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13-02-2018 | Immunoglobulin A vasculitis | Review | Article

IgA Vasculitis in Adults

Journal:
Current Treatment Options in Rheumatology

Authors: MB BCh BAO BMedSc MRCPI Sarah M. Moran, MD CM, PhD, FRCPC Heather N. Reich

Publisher: Springer International Publishing

Abstract

Immunoglobulin A vasculitis (IgAV) is a small vessel vasculitis with skin, joint, gastrointestinal and renal manifestations. Our understanding of the natural history of this disease is limited due to the overall low incidence of IgAV in adults and a lack of consensus regarding diagnostic criteria. In this review, we describe IgAV in the adult population, focusing on diagnostic and classification systems, and treatments strategies.
Recent data from larger longitudinal adult cohorts demonstrate that IgAV is associated with significant morbidity and mortality. Treatment regimen remains controversial but emerging retrospective observational data support potential benefit of immunosuppression. As illustrated in trials of IgA nephropathy, immunosuppression carries significant risks of toxicity.
Treatment regimen and selection of patients who will benefit from treatment remains challenging. Prospective treatment trials are needed to elucidate both the patient populations that will derive benefit and what treatment is most efficacious.

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