医学
显微镜下多血管炎
血管炎
肉芽肿伴多发性血管炎
内科学
Churg-strauss综合征
系统性血管炎
皮肤病科
疾病
结缔组织病
重症监护医学
免疫学
硫唑嘌呤
病理
结节性多动脉炎
ANCA相关性血管炎
纤维化
抗中性粒细胞胞浆抗体
临床试验
作者
Déborah Eshagh,Thomas Quéméneur,Alexandre Karras,V. Queyrel,Jean‐François Augusto,Christian Agard,V. Audard,Alexandre Cez,Marion Couderc,Pierre Duffau,C. Durel,Stanislas Faguer,Noémie Jourde‐Chiche,Aurélie Lavergne,Christian Lavigne,Nicolas Limal,Rafik Mesbah,A. Servettaz,Carole Philipponnet,P. Smets
摘要
OBJECTIVE: Systemic sclerosis (SSc) is an autoimmune disease characterized by autoantibody production, fibrosis, and vasculopathy. The coexistence of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) in SSc is rare and poorly characterized, with limited data on the impact of treatments, particularly high-dose glucocorticoids (GCs), on both conditions. This study aimed to describe the clinical phenotype, management, and outcomes of patients with overlapping SSc and AAV. METHODS: We conducted a multicenter retrospective study in 18 French centers, including patients who met the 2013 American College of Rheumatology (ACR)/EULAR criteria for SSc and the 2022 ACR/EULAR criteria for AAV. Clinical, biologic, and radiologic data were collected. RESULTS: We included 30 patients (median age 51.5 years, 83% female). SSc preceded AAV in all cases; 27% had diffuse cutaneous SSc, whereas 73% had limited cutaneous SSc. Anti-Scl70 antibodies were detected in 50%, and interstitial lung disease (ILD) was present in 80%, predominantly with a fibrosing nonspecific interstitial pneumonia pattern (54%). AAV was microscopic polyangiitis in 90%, with myeloperoxidase (MPO)-ANCA positivity in 93%. Renal involvement was common (76%), with a median serum creatinine level of 170 μmol/L (interquartile range [IQR] 120-361 μmol/L) and proteinuria (urine protein to creatinine ratio of 2 g/g creatinine [IQR 0.9-2.3 g/g creatinine]). All patients received GCs in combination with cyclophosphamide (50%) or rituximab (47%). No cases of scleroderma renal crisis were observed. SSc manifestations, including ILD and skin involvement, remained stable during follow-up. CONCLUSION: AAV, predominantly microscopic polyangiitis with MPO-ANCA, can occur in SSc, particularly in patients with fibrosing ILD and anti-Scl70. Standard vasculitis treatments appear to be effective and do not worsen outcomes in SSc.