13-year history of scleroderma complicated by renal crisis and thrombotic microangiopathy treated with plasma exchange: A case report

Abstract

summaryA 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. Key words

Thrombotic Microangiopathy After Spontaneous Pheochromocytoma Rupture: A Rare MEN 2A Case

“…After excluding the primary TMA and the possible secondary causes, the patient was evaluated for secondary TMA due to malignant hypertension after the rupturing of pheochromocytoma. Although plasma exchange is recommended for primary TMA, some studies show that plasma exchange therapy is beneficial in secondary TMA as well (19)(20)(21)(22). Since the platelet count of the patient increased above 200 (x10 3 /uL), along with a sufficient increase in hemoglobin level, plasma exchange therapy was discontinued.…”