Pradhan Vandana D, Badakere S S, Ghosh K, Almeida A
Department of Autoimmune Disorders, Institute of Immunohaematology, Indian Council of Medical Research, K. E. M. Hospital, Mumbai, India.
Indian J Med Sci. 2005 Jul;59(7):292-300.
Wegener's granulomatosis (WG) is being increasingly diagnosed in India, which exists in two forms, the 'limited Wegener's granulomatosis' (LWG) having upper respiratory tract (URT) and lower respiratory tract (LRT) involvement and the 'classical Wegener's granulomatosis' (CWG), with the triad of URT, LRT involvement along with kidney involvement. Cytoplasmic ANCA (C-ANCA) or anti-Proteinase3 (anti-PR3), which is highly diagnostic for WG, rarely perinuclear ANCA (P-ANCA) may exist.
To detect anti-neutrophil cytoplasmic antibodies (ANCA) and correlate it with serological, hematological parameters, and the Birmingham Vasculitis Activity Score (BVAS).
Twenty-three clinically and histopathologically proven WG (16 CWG, 7 LWG) were studied.
C-ANCA and P-ANCA patterns were identified by immunofluorescence and specificities were confirmed by 'alpha granule' enzyme linked immunosorbent assay (ELISA), anti-PR3, anti-MPO (myeloperoxidase) and anti-Lactoferrin (anti-LF) by ELISA.
LRT involvement was seen in 91.3%, URT in 78.3%, and renal manifestations in 69.6% cases. The BVAS in CWG was significantly higher than BVAS in the LWG. Decreased hemoglobin, increased WBC counts, ESR, CRP and Creatinine were seen in CWG as compared to LWG. The C-ANCA was present in 65.2% patients and P-ANCA in 13% cases. Anti-PR3 was seen in 69.6% patients and anti-LF in 17.4% cases. Severity of disease and ANCA was higher in CWG than in LWG.
Vasculitis syndromes are known to overlap and many go undetected; therefore ANCA testing, along with the clinical and histopathological observations may be helpful in early detection and management of WG cases.
韦格纳肉芽肿（WG）在印度的诊断率日益增高，它有两种形式，即“局限性韦格纳肉芽肿”（LWG），累及上呼吸道（URT）和下呼吸道（LRT）；以及“典型韦格纳肉芽肿”（CWG），具有上呼吸道、下呼吸道受累以及肾脏受累的三联征。对WG具有高度诊断价值的胞浆型抗中性粒细胞胞浆抗体（C-ANCA）或抗蛋白酶3（抗PR3），也可能存在少量核周型抗中性粒细胞胞浆抗体（P-ANCA）。
检测抗中性粒细胞胞浆抗体（ANCA），并将其与血清学、血液学参数以及伯明翰血管炎活动评分（BVAS）相关联。
对23例经临床和组织病理学证实的WG患者（16例CWG，7例LWG）进行研究。
通过免疫荧光法鉴定C-ANCA和P-ANCA模式，并通过“α颗粒”酶联免疫吸附测定（ELISA）确认其特异性，通过ELISA检测抗PR3、抗髓过氧化物酶（抗MPO）和抗乳铁蛋白（抗LF）。
91.3%的病例出现下呼吸道受累，78.3%出现上呼吸道受累，69.6%出现肾脏表现。CWG的BVAS显著高于LWG的BVAS。与LWG相比，CWG患者血红蛋白降低、白细胞计数增加、血沉、C反应蛋白和肌酐升高。65.2%的患者存在C-ANCA，13%的病例存在P-ANCA。69.6%的患者出现抗PR3，17.4%的病例出现抗LF。CWG的疾病严重程度和ANCA水平高于LWG。
已知血管炎综合征存在重叠，许多未被发现；因此，ANCA检测以及临床和组织病理学观察可能有助于WG病例的早期检测和管理。
