ulcerative colitis and IBD are associated with seronegative arthritides, not RA. The condition can also cause various extra-articular manifestations such as ocular symptoms, rheumatoid nodules and pulmonary fibrosis. Scleritis, episcleritis and keratoconjunctivitis sicca are more common than uveitis. Early intervention with disease-modifying antirheumatic drugs (DMARDs) plays a decisive role in successful treatment.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 22
Correct
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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: 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 23
Incorrect
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A 28 year old male arrives at the clinic complaining of fever, arthralgia and urethritis. On examination, the ankle is swollen and there is a pustular rash on the dorsal foot. What is the most likely diagnosis?
Your Answer: Reactive arthritis
Correct Answer: Disseminated gonorrhoea
Explanation:DGI presents as two syndromes: 1) a bacteremic form that includes a triad of tenosynovitis, dermatitis, and polyarthralgias without purulent arthritis and 2) a septic arthritis form characterized as a purulent arthritis without associated skin lesions. Many patients will have overlapping features of both syndromes. Time from infection to clinical manifestations may range from 1 day to 3 months. There is no travel history and the rash of Lyme disease is not purulent. Reactive arthritis presents with conjunctivitis, urethritis and arthritis usually with a red hot tender and swollen joint.
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This question is part of the following fields:
- Connective Tissue
- Medicine
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Question 24
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: Anti-nuclear antibody titres
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 25
Incorrect
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A 64 year old female presents with sudden onset pain, swelling and stiffness in her right knee. Her medical history is significant for osteoarthritis affecting her hand joints and diet controlled diabetes mellitus. On examination, the right knee is swollen, erythematous and tender. Which of the following tests would be most useful in the diagnosis of this case?
Your Answer: X-ray of the knee
Correct Answer: Aspiration and examination of the synovial fluid
Explanation:Arthrocentesis should usually be done when there is a suspicion of pseudogout or septic arthritis as in this case which leads to an early diagnosis and prompt treatment. Polarized microscopy demonstrates weakly positively birefringent rhomboid crystals which are blue when parallel to light and yellow when perpendicular to light. Elevated serum uric acid levels that cause gout are usually found after large consumption of alcohol or meat, or post surgery. Autoimmune diseases like SLE, RA etc require an autoimmune screen.
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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: Medial 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 27
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: Sjogren's syndrome
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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Question 28
Incorrect
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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: Pseudogout
Correct 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 29
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: Dizygotic twin
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 30
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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SESSION STATS - PERFORMANCE PER SPECIALTY
