TY - JOUR T1 - IgA Vasculitis with Underlying Liver Cirrhosis: A French Nationwide Case Series of 20 Patients JF - The Journal of Rheumatology JO - J Rheumatol DO - 10.3899/jrheum.200293 SP - jrheum.200293 AU - Ines Elhani AU - Evangéline Pillebout AU - Benjamin Terrier AU - Antoine Hankard AU - François Vrtovsnik AU - Noémie Jourde-Chiche AU - Sophie Greillier AU - Matthieu Groh AU - Nabil Belfeki AU - Adrien Bigot AU - Hubert de Boysson AU - Georges-Philippe Pageaux AU - Loïc Raffray AU - Geoffrey Urbanski AU - Isabelle Ollivier AU - Francois Maillot AU - Achille Aouba AU - Alexandra Audemard-Verger Y1 - 2020/08/15 UR - http://www.jrheum.org/content/early/2021/01/25/jrheum.200293.abstract N2 - Objective Immunoglobulin A vasculitis (IgAV) and nephropathy (IgAN) share common immunological mechanisms. Liver cirrhosis is well known to be associated with IgAN. Here, we aimed to describe the presentation and outcome of IgAV patients with underlying cirrhosis. Methods We conducted a French nationwide retrospective study of adult patients presenting with both IgAV and cirrhosis. Baseline characteristics were compared to those of the 260 patients included in the French nationwide IgAV registry (IGAVAS). Results Twenty patients were included, and 7 (35%) were female. The mean ± SD age was 62.7 ± 11 years. At baseline, compared with IGAVAS patients, patients with underlying cirrhosis were older (62.7 ± 11 vs 50.1 ± 18, P < 0.01) and displayed more constitutional symptoms (weight loss 25% vs 8%, P = 0.03). Patients with underlying cirrhosis were also more likely to exhibit elevated serum IgA levels (5.6 g/L vs 3.6 g/L, P = 0.02). Cirrhosis and IgAV were diagnosed simultaneously in 12 patients (60%). Cirrhosis was mainly related to alcohol intake (n = 15, 75%), followed by nonalcoholic steato-hepatitis (n = 2), chronic viral hepatitis (n = 1), hemochromatosis (n = 1), and autoimmune hepatitis (n = 1). During follow-up with a median of 17 months (IQR 12–84), 10/13 (77%) exhibited IgAV remission at Month 3. One patient presented a minor relapse. Six patients died, but no deaths were related to IgAV. Conclusion We report the first case series of IgAV patients with underlining cirrhosis, to our knowledge, which was mainly alcohol related. The liver disease did not seem to affect baseline vasculitis characteristics. Physicians should investigate the existence of liver cirrhosis at IgAV diagnosis, especially in the context of alcohol abuse. ER -