[13-year history of scleroderma complicated by renal crisis and thrombotic microangiopathy treated with plasma...

A 77-year-old man with a 13-year history of systemic sclerosis (SSc) was admitted to our hospital with fever, appetite loss, and disorientation. The patient was well 2 days prior to the admission and had been taking a low dose of a steroid and vasodilators over the previous 10 years. In regular clinic visits, his blood pressure was normotensive and serum creatinine (Cr) was within the normal range. On admission, hypertension (blood pressure 214/105 mmHg), proteinuria, and hypercreatinemia (3.6 mg/dL) led to the diagnosis of sclerotic renal crisis (SRC). Thrombocytopenia (5.4 × 10(4)/μL), erythrocyte fragmentation, and elevated lactate dehydrogenase were suggestive of thrombotic microangiopathy (TMA). The immediate initiation of angiotensin-converting enzyme inhibitor therapy and plasma exchange (PE) rapidly improved the disorientation and thrombocytopenia. It is notable that SRC might occur in patients with a 13-year history of SSc. PE should be considered as a treatment option for SRC complicated by TMA.