#1993 Recommendations on management of adult-onset IgA vasculitis proposed by the European IgA vasculitis study group

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Abstract Background and Aims IgA vasculitis (IgAV) is a small-vessel vasculitis with predominant IgA deposits that involves the skin, the joints, the gastrointestinal tract and the kidneys, causing glomerulonephritis pathologically indistinguishable from IgA nephropathy [1]. Adults are less frequently affected than children but more often experience a relapsing/refractory course with severe kidney involvement [2]. As the treatment of this condition remains controversial, especially for adults, we have been working on developing recommendations for the management of adult-onset IgAV. Here, we report on the progress of this initiative with a focus on kidney involvement. Method The European IgA Vasculitis Study Group (EUGAVAS) is a multidisciplinary group of experts in the field of IgAV established in 2023. Members were initially asked to vote on key questions using a Delphi approach and questions that achieved a level of agreement ≥70% drove a systematic literature review (SLR) on Medline/Pubmed, Cochrane and Embase databases. Small working groups of 4–5 members drafted recommendation statements based on the SLR results and, where required, expert opinion. The preliminary statements were then discussed and amended by the whole group and are currently being voted on in the second Delphi round. The initiative was endorsed by the European Vasculitis Society (EUVAS). Results EUGAVAS consists of 38 experts, including 11 nephrologists, 10 internists/rheumatologists, four dermatologists, four pediatricians, four fellows, two pathologists, one representative IgAV patient, one vasculitis nurse, and one methodologist. Following the first Delphi round, 14 out of 16 key questions were retained. The SLR identified 335 relevant publications out of 3,784 abstracts reviewed (1,058 excluded as duplicates). The group agreed that the level of evidence available in the literature was insufficient to establish guidelines and drafted recommendation statements. Of the 14 preliminary statements, three focus on diagnosis and classification, three on disease staging, six on treatment, including general and organ-specific principles, and two on disease assessment and patient follow-up. Four statements are specifically dedicated to kidney involvement and discuss: 1) definition of kidney involvement and role of baseline prognostic factors; 2) indications to kidney biopsy and histological classification; 3) goals of treatment and options according to severity of kidney involvement; 4) role of nephroprotective measures. Kidney involvement is also discussed in other statements on definition of response and remission, use of biologic therapies, such as rituximab, and frequency and modality of patient follow-up. Conclusion A set of recommendations on management of adult-onset IgAV has been developed following a standardized approach and will soon be available for communication. The result of this initiative may help clinicians, especially nephrologists, manage patients with this condition and improve their prognosis.