RT Journal Article
SR Electronic
T1 IgA Vasculitis with Underlying Liver Cirrhosis: A French Nationwide Case Series of 20 Patients
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP jrheum.200293
DO 10.3899/jrheum.200293
A1 Ines Elhani
A1 Evangéline Pillebout
A1 Benjamin Terrier
A1 Antoine Hankard
A1 François Vrtovsnik
A1 Noémie Jourde-Chiche
A1 Sophie Greillier
A1 Matthieu Groh
A1 Nabil Belfeki
A1 Adrien Bigot
A1 Hubert  de Boysson
A1 Georges-Philippe Pageaux
A1 Loïc Raffray
A1 Geoffrey Urbanski
A1 Isabelle Ollivier
A1 Francois Maillot
A1 Achille Aouba
A1 Alexandra Audemard-Verger
YR 2020
UL http://www.jrheum.org/content/early/2021/01/25/jrheum.200293.abstract
AB 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.