Kawabata K, Makino H, Nagake Y, Morita Y, Akiyama K, Ota K, Sugimoto H, Ikeda S, Ota Z
Third Department of Internal Medicine, Okayama University Medical School, Japan.
Nihon Jinzo Gakkai Shi. 1994 Sep;36(9):1067-74.
A 46-year-old woman who noticed tightness of the skin in September, 1993, was admitted to a local hospital due to hypertension, congestive heart failure and renal dysfunction on the 2nd of November. After admission, renal function deteriorated progressively. A diagnosis of scleroderma renal crisis (SRC) was suspected from her skin biopsy and clinical course. She was referred to our hospital for further evaluation and maintenance of hemodialysis. Her blood pressure was kept normal by anti-hypertensive drugs including cilazapril. Acute interstitial pneumonia, microangiopathic hemolytic anemia and thrombocytopenia appeared during her clinical course. Corticosteroid therapy was effective for acute interstitial pneumonia, but in-effective for thrombocytopenia. Plasma exchange was not effective for thrombocytopenia, which was successfully treated with intravenous gamma-globulin therapy. She died of cytomegaloviral encephalitis, which might have resulted from immunodeficiency caused by prolonged corticosteroid therapy and uremia. Complications other than SRC might have appeared during the clinical course based on the immune disorder of progressive systemic sclerosis itself. In order to improve the prognosis of patients with SRC such complications should be detected promptly and treated correctly.
一名46岁女性于1993年9月发现皮肤紧绷，11月2日因高血压、充血性心力衰竭和肾功能不全入住当地医院。入院后，肾功能逐渐恶化。根据其皮肤活检和临床病程，怀疑为硬皮病肾危象（SRC）。她被转诊至我院进行进一步评估和维持血液透析。通过包括西拉普利在内的抗高血压药物，她的血压保持正常。在其临床病程中出现了急性间质性肺炎、微血管病性溶血性贫血和血小板减少症。皮质类固醇疗法对急性间质性肺炎有效，但对血小板减少症无效。血浆置换对血小板减少症无效，静脉注射丙种球蛋白疗法成功治疗了该病症。她死于巨细胞病毒性脑炎，这可能是长期皮质类固醇疗法和尿毒症导致的免疫缺陷所致。基于进行性系统性硬化症本身的免疫紊乱，临床病程中可能出现了除SRC之外的并发症。为改善SRC患者的预后，应及时发现并正确治疗此类并发症。
