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狼疮患者急性狼疮性心肌炎、肺动脉高压和手指坏疽同时发作:血管炎可能导致这些罕见疾病。

Concurrent onset of acute lupus myocarditis, pulmonary arterial hypertension and digital gangrene in a lupus patient: a possible role of vasculitis to the rare disorders.

机构信息

Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan.

Division of Cardiology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan.

出版信息

Mod Rheumatol Case Rep. 2020 Jan;4(1):21-27. doi: 10.1080/24725625.2019.1650698. Epub 2019 Aug 13.

Abstract

Acute lupus myocarditis and pulmonary arterial hypertension (PAH) are rare complications associated with systemic lupus erythematosus (SLE). No previous reports have shown the coexistence of these disorders. Here we present a 41-year-old patient with SLE who concurrently developed severe acute lupus myocarditis and PAH with digital gangrene as an initial manifestation. Acute lupus myocarditis and PAH were successfully treated with prednisolone and intravenous cyclophosphamide pulse therapy (600-700 mg × 6) along with anticoagulant therapy. Catheter-directed thrombolysis was required for digital gangrene caused by vasculitis. Concurrent development of these rare disorders may represent a common mechanism such vasculitis as an underlining cause of SLE.

摘要

急性狼疮性心肌炎和肺动脉高压(PAH)是系统性红斑狼疮(SLE)相关的罕见并发症。目前尚无相关报道显示这两种疾病同时存在。本文报道了一例 41 岁 SLE 患者,以指端坏疽为首发表现,同时发生严重的急性狼疮性心肌炎和 PAH。经泼尼松龙和静脉注射环磷酰胺脉冲治疗(600-700mg×6)联合抗凝治疗后,急性狼疮性心肌炎和 PAH 得到成功治疗。针对由血管炎引起的指端坏疽,还需要进行导管定向溶栓治疗。这些罕见疾病的同时发生可能代表了一种共同的机制,即血管炎是 SLE 的潜在病因。

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