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Alan Dronate is a 56 year old man who has presented to the Emergency Assessment Unit with fatigue and feeling generally unwell. He states he feels very weak, particularly at the shoulders. He has had a cough for around 3 months now and feels that he has lost about 1 stone in weight in that time, unintentionally. He has a background of hypercholesterolaemia and takes atorvastatin 40mg nocte.
You perform a thorough examination. He has proximal weakness, with power 3/5 at both shoulders and 4/5 at both hips. Otherwise he is neurologically intact. On examination of his hands, he has a dupruyten's contracture on the fourth finger of his right hand, and nail fold infarcts, with a number of papules on his knuckles. He has peri-orbital oedema and flagellate erythema. Chest and abdominal examination are grossly normal.
His initial blood tests include: Hb 121 WCC 8.4 Urea 5.6 Creatinine 73 CK: 29,000

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ANA: Elevated cANCA: negative pANCA: negative Anti-Jo: positive ds-DNA: negative
XR Chest report: no evidence pleural effusion or consolidation. bony landmarks normal. There is a hilar mass right lung which appears suspicious.
CT head report: no evidence acute infarction or haemorrhage. Normal examination.
ESR 24

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Correct answer: Dermatomyositis

The most remarkable finding here in the non-specific presentation is the highly elevated CK. Causes of raised CK may include rhabdomyalysis secondary to long lie, statin, steroid use, or polymyositis. There is statin use here, which may be a red herring, however, given the fact that other symptoms are present. There is proximal weakness, suggestive of polymyositis. However, skin exam shows papules on his knuckles, peri-orbital oedema and flagellate erythema: all signs associated with dermatomyositis - the cause of this presentation.

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