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2014 ; 2014
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BMJ Case Rep
2014[May]; 2014
(ä): ä PMID24876209
show ga
A patient presented with neuromuscular, respiratory and cardiac symptoms and was
initially diagnosed with amyotrophic lateral sclerosis (ALS), myocardial
ischaemia and pneumonia. He developed unexplained progressive kidney failure over
the ensuing week, and his kidney biopsy showed thrombotic microangiopathy that
led to the correct diagnosis of normotensive scleroderma renal crisis. His
clinical presentation and course were consistent with systemic sclerosis and
normotensive scleroderma renal crisis. He was treated with an ACE inhibitor
(ACEi) and haemodialysis with significant functional improvement over the next
3?months to his prior baseline with the exception of kidney failure. This case
highlights a diagnostic challenge requiring astute history and physical
examination skills, and the value of a kidney biopsy in providing the final
diagnosis.