Department of Rheumatology, Tel Aviv Sourasky Medical Center and the Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Semin Arthritis Rheum. 2011 Oct;41(2):223-9. doi: 10.1016/j.semarthrit.2010.11.001. Epub 2011 Feb 1.
To report 3 cases ANCA-associated vasculitis (AAV) that developed in patients suffering from systemic sclerosis (SSc) and to review previously reported cases.
We describe 3 patients diagnosed with SSc who developed severe AAV that presented as crescentic glomerulonephritis (GN) and/or alveolar hemorrhage. A retrospective review of the literature was performed using the PubMed database.
The first patient presented with rapidly progressive renal failure and then with 2 episodes of massive alveolar hemorrhage. She was partially refractory to treatment with corticosteroids and cyclophosphamide but responded promptly to treatment with rituximab. The second patient suffered from 2 episodes of fulminant alveolar hemorrhage; the first responded to intravenous corticosteroids, but the second was fatal despite aggressive immune suppression with corticosteroids and cyclophosphamide. The third patient presented with a clinical picture compatible with scleroderma renal crisis (SRC) but was later diagnosed with crescentic GN and subsequently died from probable alveolar hemorrhage. Thirty-seven cases of AAV in SSc patients have been described in the English literature. Clinical manifestations include rapidly progressive GN, alveolar hemorrhage, limb ischemia, and vasculitic skin rash. In contrast to SRC that usually develops early in the course of SSc, ANCA-associated GN in SSc patients occurred later, after several years of illness. Hypertension, microangiopathic hemolytic anemia, and thrombocytopenia that are the hallmark of SRC were observed only in a minority of AAV cases. Almost all cases of AAV in SSc were positive for MPO-ANCA.
AAV in the setting of SSc is a diagnostic challenge. Differential diagnosis from SRC is crucial as treatment approach for these conditions completely differs. High doses of corticosteroids and immune suppression are advocated in severe AAV. In resistant cases, treatment with rituximab may be considered.
报告 3 例发生在系统性硬化症（SSc）患者中的抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）病例，并复习以往报道的病例。
我们描述了 3 例诊断为 SSc 的患者，他们发生了严重的 AAV，表现为新月体性肾小球肾炎（GN）和/或肺泡出血。使用 PubMed 数据库进行文献回顾。
第 1 例患者表现为进行性肾功能衰竭，随后发生 2 次大咯血。她对皮质类固醇和环磷酰胺的治疗部分耐药，但对利妥昔单抗治疗反应迅速。第 2 例患者发生 2 次暴发性肺泡出血；第 1 次对静脉注射皮质类固醇有反应，但第 2 次尽管用皮质类固醇和环磷酰胺进行了积极的免疫抑制治疗，但仍致命。第 3 例患者表现为符合硬皮病肾危象（SRC）的临床表现，但后来被诊断为新月体性 GN，随后死于可能的肺泡出血。在英文文献中已描述了 37 例 SSc 患者的 AAV。临床表现包括进行性 GN、肺泡出血、肢体缺血和血管炎皮疹。与通常在 SSc 病程早期发生的 SRC 不同，SSc 患者的 ANCA 相关性 GN 发生在疾病数年后。只有少数 AAV 病例观察到 SRC 的标志性特征，如高血压、微血管性溶血性贫血和血小板减少。几乎所有 SSc 中的 AAV 病例均为 MPO-ANCA 阳性。
SSc 中的 AAV 是一个诊断挑战。与 SRC 的鉴别诊断至关重要，因为这两种疾病的治疗方法完全不同。严重的 AAV 主张使用大剂量皮质类固醇和免疫抑制治疗。在耐药的情况下，可考虑使用利妥昔单抗治疗。
