Ubukata Masamitsu, Mitsuhashi Atsushi, Nishizawa Yuki, Fujii Teruhiro, Hara Masaki, Ohta Akihito, Nitta Kosaku
Division of Nephrology, Department of Medicine, Tokyo Metropolitan Komagome Hospital, Japan.
Department IV, Internal Medicine, Tokyo Women's Medical University, Japan.
Intern Med. 2018 Dec 1;57(23):3479-3483. doi: 10.2169/internalmedicine.0980-18. Epub 2018 Aug 10.
A 70-year-old man with prior Raynaud's phenomena developed hypertension and renal insufficiency. Raynaud's phenomena, finger skin thickening, interstitial lung disease, and positive anticentromere antibody findings indicated systemic sclerosis (SSc). Based on the presence of SSc, severe hypertension with rapidly progressive renal failure, and proliferative and obliterative arteriolar vasculopathy, scleroderma renal crisis (SRC) was diagnosed. Despite good blood pressure control with antihypertensive drugs, hemodialysis was initiated and could not be withdrawn owing to unimproved renal dysfunction. Although SRC in anticentromere antibody-positive limited cutaneous SSc is extremely rare, some patients may develop SRC, and their renal prognosis may be poor.
一名既往有雷诺现象的70岁男性出现高血压和肾功能不全。雷诺现象、手指皮肤增厚、间质性肺疾病以及抗着丝点抗体阳性结果提示系统性硬化症（SSc）。基于存在SSc、伴有快速进展性肾衰竭的严重高血压以及增生性和闭塞性小动脉血管病变，诊断为硬皮病肾危象（SRC）。尽管使用抗高血压药物血压控制良好，但仍开始了血液透析，且由于肾功能未改善无法停止。虽然抗着丝点抗体阳性的局限性皮肤型SSc中的SRC极为罕见，但一些患者可能会发生SRC，且其肾脏预后可能较差。
