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IgA-mediated anti-glomerular basement membrane disease: an uncommon mechanism of Goodpasture's syndrome.

Moulis G, Huart A, Guitard J, Fortenfant F, Chauveau D - Clin Kidney J (2012)

Bottom Line: Despite immunosuppressive regimen, the disease progressed to end-stage renal failure (ESRF).Transplantation was not associated with recurrence in the kidney graft.We reviewed the 11 previously reported cases of IgA-mediated GP.

View Article: PubMed Central - PubMed

Affiliation: Néphrologie et Immunologie Clinique , CHU Rangueil , Toulouse , France ; Université de Toulouse , Toulouse , France ; Centre de Référence des Maladies Rénales Rares, Toulouse , France.

ABSTRACT
Goodpasture's (GP) disease is usually mediated by IgG autoantibodies. We describe a case of IgA-mediated GP, in a patient presenting with isolated rapidly progressive glomerulonephritis. The diagnosis was established on kidney biopsy, since routine enzyme-linked immunosorbent assay (ELISA) targeted at IgG circulating autoantibodies failed to detect the nephritogenic antibodies. Immunofluorescence microscopy showed intense linear deposition of IgA along the glomerular capillary walls. An elevated titre (1:80) of circulating IgA anti-glomerular basement membrane (GBM) antibodies was retrospectively demonstrated by indirect fluorescence. Despite immunosuppressive regimen, the disease progressed to end-stage renal failure (ESRF). Transplantation was not associated with recurrence in the kidney graft. We reviewed the 11 previously reported cases of IgA-mediated GP.

No MeSH data available.


Related in: MedlinePlus

IgA-mediated anti-GBM disease. (A) Light microscopy: segmental crescentic glomerulonephritis. (B)–(D) Immunofluorescence microscopy: (B) intense and linear diffuse deposits along the GBM for IgA, (C) mild linear deposits along the GBM for IgG and (D) weak deposits with granular staining for C3.
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SFS087F1: IgA-mediated anti-GBM disease. (A) Light microscopy: segmental crescentic glomerulonephritis. (B)–(D) Immunofluorescence microscopy: (B) intense and linear diffuse deposits along the GBM for IgA, (C) mild linear deposits along the GBM for IgG and (D) weak deposits with granular staining for C3.

Mentions: A kidney biopsy was performed (Figure 1). Pathological examination showed crescentic glomerulonephritis with both cellular and fibrocellular crescents and Bowman capsule's rupture. Immunofluorescence microscopy showed an intense linear deposition along the GBM with anti-alpha, anti-kappa and anti-lambda sera. Staining with anti-gamma revealed a similar but weak pattern. These results prompted us to check for circulating IgA anti-GBM: indirect fluorescence on macaque kidney slices using anti-IgA as secondary antibodies demonstrated circulating IgA anti-GBM antibodies. The titre was elevated at 1:80.Fig. 1.


IgA-mediated anti-glomerular basement membrane disease: an uncommon mechanism of Goodpasture's syndrome.

Moulis G, Huart A, Guitard J, Fortenfant F, Chauveau D - Clin Kidney J (2012)

IgA-mediated anti-GBM disease. (A) Light microscopy: segmental crescentic glomerulonephritis. (B)–(D) Immunofluorescence microscopy: (B) intense and linear diffuse deposits along the GBM for IgA, (C) mild linear deposits along the GBM for IgG and (D) weak deposits with granular staining for C3.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4400543&req=5

SFS087F1: IgA-mediated anti-GBM disease. (A) Light microscopy: segmental crescentic glomerulonephritis. (B)–(D) Immunofluorescence microscopy: (B) intense and linear diffuse deposits along the GBM for IgA, (C) mild linear deposits along the GBM for IgG and (D) weak deposits with granular staining for C3.
Mentions: A kidney biopsy was performed (Figure 1). Pathological examination showed crescentic glomerulonephritis with both cellular and fibrocellular crescents and Bowman capsule's rupture. Immunofluorescence microscopy showed an intense linear deposition along the GBM with anti-alpha, anti-kappa and anti-lambda sera. Staining with anti-gamma revealed a similar but weak pattern. These results prompted us to check for circulating IgA anti-GBM: indirect fluorescence on macaque kidney slices using anti-IgA as secondary antibodies demonstrated circulating IgA anti-GBM antibodies. The titre was elevated at 1:80.Fig. 1.

Bottom Line: Despite immunosuppressive regimen, the disease progressed to end-stage renal failure (ESRF).Transplantation was not associated with recurrence in the kidney graft.We reviewed the 11 previously reported cases of IgA-mediated GP.

View Article: PubMed Central - PubMed

Affiliation: Néphrologie et Immunologie Clinique , CHU Rangueil , Toulouse , France ; Université de Toulouse , Toulouse , France ; Centre de Référence des Maladies Rénales Rares, Toulouse , France.

ABSTRACT
Goodpasture's (GP) disease is usually mediated by IgG autoantibodies. We describe a case of IgA-mediated GP, in a patient presenting with isolated rapidly progressive glomerulonephritis. The diagnosis was established on kidney biopsy, since routine enzyme-linked immunosorbent assay (ELISA) targeted at IgG circulating autoantibodies failed to detect the nephritogenic antibodies. Immunofluorescence microscopy showed intense linear deposition of IgA along the glomerular capillary walls. An elevated titre (1:80) of circulating IgA anti-glomerular basement membrane (GBM) antibodies was retrospectively demonstrated by indirect fluorescence. Despite immunosuppressive regimen, the disease progressed to end-stage renal failure (ESRF). Transplantation was not associated with recurrence in the kidney graft. We reviewed the 11 previously reported cases of IgA-mediated GP.

No MeSH data available.


Related in: MedlinePlus