ANCA: Serology in Wegener′s granulomatosis

Abstract

BACKGROUND AND OBJECTIVES 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 antiProteinase3 (anti-PR3), which is highly diagnostic for WG, rarely perinuclear ANCA (P-ANCA) may exist. Aims To detect anti-neutrophil cytoplasmic antibodies (ANCA) and correlate it with serological, hematological parameters, and the Birmingham Vasculitis Activity Score (BVAS). SETTINGS AND DESIGN Twenty-three clinically and histopathologically proven WG (16 CWG, 7 LWG) were studied. MATERIAL AND METHODS C-ANCA and P-ANCA patterns were identified by immunofluorescence and specificities were confirmed by 'α granule' enzyme linked immunosorbent assay (ELISA), anti-PR3, anti-MPO (myeloperoxidase) and anti-Lactoferrin (anti-LF) by ELISA. RESULTS 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. Conclusions 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.

Optic perineuritis secondary to Wegener's granulomatosis

“…Autoantibodies against cyto-plasmic components of neutrophils, granulocytes and monocytes (ANCAs) are often present in WG, found in 88-92.6% of patients. 17 ANCA positivity, however, is less frequent among patients with the limited form of WG, reported in only 60-65%. 18,19 The presence of cANCA (in which the target antigen is proteinase-3) correlates best with disease activity in WG.…”

“…18,19 The presence of cANCA (in which the target antigen is proteinase-3) correlates best with disease activity in WG. 17 In contrast, p-ANCA positivity (in which the antigen is myeloperoxidase) is uncommon in WG and may be associated with a milder form of the disease. 20 In our series, cANCA was positive in one patient and pANCA positive in two.…”

Wegener’s Granulomatosis Masquerading as Pansinusitis and Nasal Polyposis: A Diagnostic Dilemma

“…Of the 25 cases analyzed by Kumar A et al [8], only 2 had limited disease involving the upper respiratory tract. Pradhan VD et al [9] reported limited disease in 7 out of the 28 patients studied, out of which 6 had upper respiratory tract involvement; of these cases, 71.4% showed ANCA positivity as compared to 81.2% cases of classical WG. The WGET cohort of 180 patients included 52 patients with limited disease, 88.5% of whom had ENT involvement [6].…”

Immune deposits in cutaneous lesions of Wegener′s granulomatosis: Predictor of an active disease

“…[2] Four studies from India have described cutaneous manifestations in 32–39% of patients with WG, but none have described immunofluorescence as well as histopathologic findings in skin lesions. [4–7] To our knowledge and based on our data search, this is the first study from India that shows the presence of immune deposits in skin biopsies of WG. These studies and our results challenge the current notion that WG is a genuine pauci-immune vasculitis.…”