IgA Nephropathies
Journal
Autoantibodies, Third Edition
ISBN
9780444563781
Date Issued
2014-01-01
Author(s)
Abstract
IgA nephropathy (IgAN) is the most common glomerulonephritis in the world. The hallmark of IgAN is underglycosylation in the hinge region of IgA1. Increasing evidence supports the underglycosylated IgA-containing immune complex, including that IgG antibodies against the glycans of the hinge region of IgA1 are key factors for mesangial deposition and then trigger inflammation and glomerular injury. The presence of aberrantly glycosylated IgA1 (first hit) alone is insufficient to cause IgAN. A second “hit” or multiple processes are required to establish permanent renal injury. Genetic predisposition influences the expression of these hit steps. The polymeric IgA is produced after aberrant mucosal IgA response. The displacement of mucosal B cells to systemic lymphoid organs and bone marrow may arise from abnormal trafficking of lymphocytes along the mucosa-bone marrow axis, involving changes of chemokines and adhesion molecules. This review summarizes the work on the IgA immune response, the mechanism of underglycosylation of IgA1, and the pathological effect of mesangial IgA deposition in IgAN. A greater understanding of the pathogenesis will provide a better approach for diagnosis and the development of novel disease-specific therapy.
Subjects
Berger disease | glomerulonephritis | IgA | IgA nephropathy | immune complex
Type
book part