A Sinister Crisis in Scleroderma

We describe a presentation of Systemic Sclerosis (SSc) with rapid development of Scleroderma Renal Crisis (SRC) and multiorgan failure leading to death. Diagnoses were confirmed on post-mortem examination. SSc usually presents with skin thickening of the fingers and the presence of Anti-Nuclear Antibodies (ANA).Other associated clinical findings include fingertip lesions, telangiectasia, abnormal nailfold capillaries, pulmonary arterial hypertension, interstitial lung disease, and Raynaud's phenomenon. ANA testing is negative in only a small minority of patients. Scleroderma Renal Crisis is characterized by acute renal failure, hypertension, and microangiopathic hemolytic anemia with thrombocytopenia, but occurs without hypertension in some cases. This case demonstrates an atypical case of SSc and SRC in that ANA testing was negative and Scleroderma Renal Crisis developed without hypertension. This presentation illustrates the challenge in diagnosing and managing SSc and SRC in a critically ill patient.