Ines Elhani1, Evangéline Pillebout2, Benjamin Terrier3, Antoine Hankard1, François Vrtovsnik4, Noémie Jourde-Chiche5, Sophie Greillier5, Matthieu Groh6, Nabil Belfeki7, Adrien Bigot8, Hubert de Boysson1, Georges-Philippe Pageaux9, Loïc Raffray10, Geoffrey Urbanski11, Isabelle Ollivier12, Francois Maillot8, Achille Aouba1, Alexandra Audemard-Verger13. 1. I. Elhani, MD, A. Hankard, MD, H. de Boysson, MD, A. Aouba, MD, Department of Internal Medicine, Caen, Normandie Université, UNICAEN, CHU de Caen Normandie. 2. E. Pillebout, MD, Department of Nephrology, Hôpital Saint Louis, AP-HP, Paris. 3. B. Terrier, MD, Université Paris Descartes, and Department of Internal Medicine, Hôpital Cochin, and National Referral Center for Systemic and Autoimmune Diseases, Hôpital Cochin, Paris. 4. F. Vrtovsnik, MD, Department of Nephrology, Bichat-Claude Bernard Hospital, AP-HP, and Inserm U1149 - CRI, University of Paris, Paris. 5. N. Jourde-Chiche, MD, S. Greillier, MD, Aix-Marseille Univ, C2VN, INSERM, INRA, Centre de Néphrologie et Transplantation Rénale, CHU de la Conception, AP-HM, Marseille. 6. M. Groh, MD, Department of Internal Medicine, National Referral Center for Hypereosinophilic Syndrome (CEREO), Suresnes. 7. N. Belfeki, MD, Department of Internal Medicine, Groupe Hospitalier Sud Ile de France, Melun. 8. A. Bigot, MD, F. Maillot, MD, A. Audemard-Verger, MD, PhD, Department of Internal Medicine and Clinical Immunology, CHRU Tours, and University of Tours, Tours. 9. G.P. Pageaux, MD, Liver Transplantation Unit, Digestive Department, Saint Eloi University Hospital, University of Montpellier, Montpellier. 10. L. Raffray, MD, Department of Internal Medicine, Centre Hospitalier Universitaire de la Réunion, Réunion. 11. G. Urbanski, MD, Department of Internal Medicine, Centre Hospitalier Universitaire d'Angers, Angers. 12. I. Ollivier, MD, Department of Hepato-Gastroenterology and Nutrition, Caen University Hospital, Caen, France. 13. A. Bigot, MD, F. Maillot, MD, A. Audemard-Verger, MD, PhD, Department of Internal Medicine and Clinical Immunology, CHRU Tours, and University of Tours, Tours; a.audemard-verger@chu-tours.fr.
Abstract
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.
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 patientsdied, 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.