Stage 4 CSIM2 - Multi-system Disease Quiz

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0 [{"id":638446,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:56:16","updated_at":"2018-12-07 08:56:16","questionName":"What percentage of Giant Cell Arteritis have underlying Polymyalgia Rheumatica?","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":null,"position":null,"explanation":"","question_score_id":null,"lang":"","questionAudioPath":null},{"id":638445,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:55:15","updated_at":"2018-12-07 08:55:15","questionName":"Which 2 are ANCA positive?","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":null,"position":null,"explanation":"Wegeners\r\nChurg Strauss\r\n","question_score_id":null,"lang":"","questionAudioPath":null},{"id":638435,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:34:33","updated_at":"2018-12-07 08:34:33","questionName":"A 42 year old woman is referred to ENT under the two-week wait rule with painless asymmetrical parotid swelling. On direct questioning she states that she has been having problems with dryness of the eyes and mouth for several weeks, as well as pain and stiffness in her wrists. She has had multiple dental operations this year for severe caries, and complains of halitosis which is interfering with her work.\r\n\r\nOn examination, there is bilateral non-tender parotid swelling larger on the right side. She has a dry tongue with an overlying yellow\/white plaque. Cranial nerves are intact bilaterally.\r\n\r\nWhat is the most likely diagnosis?\r\n","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":null,"position":null,"explanation":"Correct answer: Sj\u00f6gren\u2019s syndrome\r\n\r\nThe presence of sicca symptoms (dry eyes, a dry mouth causing bad breath and dental disease) makes Sj\u00f6gren\u2019s the likely diagnosis here.\r\n\r\nHIV can cause salivary gland enlargement and joint pains but is unlikely to produce sicca symptoms.\r\nParotid adenocarcinoma would normally cause unilateral salivary gland enlargement, and may involve the facial nerve causing a palsy.\r\nParotid pleomorphic adenoma would again usually be unilateral and wouldn\u2019t explain the sicca symptoms or joint pains.\r\nMumps would be more likely to mention a fever, and the swelling tends to be painful.\r\n","question_score_id":null,"lang":"","questionAudioPath":null},{"id":638437,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:36:54","updated_at":"2018-12-07 08:36:54","questionName":"A 30 year old woman attends Gynaecology Outpatients for investigation of recurrent miscarriage. She is G4P1, with her previous three pregnancies ending in miscarriage between 12 and 19 weeks gestation. Her first pregnancy was carried to term, and was complicated by a popliteal deep vein thrombosis.\r\nOn examination, livedo reticularis is noted over both lower limbs. The remainder of the examination is unremarkable.\r\nWhich investigation is most diagnostic?\r\n","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":null,"position":null,"explanation":"Correct answer: anti-cardiolipin antibody\r\n\r\nThis lady already has one feature of possible APLS, so a positive antiphospholipid antibody at this appointment (then repeated 12 weeks after) would confirm the diagnosis.\r\n\r\nAnti-dsDNA may be positive given the association between SLE and APLS, but there are insufficient other SLE diagnostic criteria here for this to be a diagnostic test (you would need to do multiple other tests alongside it)\r\nANCA would not be a diagnostic test in and of itself (it may just hint at an ANCA-associated vasculitis being present)\r\nAnti-RNA polymerase III is for diffuse systemic sclerosis \u2013 no other features of this are present in the stem\r\nAnti-U1-RNP is for mixed connective tissue disease \u2013 again, no other features of this are present.\r\n","question_score_id":null,"lang":"","questionAudioPath":null},{"id":638442,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:48:23","updated_at":"2018-12-08 10:12:37","questionName":"A 64-year-old woman comes to the emergency department because of sinusitis, a runny nose, and hemoptysis for the past 3 months. The nasal drainage is often purulent and occasionally contains blood. The patient has also experienced new joint pains and fatigue during the same time-period. Examination shows tenderness to percussion over the maxillary sinuses; however, is otherwise non-contributory. Urinalysis shows 3+ microscopic haematuria and 2+ proteinuria. Chest X-ray is shown below. What is the most likely diagnosis? ","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":"volume-1\/csim-multi-system-disease-quiz\/wegxray.jpg","position":null,"explanation":"Granulomatosis with polyangiitis (GPA) is an autoimmune condition that affects the lungs and kidneys. It is associated with cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) and commonly causes nasal inflammation resulting in bloody or purulent nasal discharge.","question_score_id":null,"lang":"","questionAudioPath":null},{"id":638438,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:38:18","updated_at":"2018-12-07 08:38:18","questionName":"A 46 year old woman presents to her GP in December with a six week history of discolouration and pain in her fingers. She reports her fingers turning white on exposure to cold and being difficult to use, then turning bright red with gradual return of function. She also reports a gradual stiffening of her finger movements during this time which is interfering with her work as a software engineer.\r\nOn examination, she has generalised finger swelling with no obvious synovitis. There are isolated tender pits over few finger pulps bilaterally. Multiple telangiectasia are noted over the face and trunk. Blood tests are significant for a positive anti-centromere antibody.\r\n\r\nWhat is the most likely diagnosis?