IgA Vasculitis with necrotizing arteritis: a multicenter retrospective study from the French Vasculitis Study Group and systematic review of the literature

Clinical Snapshot

Key Findings

• P Value: Significance levels not specified

• Effect Size: Higher rates of multi-organ involvement and mortality compared to classical IgAV

• Primary Outcome: IgAV-NA characterized as severe phenotype with life-threatening complications

• Nnt Or Sensitivity: Median age 54.5 years; 16/30 biopsy-confirmed, 14/30 imaging-confirmed necrotizing arteritis

• Confidence Interval: Not reported

Clinical Application

Requires access to vascular imaging and specialized vasculitis expertise for diagnosis and management Relevant to Australian rheumatology and immunology practice; aligns with RACGP chronic disease management principles; may impact PBS immunosuppressive therapy decisions Adult patients with IgA vasculitis, particularly those with severe or atypical presentations

Abstract

IgA vasculitis (IgAV) primarily affects small vessels, but rare cases with necrotizing arteritis (NA) raise questions about overlap with polyarteritis nodosa (PAN). To characterize IgAV with necrotizing arteritis (IgAV-NA) and compare its phenotype with classical IgAV and PAN. We performed a multicenter retrospective study combined with a systematic literature review (1990-2025). Patients fulfilled EULAR/PRINTO/PRES IgAV criteria, had pathological or imaging evidence of NA in small or medium arteries, and were ANCA-negative. Thirty patients were included (7 from databases, 23 from the literature). NA was confirmed by biopsy (n = 16) or vascular imaging (n = 14). Clinical features, treatments, remission, and mortality were compared with 257 adult IgAV and 196 PAN patients. Median age was 54.5 years. IgAV-NA was characterized by severe manifestations, including gastrointestinal bleeding, perforation, surgical abdomen, neuropathy, pancreatitis, and livedo. Compared with classical IgAV, IgAV-NA showed significantly higher rates of multi-organ involvement and mortality. Compared with PAN, IgAV-NA shared vascular complications but had less fever and neuropathy. Despite arterial involvement, patients did not fulfil PAN criteria. IgAV-NA represents a rare, severe IgAV phenotype with life-threatening complications rather than an IgAV-PAN overlap. Severe or atypical IgAV presentations should prompt vascular imaging and intensified immunosuppression.

References

1. 1.Ribereau-Gayon, E., Carbonnelle-Puscian, A., Ottavi, M., Donzel, M., Puéchal, X., Sundekötter, C., Audemard-Verger, A., Rohmer, J., Balme, B., Fauquier, A., Grienay-Poletto, N., Novel-Catin, E., Liegeon, A.-L., Chollat-Namy, A., Terrier, B., & Durel, C.-A. (2026). IgA Vasculitis with necrotizing arteritis: a multicenter retrospective study from the French Vasculitis Study Group and systematic review of the literature. *Autoimmunity*. https://doi.org/10.1080/08916934.2026.2677546