Alexandra Audemard-Verger, Evangéline Pillebout, Eva Baier, Ingeborg M Bajema, Jonathan Barratt, George Bertsias, Annette Bruchfeld, Santos Castañeda, Maria C Cid, Rosanna Coppo, Haner Direskeneli, Carmela Errichiello, Jürgen Floege, Miguel A González-Gay, Ahmet Gül, Antoine Hankard, Alojzija Hočevar, Marie Jachiet, Marija Jelusic, Noémie Jourde-Chiche, Alfred D Mahr, Thomas Neumann, Louise Oni, Seza Özen, Alan D Salama, Cord Sunderkötter, Benjamin Terrier, Giorgio Trivioli, David Jayne, Augusto Vaglio, collaborators of the EUGAVAS Study Group on behalf of the European Vasculitis Society (EUVAS)
IgA vasculitis (IgAV) is an immune complex-mediated small-vessel vasculitis that typically affects the skin, gastrointestinal tract, kidneys and joints. Childhood-onset IgAV is a common disease and usually follows a self-limiting course, whereas adult-onset IgAV is considerably less frequent and is associated with a poorer prognosis. The diagnosis, assessment and management of adult-onset IgAV remain challenging owing to the absence of validated diagnostic criteria for adults and lack of IgAV-specific standardized disease activity scores. Short-term outcomes are mainly determined by gastrointestinal complications, whereas kidney involvement and the risk of progression to chronic kidney disease are the major determinants of long-term prognosis. The treatment of adult-onset IgAV is limited by the paucity of high-quality clinical trials and standardized therapeutic approaches. Here, we present evidence-based, multidisciplinary guidelines for the diagnosis and management of adult-onset IgAV that reflect advances in understanding of pathogenesis, differential diagnoses and treatment over the past two decades. Developed by a panel of leading European experts on the basis of systematic literature review and expert opinion, these guidelines comprise 14 recommendation statements and overarching principles that provide a structured and pragmatic clinical framework for the diagnosis, treatment and follow-up of adult-onset IgAV.