Division of Rheumatology, Department of Medicine, Mount Sinai Hospital, University Health Network, University of Toronto, Toronto, ON, Canada.
Rheumatol Int. 2013 Jan;33(1):139-43. doi: 10.1007/s00296-011-2359-z. Epub 2012 Jan 12.
The aim of the study was to describe the occurrence of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) in systemic sclerosis (SSc) patients. SSc patients who developed biopsy-proven AAV were identified. Their clinical manifestations, autoantibodies, presentation with vasculitis, treatment and outcome were described and compared with previously reported patients with these two conditions. Of 985 patients, 3 were identified. All patients had interstitial lung disease, and all presented with acute renal failure, proteinuria and hematuria, and were P-ANCA- and anti-Scl-70-positive. One required hemodialysis. Two were hypertensive; additionally, one patient had sinusitis, and another had monoarthritis and a macular rash. All were treated with high-dose corticosteroids and responded to therapy and attained remission at 6 months. At 1 year, one patient died of pneumonia. ANCA-associated vasculitis is a rare but serious finding in SSc patients. Positive anti-Scl-70 antibody is found commonly in these patients. Different treatment modalities are effective. Serious infections can complicate therapy and lead to death.
本研究旨在描述抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）在系统性硬化症（SSc）患者中的发生情况。确定了发生经活检证实的 AAV 的 SSc 患者。描述了他们的临床表现、自身抗体、血管炎表现、治疗和预后，并与以前报告的具有这两种情况的患者进行了比较。在 985 例患者中，发现了 3 例。所有患者均有间质性肺病，均表现为急性肾衰竭、蛋白尿和血尿，且均为 P-ANCA 和抗 Scl-70 阳性。1 例需要血液透析。2 例患者为高血压；此外，1 例患者有鼻窦炎，另 1 例有单关节炎和斑丘疹。所有患者均接受大剂量皮质类固醇治疗，对治疗有反应，并在 6 个月时达到缓解。1 年后，1 例患者死于肺炎。ANCA 相关性血管炎是 SSc 患者中一种罕见但严重的发现。这些患者中常见抗 Scl-70 抗体阳性。不同的治疗方式均有效。严重感染会使治疗复杂化并导致死亡。
