IgA Vasculitis Across the Ages: Is It Time for a Precision Medicine Approach?

Abstract

IgA vasculitis (IgAV; formerly Henoch-Schönlein purpura) is a systemic small vessel vasculitis most commonly affecting the skin, gut, joints, and kidneys. Nephritis is the most concerning complication for all ages because it carries the risk of progression to irreversible end-stage kidney failure. The multiorgan nature of the disease, variation in clinical presentation, and unpredictable disease course pose a substantial challenge to timely diagnosis, risk stratification, and a unified approach to management. Precision medicine is defined as a health care approach that uses genetic and molecular profiling alongside phenotypic and environmental data to generate insights to prevent or treat disease. This review article aims to provide an overview of IgAV using the latest literature to highlight three key areas in which precision medicine may have a role in advancing patient outcomes. These three areas are as follows: early phenotyping, risk stratification, and evidence-based management. Due to the presence of nephritis bringing the greatest risk of morbidity and mortality for patients with this disease, kidney involvement forms the focal point of this review. Like other forms of glomerulonephritis, there are defined stages, from diagnosis to early signs of nephritis, histologically established nephritis, chronic kidney disease, and ultimately kidney failure. Advancing a disease for which there has been very little progress provides a huge opportunity to incorporate precision medicine from the outset to augment traditional monitoring and provide more rapid evidence generation. With transformative treatments on the horizon for IgA-related glomerular diseases, improving patient outcomes to prevent kidney failure in IgAV is becoming a near reality.