Alexandra Audemard-Verger1, Evangéline Pillebout2, Zahir Amoura3, Patrice Cacoub4,5, Noémie Jourde-Chiche6, Bertrand Lioger7, Nihal Martis8, Guillaume Moulis9, Etienne Rivière10, Aurélie Baldolli11, Charlotte Girard12, Julie Goutte13, Noémie Le Gouellec14, Loïc Raffray15, Geoffrey Urbanski16, Sébastien Sanges17,18, Francois Maurier19, Eric Thervet20,21, Achille Aouba22, Loïc Guillevin3,21,23, Francois Maillot1, Benjamin Terrier3,21,23. 1. Department of Internal Medicine and Clinical Immunology, CHRU Tours, University of Tours, Tours. 2. Department of Nephrology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris (AP-HP). 3. Department of Internal Medicine. 4. Department of Internal Medicine and Clinical Immunology, Hôpital Pitié-Salpétrière. 5. Inflammation-Immunopathology-Biotherapy Department (DHU i2B), UMR 7211, UPMC Université Paris 06, Sorbonne Universités, Paris. 6. Department of Nephrology, CHU, Marseille. 7. Department of Internal Medicine, Hopital Saint Louis, APHP, Paris. 8. Department of Internal Medicine, CHU, Nice. 9. Department of Internal Medicine, CHU, Toulouse. 10. Department of Internal Medicine, CHU, Bordeaux. 11. Department of infectious Disease, CHU, Caen. 12. Department of Internal Medicine, CHU Edouard Herriot, Lyon. 13. Department of Internal Medicine, CHU, Saint-Etienne. 14. Department of Internal Medicine and Nephrology, CH, Valenciennes. 15. Department of Internal Medicine, CHU, La Réunion. 16. Department of Internal Medicine, CHU, Angers, France. 17. Département de Médecine Interne et Immunologie Clinique, CHU. 18. University Lille, INSERM, U995-LIRIC-Lille Inflammation Research International Centre, Lille. 19. Department of Internal Medicine, Hôpitaux Privés, Metz. 20. Department of Nephrology, Hôpital Européen Georges Pompidou, AP-HP. 21. Université Paris Descartes. 22. Department of Internal Medicine, CHU, Caen. 23. National Referral Centre for Systemic and Autoimmune Diseases, Hôpital Cochin, Paris, France.
Abstract
OBJECTIVES: To describe the clinical presentation, treatments and prognosis of gastrointestinal (GI) involvement in adult IgA vasculitis (IgAV). METHODS: Data from 260 adults with IgAV included in a French multicentre retrospective survey were analysed. Presentation and outcomes of patients with (GI+) and without (GI-) GI involvement were compared. RESULTS: One hundred and thirty-seven (53%) patients had GI involvement. Initial manifestations were abdominal pain in 99%, intestinal bleeding in 31%, diarrhoea in 26% and acute surgical abdomen in only 4%. Abdominal imaging revealed thickening of intestinal wall in 61%, and endoscopies revealed abnormalities in 87%, mostly mucosal ulcerations. GI+ vs GI- patients were younger (46 ± 18 vs 54 ± 18 years; P = 0.0004), had more constitutional symptoms (43% vs 23%; P = 0.0005) and joint involvement (72 vs 50%; P = 0.0002), and higher CRP levels (3.7 vs 1.9 mg/dl; P = 0.001). Clinical response and relapse rates were comparable between groups, and all causes mortality (2 vs 4%) and IgAV-related mortality (1% vs 2%) as well. GI-related deaths were due to intestinal perforation and mesenteric ischaemia. CONCLUSION: GI involvement is frequent in adult IgAV. GI involvement is frequent in adult IgAV. Mortality is not uncommon but does not seem to be specifically related to GI. Immunosuppressants should not be preferred as first-line therapy for GI+ patients but may be required in case of acute surgical abdomen.
OBJECTIVES: To describe the clinical presentation, treatments and prognosis of gastrointestinal (GI) involvement in adult IgA vasculitis (IgAV). METHODS: Data from 260 adults with IgAV included in a French multicentre retrospective survey were analysed. Presentation and outcomes of patients with (GI+) and without (GI-) GI involvement were compared. RESULTS: One hundred and thirty-seven (53%) patients had GI involvement. Initial manifestations were abdominal pain in 99%, intestinal bleeding in 31%, diarrhoea in 26% and acute surgical abdomen in only 4%. Abdominal imaging revealed thickening of intestinal wall in 61%, and endoscopies revealed abnormalities in 87%, mostly mucosal ulcerations. GI+ vs GI- patients were younger (46 ± 18 vs 54 ± 18 years; P = 0.0004), had more constitutional symptoms (43% vs 23%; P = 0.0005) and joint involvement (72 vs 50%; P = 0.0002), and higher CRP levels (3.7 vs 1.9 mg/dl; P = 0.001). Clinical response and relapse rates were comparable between groups, and all causes mortality (2 vs 4%) and IgAV-related mortality (1% vs 2%) as well. GI-related deaths were due to intestinal perforation and mesenteric ischaemia. CONCLUSION: GI involvement is frequent in adult IgAV. GI involvement is frequent in adult IgAV. Mortality is not uncommon but does not seem to be specifically related to GI. Immunosuppressants should not be preferred as first-line therapy for GI+ patients but may be required in case of acute surgical abdomen.
Authors: Brenna G Kelly; Delaney B Stratton; Iyad Mansour; Bekir Tanriover; Keliegh S Culpepper; Clara Curiel-Lewandrowski Journal: JAAD Int Date: 2022-06-13