\r\n","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":null,"position":null,"explanation":"Correct answer: Systemic sclerosis\r\n\r\nThe features present in this lady point to a diagnosis of systemic sclerosis, specifically the limited subtype (CREST syndrome) given her Raynauds, ?sclerodactyly, telangiectasia and positive anti-centromere. The tender pits are likely digital infarcts from severe Raynaud\u2019s.\r\n\r\nDermatomyositis can be associated with Raynaud\u2019s but would not explain the swollen fingers or positive anti-centromere.\r\nRA can be associated with Raynaud\u2019s but would not explain the telangiectasia or anti-centromere. Active rheumatoid inflammation of the hands would be expected to cause synovitis as it is an inflammatory arthritis.\r\nThis patient has no diagnostic criteria of SLE.\r\nTakayasu\u2019s arteritis could explain the upper limb ischaemia (classically pulseless arms in a younger woman) but not the anti-centromere or generalised finger swelling\/stiffening.\r\n","question_score_id":null,"lang":"","questionAudioPath":null},{"id":638449,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:57:54","updated_at":"2018-12-07 08:57:54","questionName":"A 50 year old smoker presents with exertional SOB, wheeze and malaise. Chest sounds wheezy. A FBC reveals a normocytic anaemia. What\u2019s the most likely diagnosis?","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":null,"position":null,"explanation":"","question_score_id":null,"lang":"","questionAudioPath":null},{"id":638448,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:57:20","updated_at":"2018-12-07 08:57:20","questionName":"What is the pathophysiology of Good pastures syndrome?","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":null,"position":null,"explanation":"","question_score_id":null,"lang":"","questionAudioPath":null},{"id":638447,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:56:39","updated_at":"2018-12-07 08:56:39","questionName":"What dose of corticosteroid should you give immediately to a pt presenting with visual loss and a tender scalp?","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":null,"position":null,"explanation":"","question_score_id":null,"lang":"","questionAudioPath":null},{"id":638441,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:44:22","updated_at":"2018-12-07 08:44:22","questionName":"A 73 year old woman presents to her Rheumatologist for follow-up of limited cutaneous systemic sclerosis, diagnosed 11 years previously. She reports a two month history of increasing breathlessness and decline in exercise tolerance. She denies cough, wheeze or chest pain. She has a 20 pack-year smoking history.\r\n\r\nOn examination, she is comfortable at rest with normal breath sounds and symmetrical chest expansion. A high-resolution CT chest, echocardiogram and set of pulmonary function tests are arranged, which show the below findings.\r\n\r\nWhat is the most likely diagnosis?\r\n","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":"volume-1\/csim-multi-system-disease-quiz\/MSDSBA5.png","position":null,"explanation":"Correct answer: Pulmonary arterial hypertension\r\n\r\nThe quoted pulmonary arterial pressure is sufficient to diagnose PAH here, but the examiners would not expect you to know the normal range for this! Instead the answer can be reached by knowing that this is one of the commonest internal complications of LcSSc, and by ruling the other answers out:\r\n\r\nAspiration pneumonia is a major risk for patients with LcSSc given their oesophageal dysmotility, but it would be commented upon in the HRCT if present\r\nThere is no reported bronchial dilatation on the HRCT so bronchiectasis is less likely\r\nHypersensitivity pneumonitis could fit with the symptoms but there are no clear risk factors and the CT findings are not specific for this; it is therefore less likely than the common complications of PAH (+\/- ILD) for this patient\r\nRestrictive pericarditis was not seen on the echo report.\r\n","question_score_id":null,"lang":"","questionAudioPath":null},{"id":638439,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:39:59","updated_at":"2018-12-07 08:39:59","questionName":"A 53 year old woman presents to the Emergency Department with a history of central chest pain present since waking. She is normally fit and well, and is on no regular medications. ECG on admission showed saddle-shaped ST elevation, and troponins are mildly elevated with no dynamic change. Her FBC shows a lymphocytosis with low platelets and normocytic anaemia. An echocardiogram is booked and she is transferred to the acute medical unit with a working diagnosis of pericarditis.\r\nOn clerking, she reports a recent history of fatigue and mild generalised small joint pain and stiffness. A peeling rash is noted over her cheeks and forearms, which she attributes to bad sunburn following a recent holiday to Greece.\r\n\r\nWhich investigation is most likely to \u000bbe diagnostic of her underlying illness?\r\n","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":null,"position":null,"explanation":"Correct answer: Anti-dsDNA\r\n\r\nThis question is testing your knowledge of the ACR SLE diagnostic criteria. By performing anti-dsDNA, a positive result would give you sufficient findings to make a provisional diagnosis of lupus (alongside serositis, pancytopenia, arthritis and photosensitivity).\r\n\r\nAnti-histone antibody is associated with drug-induced lupus, and this lady is on no regular medications (remember the point about negative statements in exam questions; they are likely ruling out a diagnosis)\r\nANCA-associated vasculitis could fit with this clinical syndrome but this would not be a diagnostic test in itself (a positive ANCA would just lead you to make further definitive investigations)\r\nChest X-ray would be unlikely to add any further value regarding her underlying illness, but would definitely be performed in this patient on admission.