Complement in ANCA-associated glomerulonephritis.

Background: Anti-neutrophil cytoplasmic antibodies (ANCA) are found in pauci-immune necrotizing crescentic glomerulonephritis. In the past, the role of complement in ANCA-associated vasculitis (AAV) was assumed to be minimal. More recently, however, it was found that blocking the complement cascade in a mouse model of AAV reduces glomerular damage.

Connective tissue growth factor and the cicatrization of cellular crescents in ANCA-associated glomerulonephritis.

Background: Outcome in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated glomerulonephritis (AGN) is difficult to predict. Scoring of renal biopsies has significant but limited predictive value. We investigated whether analysis of plasma and urine levels, and immunostaining of biopsies for the pro-fibrotic peptide connective tissue growth factor (CTGF), might improve prediction of renal outcome.

Estimating renal survival using the ANCA-associated GN classification.

A histopathological classification system for ANCA-associated vasculitis was recently published, but whether this system predicts renal outcome requires validation. Here, we analyzed data from 164 consecutive patients with biopsy-proven renal involvement of ANCA-associated vasculitis. The ANCA-associated GN (AGN) classification categorizes patients as having focal, mixed, crescentic, or sclerotic GN.

Improved outcome in anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis: a 30-year follow-up study.

Background: Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis has a poor prognosis. In the current study, we assessed whether prognosis in these patients improved over the last three decades.

Methods: In a large inception cohort, all consecutive patients with ANCA-associated glomerulonephritis were included between January 1979 and December 2009.

Immune complexes in acute adult-onset Henoch-Schonlein nephritis.

Background: Adult-onset Henoch-Schönlein purpura nephritis (HSPN) and primary IgA (IgAN) nephropathy have been considered indistinguishable immunohistopathologically and are often considered as two extremes of one disease entity. We postulate that adult-onset Henoch-Schönlein can be distinguished histologically from primary IgAN and that both diseases differ in their immunopathological mechanisms.

Methods: Twenty consecutive patients with adult-onset HSPN were studied.

Coexistence of anti-glomerular basement membrane antibodies and myeloperoxidase-ANCAs in crescentic glomerulonephritis.

Background: In a substantial proportion of patients with crescentic glomerulonephritis (CGN), both anti-glomerular basement membrane (GBM) antibodies and antineutrophil cytoplasmic antibodies (ANCAs) with specificity for myeloperoxidase (MPO-ANCA) are detected. In the present study, we questioned whether histological and clinical features of patients with both ANCA and anti-GBM antibodies differ from those of patients with either ANCA or anti-GBM alone.

Methods: We reviewed the Limburg renal biopsy registry (1978 to 2003; n = 1,373) for cases of CGN.

Signs and symptoms of thin basement membrane nephropathy: a prospective regional study on primary glomerular disease-The Limburg Renal Registry.

Background: To chart the epidemiology of primary glomerular disease by means of a prospective regional study in the southern part of The Netherlands.

Methods: Experienced renal technicians collected renal biopsies, blood, and 24-hour urine samples at the bed site in each of the participating hospitals. The material was processed and analyzed at the University Hospital Maastricht.

Susceptibility to cyclosporin A-induced autoimmunity: strain differences in relation to autoregulatory T cells.

Cyclosporin A-induced autoimmunity (CsA-AI), also called autoimmune syngeneic graft-vs-host disease, is a thymus dependent, T cell mediated rodent animal model of disease and is considered to be an experimental model for human scleroderma. Since adoptive transfer of CsA-AI by effector T cells can be prevented by autoregulatory T cells, there may also be a role for dominant tolerance in the resistance of certain rat strains to develop clinical manifest CsA-AI. LEW rats have been reported to be susceptible, whereas BN rats are resistant to CsA-AI.

The CD8 T cell compartment plays a dominant role in the deficiency of Brown-Norway rats to mount a proper type 1 immune response.

Differential cytokine production by T cells plays an important role in regulating the nature of an immune response. In the rat, Brown-Norway (BN) and Lewis (LEW) strains differ markedly in their susceptibility to develop either type 1 or type 2-mediated autoimmune manifestations. BN rats are susceptible to type 2-dependent systemic autoimmunity, while LEW rats are resistant.