Nagamura Norihiro, Kin Seikon
1Department of Rheumatology and Allergy, Shimane Prefectural Central Hospital, Izumo, Japan.
Department of Nephrology, Shimane Prefectural Central Hospital, Izumo, Japan.
Nagoya J Med Sci. 2016 Aug;78(3):329-34.
Systemic sclerosis (SSc) is a multiorgan disorder involving the skin, heart, lungs, kidneys, and intestines. Progressive interstitial lung disease (ILD) is a serious complication in SSc patients, and cyclophosphamide (CYC) is the only recommended therapy for this condition;(1)) however, its clinical effectiveness is not sufficient. Scleroderma renal crisis (SRC) is a rare complication, characterized by acute renal failure and progressive hypertension. Angiotensin-converting-enzyme inhibitor (ACE-i) is a widely accepted therapy for SRC. We report an SSc patient with SRC and progressive ILD who underwent treatment with CYC and successful treatment with ACE-i and plasma exchange (PE). SRC and ILD are significant contributors to morbidity and mortality among SSc patients, and the therapy for these disorders is of great interest to rheumatologists. This study presents the possibility of favorable effects of PE for SSc-associated ILD and SRC.
系统性硬化症（SSc）是一种累及皮肤、心脏、肺、肾脏和肠道的多器官疾病。进行性间质性肺病（ILD）是SSc患者的一种严重并发症，环磷酰胺（CYC）是针对这种情况唯一推荐的治疗方法；(1) 然而，其临床疗效并不充分。硬皮病肾危象（SRC）是一种罕见的并发症，其特征为急性肾衰竭和进行性高血压。血管紧张素转换酶抑制剂（ACE-i）是一种被广泛接受的SRC治疗方法。我们报告了一名患有SRC和进行性ILD的SSc患者，该患者接受了CYC治疗，并成功接受了ACE-i和血浆置换（PE）治疗。SRC和ILD是导致SSc患者发病和死亡的重要因素，这些疾病的治疗方法是风湿病学家非常感兴趣的。本研究提出了PE对SSc相关ILD和SRC可能产生有利影响的可能性。
