IgA Vasculitis with Underlying Liver Cirrhosis: a French Nationwide Case Series of 20 Patients.

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IgA 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.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).Twenty patients were included, and seven (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 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 non-alcoholic steato-hepatitis (n=2), chronic viral hepatitis (n=1), haemochromatosis (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 death was related to IgAV.We reported the first case series of IgAV patients with underlining cirrhosis, which was mainly alcohol-related. The liver disease did not seem to impact baseline vasculitis' characteristic. Physicians may investigate the existence of liver cirrhosis at IgAV diagnosis, especially in the context of alcohol abuse.


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