Abstract
The understanding of the pathogenesis of any disease is the key to effective and specific treatment of the disease. immunoglobulin A (IgA) nephropathy is an autoimmune disease of the kidney. Oxford MEST classification is commonly used to stratify patients according to the severity of the disease. Patients with IgA nephropathy seem to produce anti-GalNAc antibodies against a particularly defective IgA1. This immune complex deposits in the kidneys, leading to a type 3 hypersensitivity reaction which ultimately damages the kidneys. People of a certain genetic background and who experience upregulation of certain defective receptors seem to develop primary IgA nephropathy. Secondary IgA nephropathy could be due to dysbiosis of the microbiota in the gut, compromised gut immunity or other gut pathologies, pulmonary function abnormalities, or amyloidosis. Overproduction of IgA due to plasma cell dyscrasia or reduced clearance of IgA due to liver abnormalities could also be potential causes. Genes that predispose individuals to IgA nephropathy and intestinal abnormalities, such as Celiac disease, seem to overlap and these people tend to have a poorer prognosis and need to be placed on more intensive treatment regimens. IgA Vasculitis seems to be a systemic form of IgA nephropathy, whereby IgA deposits systemically and leads to multiple disease manifestations. Patients in high-risk groups could also be prophylactically screened for the disease and closely monitored by immunohistochemical methods such as an enzyme-linked immunosorbent assay (ELISA) or identified by genetic testing. Currently, the major treatment regimens involve supportive therapy or immunosuppressive therapy which has major side effects. More specific treatment methods such as monoclonal antibodies, immunoglobulin replacement therapy, or low-antigen-content diet could also be looked into as potential treatment options. Stem cell replacement, by way of bone marrow transplant and tonsillectomy, has been suggested as a treatment option in patients with indications.
Highlights
Immunoglobin A nephropathy (IgAN), known as Berger’s disease, is an autoimmune disease characterized by immunoglobulin A (IgA) deposits in the kidney which leads to inflammation and damage of the kidney
Exogenous microbial antigens seem to activate TLR9 in the mucosa which activates the B cells in the tonsils, which in turn leads to the production of nephritogenic GdIgA1, especially after insult to the tonsils such as an infection [44]
This leads us to believe that IgA nephropathy manifests as a secondary disease in many cases and treatment of the primary disease would automatically cure the IgA nephropathy
Summary
Immunoglobin A nephropathy (IgAN), known as Berger’s disease, is an autoimmune disease characterized by IgA deposits in the kidney which leads to inflammation and damage of the kidney. Issues with mucosal antigen exclusion and systemic hypersensitivity (hyperresponsiveness) in primary IgA nephropathy make antibodies against food antigen This triggers patients who are already susceptible to Celiac disease, which further increases IgA and increases mesangial deposition of IgA. Exogenous microbial antigens seem to activate TLR9 in the mucosa which activates the B cells in the tonsils, which in turn leads to the production of nephritogenic GdIgA1, especially after insult to the tonsils such as an infection [44] This leads us to believe that IgA nephropathy manifests as a secondary disease in many cases and treatment of the primary disease would automatically cure the IgA nephropathy. There has been a case that has been reported of a patient with complete remission of IgA nephropathy after bone marrow transplant [50]
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