Systemic Vasculitis During the Course of Systemic Sclerosis
Systemic Vasculitis During the Course of Systemic Sclerosis
复制标题
系统性硬化症过程中的系统性血管炎
作者：
T. Quéméneur;L. Mouthon;P. Cacoub;O. Meyer;U. Michon;P. Vanhille;P. Hatron;L. Guillevin;E. Hachulla
虽然在系统性硬化症(SSC)患者中存在抗中性粒细胞胞浆抗体(ANCA)的报道，但SSC与系统性脉管炎的关系却很少被描述。我们从法国血管炎研究小组和法国系统性硬化研究小组的所有成员那里获得了与SSc相关的系统性脉管炎病例的信息。我们发现了12名与SSc相关的系统性脉管炎患者：9例为ANCA相关性系统性脉管炎(AASV)，3例为混合性冷球蛋白血症血管炎(MCV)。在所有AASV患者中，SSc均为局限型。SSC的主要并发症为肺纤维化。仅2例患者在AASV发生前接受了D-青霉胺方案治疗。AASV表现为镜下多血管炎7例，肾局限性血管炎2例。9例患者中8例抗髓过氧化物酶抗体阳性。9例患者中有3例五因子评分在1以上。在3例MCV患者中，有2例确诊为Sjögren综合征。我们将我们的发现与文献综述的结果(42例先前报道的AASV合并SSC)进行了比较。AASV是最常见的类型，当肾脏受损时，其诊断可能具有挑战性。更好地认识到这种罕见的关联有助于早期诊断和适当的处理以减少损害。
AbstractAlthough the presence of antineutrophil cytoplasmic antibodies (ANCA) has been reported in patients with systemic sclerosis (SSc), the association of SSc and systemic vasculitis has rarely been described. We obtained information on cases of systemic vasculitis associated with SSc in France from the French Vasculitis Study Group and all members of the French Research Group on Systemic Sclerosis.We identified 12 patients with systemic vasculitis associated with SSc: 9 with ANCA-associated systemic vasculitis (AASV) and 3 with mixed cryoglobulinemia vasculitis (MCV). In all AASV patients, SSc was of the limited type. The main complication of SSc was pulmonary fibrosis. Only 2 patients underwent a D-penicillamine regimen before the occurrence of AASV. The characteristics of AASV were microscopic polyangiitis (n = 7) and renal limited vasculitis (n = 2). Anti-myeloperoxidase antibodies were found in 8 of the 9 patients. The Five Factor Score was above 1 in 3 of the 9 patients. Of the 3 patients with MCV, Sjögren syndrome was confirmed in 2. We compared our findings with the results of a literature review (42 previously reported cases of AASV with SSc).Although rare, vasculitis is a complication of SSc. AASV is the most frequent type, and its diagnosis can be challenging when the kidney is injured. Better awareness of this rare association could facilitate earlier diagnosis and appropriate management to reduce damage.