Journal Article

Immune complexes in acute adult-onset Henoch–Schönlein nephritis

Marc Hilhorst, Pieter van Paassen, Peter van Breda Vriesman and Jan Willem Cohen Tervaert

in Nephrology Dialysis Transplantation

Published on behalf of European Renal Association - European Dialysis and Transplant Assoc

Volume 26, issue 12, pages 3960-3967
Published in print December 2011 | ISSN: 0931-0509
Published online March 2011 | e-ISSN: 1460-2385 | DOI:
Immune complexes in acute adult-onset Henoch–Schönlein nephritis

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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. Serum was analysed for circulating IgA immune complexes; renal biopsies were analysed by light and electron microscopy (EM). As disease controls, 40 IgAN patients were studied.

Results. Intracapillary leukocyte margination was seen in 15 of the 20 patients and cellular crescent formation in all renal biopsies of the HSPN patients. IgAN biopsies showed a few small crescents without intracapillary leukocytes. In 16 HSPN patients, EM was performed and in 10, no dense deposits were found. In all biopsies of IgAN patients, typical ‘humps’ were found. In 6 of 9 analysed HSPN patients, intermediate to large circulating immune complexes were found, whereas in 4 of 28 analysed patients with primary IgAN small circulating immune complexes were found.

Conclusions. We consider adult-onset HSPN distinguishable in histology and ultrastructure from primary IgAN. We believe adult-onset Henoch–Schönlein to be a circulating immune complex disease.

Keywords: electron microscopy; glomerulonephritis; Henoch–Schönlein purpura; IgA nephropathy; immune complexes

Journal Article.  5099 words.  Illustrated.

Subjects: Nephrology

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