Clinical Features and Outcome in Patients with Glomerulonephritis and Antineutrophil Cytoplasmic Autoantibodies

Abstract

We investigated the clinical features and outcome of 60 patients with antineutrophil cytoplasmic autoantibodies with cytoplamic staining (C-ANCA)- and with (peri)nuclear staining (P-ANCA)-associated glomerulonephritis. Virtually all patients with C-ANCA had antibodies against proteinase 3, which corresponded with a clinical and/or histological diagnosis of Wegener’s granulomatosis (WG). P-ANCA were associated with antibodies against myeloperoxidase. Although more often associated with renal-limited disease, a significant number of patients with P-ANCA had also symptoms of organs known to be preferentially affected with WG. The majority of patients presented with rapidly progressive glomerulonephritis which responded favorably to immunosuppressive therapy, albeit at the expense of a considerable number of fatal infections. Furthermore, a more protracted form of glomerulonephritis was recognized. A kidney biopsy scoring system appeared to be of value in predicting which patients would benefit most from treatment. C-ANCA and P-ANCA appear to identify a subset of patients with vasculitis that share common clinical features and a favorable response to immunosuppressive therapy.

Characteristics of Chinese patients with Wegener's granulomatosis with anti-myeloperoxidase autoantibodies

“…However, it was reported that sera from a minority of patients with cANCA recognized autoantigens other than PR3 (such as MPO) [17], which was supported by our previous study [15]. In the current study, sera with cANCA exclusively recognized PR3, and sera with pANCA exclusively recognized MPO.…”

Clinical and Histologic Determinants of Renal Outcome in ANCA-Associated Vasculitis

“…Therefore, apart from reflecting the functioning part of the kidney, normal glomeruli also are a relatively constant parameter, the combination of which may explain their strength as a predictive parameter. Also intraepithelial tubular infiltrates are predictive of GFR 12 , and their presence may well be related to the development of tubular atrophy, a widely known parameter of chronic renal failure in general and associated with worse renal outcome in ANCA-associated vasculitis (11,18).…”

Circulating adhesion molecules ICAM-1, VCAM-1 and E-selectin in systemic vasculitis : marked differences between Wegener's granulomatosis and systemic lupus erythematosus

“…Kidney biopsies were scored according to an activity index (AI) and chronicity index (CI, Table 2), which was adapted from the scoring system used in SLE [3,10]: semiquantitative scale (0-3; absent, mild, moderate, severe); necrosis, cellular, and fibrous crescents are multiplied by a factor of 2; the maximal AI is 24 and the maximal CI 18. Glomerular lesions were classified into four categories (Table 3).…”