IgA nephropathy

A vasculitis?

Norella C T Kong

    Research output: Contribution to journalArticle

    Abstract

    Vasculitis is an inflammation of blood vessels which leads to necrosis and infarction of the endorgans involved. IgA nephropathy (IgAN) is the kidney-limited expression of a single disease with HenochSchonlei purpura as the systemic vasculitic form. Renal histology is indistinguishable except for the chronic progressive nature of IgAN, in 20-30% of patients. A common immunopathogenesis occurs in which a genetic abnormality enhances lgh immune response to exogeneous (and endogenous) antigens in mucosae with the excessive production of macromolecular IgA1. Renal damage is triggered by mesangial deposits of IgA1. Both are associated with chronic liver disease, gut neoplasms and lymphomas and IgAN, in particular with autoimmune diseases such as dermatitis herpetiformes, coeliac disease, ankylosing spondylitis and Reiter's disease. Immunodysregulation with reduced T suppressor cell function and enhanced T helper cell function with resultant autoantibodies is highly reminiscent of systemic lupus erythematosus (SLE). The many autoantibodies and characteristic vasculitic lesions of SLE are well documented. Autoantibodies have been demonstrated in IgAN and include cold-reactive antinuclear antibodies (ANA), IgA-rheumatoid factor, IgA-antinuclear cytoplasmic antibody, anti-endothelial cell antibody (AECA) and IgA.fibrinonectin. Their clinical significance remains controversial. Extrarenal involvement of the skin, eyes and joints occasionally occurs, although systemic capillaritis is rarely reported. It is tempting to speculate that the severe renal biopsy changes of necrosis, polymorph infiltrate and crescent formation occurring in a third of IgAN patients reflect true glomerular capillaritis. Nevertheless, on the balance of current evidence, IgAN cannot be classified as a vasculitis.

    Original languageEnglish
    Pages (from-to)23-26
    Number of pages4
    JournalNephrology
    Volume3
    Issue number1
    Publication statusPublished - 1997

    Fingerprint

    Vasculitis
    Immunoglobulin A
    Autoantibodies
    Kidney
    Antinuclear Antibodies
    Systemic Lupus Erythematosus
    Necrosis
    Reactive Arthritis
    Purpura
    Rheumatoid Factor
    Ankylosing Spondylitis
    Celiac Disease
    Dermatitis
    Helper-Inducer T-Lymphocytes
    Infarction
    Autoimmune Diseases
    Blood Vessels
    Liver Diseases
    Lymphoma
    Histology

    Keywords

    • autoantibodies
    • Henoch-Schonlein purpura
    • immune regulation

    ASJC Scopus subject areas

    • Nephrology

    Cite this

    Kong, N. C. T. (1997). IgA nephropathy: A vasculitis? Nephrology, 3(1), 23-26.

    IgA nephropathy : A vasculitis? / Kong, Norella C T.

    In: Nephrology, Vol. 3, No. 1, 1997, p. 23-26.

    Research output: Contribution to journalArticle

    Kong, NCT 1997, 'IgA nephropathy: A vasculitis?', Nephrology, vol. 3, no. 1, pp. 23-26.
    Kong NCT. IgA nephropathy: A vasculitis? Nephrology. 1997;3(1):23-26.
    Kong, Norella C T. / IgA nephropathy : A vasculitis?. In: Nephrology. 1997 ; Vol. 3, No. 1. pp. 23-26.
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