全身性皮质类固醇和静脉注射环磷酰胺成功治疗与系统性红斑狼疮相关的获得性纯红细胞发育不全。

IF 0.9 Q4 RHEUMATOLOGY
Takafumi Tsushima, Masashi Fukuta, Natsumi Yoda, Chiharu Kimeda, Kazusuke Tanaka, Kosuke Matsuo, Yasuhito Hatanaka, Rena Matsumoto, Sonoko Shimoji, Yoshikazu Utsu, Shin-Ichi Masuda, Nobuyuki Aotsuka
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引用次数: 0

摘要

通常获得性纯红细胞发育不全(PRCA)可能与潜在的自身免疫性疾病有关。与系统性红斑狼疮(SLE)相关的PRCA报告是罕见的,没有治疗策略已被记录。我们报告一例与SLE相关的PRCA对静脉注射环磷酰胺(IVCY)和全身皮质类固醇有反应。一名62岁日本女性被诊断为PRCA和SLE。初次就诊时,患者同时出现严重的蛋白尿、大量胸腔积液和间质改变。值得注意的是,环孢素和皮质类固醇不能改善患者的PRCA和SLE状态。在IVCY和皮质类固醇治疗后,患者的网织红细胞计数和贫血得到改善。与SLE相关的其他各种症状也有所改善。环磷酰胺通常以小剂量连续口服给药。然而,在这种情况下,我们不需要在IVCY后开始这种持续的低剂量治疗。总的来说,本研究强调了包括IVCY在内的治疗SLE相关PRCA的治疗策略。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Successful treatment by systemic corticosteroids and intravenous cyclophosphamide in acquired pure red cell aplasia associated with systemic lupus erythematosus.

Commonly acquired pure red cell aplasia (PRCA) can be associated with an underlying autoimmune disease. Reports of PRCA associated with systemic lupus erythematosus (SLE) are rare, and no treatment strategies have been documented. We report a case of PRCA associated with SLE that responded to intravenous cyclophosphamide (IVCY) and systemic corticosteroids. A 62-year-old Japanese female was diagnosed with PRCA and SLE. At the time of the initial visit, the patient simultaneously presented with severe proteinuria, massive pleural effusion, and interstitial changes. Notably, cyclosporine and corticosteroids did not improve PRCA and SLE status of the patient. After IVCY and corticosteroid administration, the patient's reticulocyte count and anaemia improved. Various other symptoms associated with SLE also improved. Cyclophosphamide is typically administered in PRCA in small, continuous oral doses. However, in this case, we did not need to initiate this continuous, low-dose treatment after IVCY. Overall, this study highlights therapeutic strategies involving IVCY in treating PRCA associated with SLE.

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