Guillevin L, Visser H, Noel L H, Pourrat J, Vernier I, Gayraud M, Oksman F, Lesavre P
Department of Internal Medicine, Hôpital Avicenne, Bobigny, France.
J Rheumatol. 1993 Aug;20(8):1345-9.
Antibodies directed against components of neutrophil cytoplasm have been detected in various systemic vasculitides and especially in Wegener's granulomatosis. In polyarteritis nodosa (PAN) and Churg-Strauss syndrome, few data are available and correlation between clinical manifestations and antineutrophil cytoplasm antibodies (ANCA) has not been established. Therefore, we tested, before treatment of vasculitis, 62 consecutive patients suffering from PAN with hepatitis B virus (HBV) markers, PAN of unknown etiology or Churg-Strauss syndrome.
Only patients with PAN and Churg-Strauss syndrome were included in the study. The diseases were histologically and/or angiographically proven. Every patient's serum was tested by an indirect immunofluorescence assay (IFA) and, in 37 cases, by an enzyme linked immunosorbent assay (ELISA).
ANCA detected by IFA were observed in 10.7% of the patients with PAN with HBV markers, in 27.3% of the patients with PAN without HBV markers and in 66.7% of the patients with Churg-Strauss syndrome. When ELISA was performed, 11.1% of the patients with PAN associated with HBV infection, 20% of the patients with PAN without HBV markers and 55.6% of the patients with Churg-Strauss syndrome were positive. ANCA were positively correlated with asthma and purpura and negatively correlated with HBV markers.
Regardless of the technique used, Churg-Strauss syndrome was associated with ANCA in about 60% of the cases while, in PAN of unknown etiology, ANCA were found in about 25% of cases. In contrast, IFA and ELISA only detected ANCA in a limited number of cases of PAN related to HBV infection. ELISA positivity in patients with PAN and Churg-Strauss syndrome was usually associated with antimyeloperoxidase antibodies. In our cases of PAN, ANCA and purpura were significantly correlated, suggesting that, in these cases, small vessels are involved and therefore macroscopic and microscopic PAN coexist. Thus it seems that ANCA are essentially present in the cases of small vessel vasculitis, as has been described, and are not a marker of pure macroscopic PAN, at least at our present level of understanding of these antibodies.
在多种系统性血管炎中，尤其是韦格纳肉芽肿病中，已检测到针对中性粒细胞胞浆成分的抗体。在结节性多动脉炎（PAN）和变应性肉芽肿性血管炎（CSS）中，相关数据较少，且临床表现与抗中性粒细胞胞浆抗体（ANCA）之间的相关性尚未确立。因此，我们在血管炎治疗前，对62例连续患有PAN且伴有乙型肝炎病毒（HBV）标志物、病因不明的PAN或CSS的患者进行了检测。
本研究仅纳入PAN和CSS患者。疾病经组织学和/或血管造影证实。每位患者的血清通过间接免疫荧光法（IFA）检测，37例患者还通过酶联免疫吸附测定（ELISA）检测。
在伴有HBV标志物的PAN患者中，10.7%通过IFA检测到ANCA；在无HBV标志物的PAN患者中，27.3%检测到ANCA；在CSS患者中，66.7%检测到ANCA。进行ELISA检测时，伴有HBV感染的PAN患者中11.1%呈阳性，无HBV标志物的PAN患者中20%呈阳性，CSS患者中55.6%呈阳性。ANCA与哮喘和紫癜呈正相关，与HBV标志物呈负相关。
无论采用何种技术，CSS约60%的病例与ANCA相关，而在病因不明的PAN中，约25%的病例可检测到ANCA。相比之下，IFA和ELISA仅在少数与HBV感染相关的PAN病例中检测到ANCA。PAN和CSS患者的ELISA阳性通常与抗髓过氧化物酶抗体相关。在我们的PAN病例中，ANCA与紫癜显著相关，提示在这些病例中存在小血管受累，因此宏观和微观的PAN并存。因此，似乎ANCA主要存在于小血管血管炎病例中，正如所描述的那样，至少在我们目前对这些抗体的理解水平上，它不是单纯宏观PAN的标志物。