\r\nPericardiocentesis would treat\/diagnose a pericardial effusion; we already have a working diagnosis of pericarditis as explained in the stem, so this would not tell you much more about the underlying diagnosis that is being hinted at (SLE).\r\n","question_score_id":null,"lang":"","questionAudioPath":null},{"id":638443,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:51:34","updated_at":"2018-12-07 08:51:34","questionName":"A 33-year-old man comes to the clinic because of new skin lesions for the past 9 months. The patient says that the skin lesions have been growing in number and that they tend to blister and then form a black center. Medical history is contributory for asthma for which he requires an albuterol inhaler as needed. Physical examination shows bleeding areas on the arms and legs with a central black dimple. Laboratory test shows 11% of eosinophils in the blood. Which of the following serologies is most likely to be positive or elevated in this patient's condition?","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":null,"position":null,"explanation":"Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) is an ANCA-associated (p-ANCA) small vessel vasculitis that is characterized by eosinophilia, asthma, and vasculitis. Skin findings include blisters and bleeding areas with a central black dimple representing tissue necrosis. ","question_score_id":null,"lang":"","questionAudioPath":null},{"id":638440,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:41:33","updated_at":"2018-12-07 08:41:33","questionName":"A 64 year old man presents to his GP complaining of painful shoulders and feeling \u201ctired all the time\u201d. He denies weight loss, joint stiffness or visual changes. His family history is significant for rheumatoid arthritis in his mother.\r\nOn examination, there is an violaceous rash around both eyes and the base of the neck. Multiple scaly papules are present over the extensor surfaces of both hands. Muscle strength is 4\/5 proximally and 5\/5 distally, and resisted movement is mildly painful.\r\n\r\nWhich investigation is most diagnostic?\r\n","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":null,"position":null,"explanation":"Correct answer: Muscle biopsy\r\n\r\nOf all of these tests, muscle biopsy is the one that would give you a definitive diagnosis of dermatomyositis.\r\n\r\nAnti-Scl-70 is associated with diffuse systemic sclerosis (does not fit with the question stem)\r\nAnti-Jo-1 is associated with antisynthetase syndrome\/polymyositis (the skin findings are not consistent with this diagnosis)\r\nCK would be performed in this patient and is very likely to be elevated \u2013 but this is not specific and would not lead to a final diagnosis\r\nESR is likely to be elevated but is even less specific than CK in this instance.\r\n","question_score_id":null,"lang":"","questionAudioPath":null},{"id":638436,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:35:56","updated_at":"2018-12-07 08:35:56","questionName":"A 62 year old man presents to his GP with a one month history of progressive muscle weakness and weight loss. On direct questioning his only further symptom is a chronic non-productive cough. He denies any small or large joint pain and swelling. There is no significant family history. He has a 30 pack-year smoking history.\r\nOn examination he is unable to stand independently from a chair, with 3\/5 power proximally and 5\/5 power distally in all four limbs. Reflexes are normal and cranial nerves are intact. There is prominent non-tender muscle wasting at the shoulders and hips. On inspection of the hands, a pigmented rash is noted over his palmar creases. Respiratory, cardiac and abdominal examination is unremarkable.\r\n\r\nWhat is the most likely diagnosis?\r\n","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":null,"position":null,"explanation":"Correct answer: Small cell lung cancer\r\n\r\nThis question was intended to remind you that lots of the Stage 4 conditions will be presenting in a multi-system manner. Out of the available options, the diagnosis that best fits is SCLC as he has been losing weight with a new cough and a significant smoking history, and he has features of excess ACTH secretion (pigmented palmar creases). The muscle weakness could be caused either by a paraneoplastic Cushings syndrome (from ectopic ACTH secretion) causing proximal myopathy, or it could be neurological in origin e.g. Lambert-Eaton Myasthenic Syndrome (LEMS) in which you\u2019d expect the weakness to improve on repeated exercise.\r\n\r\nDermatomyositis is a strong differential for this case, but wouldn\u2019t explain the palmar pigmentation (which is the key differentiator in this question). The rest of the history would fit however, especially if he had a co-existent cancer causing the weight loss.\r\nPituitary macroadenoma would likely produce some further cranial symptoms\/signs, e.g. bitemporal hemianopia, and would not explain the cough.\r\nPMR would usually be painful, and doesn\u2019t explain the cough. There is no reference to joint stiffness in the question which is a key feature of this condition.\r\nSLE could probably cause all of these symptoms (through some lateral thinking) but there are insufficient ACR criteria here to make a likely diagnosis.\r\n\r\nThis is a tricky question, so well done if you got it right!","question_score_id":null,"lang":"","questionAudioPath":null},{"id":638444,"quiz_id":"30700","answer_id":null,"answerType_id":"0","created_at":"2018-12-07 08:54:30","updated_at":"2018-12-07 08:54:30","questionName":"Which vasculitis is associated with Hep B?","questionTimeSeconds":"0","questionTimeMinutes":"2","questionImagePath":null,"position":null,"explanation":"","question_score_id":null,"lang":"","questionAudioPath":null}]
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What percentage of Giant Cell Arteritis have underlying Polymyalgia Rheumatica?

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