Antoine Hankard1, Jean-Marie Michot2, Benjamin Terrier3,4,5, Benoît Brihaye6, Johan Chanal7, Christian Combe8, Alexandre Karras9, Geoffrey Urbanski10, Zahir Amoura11, Anne-Sophie Darrigade12, Alban Deroux13, Florent Guerville8, Le Sébastien Burel14, Gwénola Maigné1, Arsène Mekinian15, Guillaume Moulis16, Etienne Riviere17, Carole Vandamme-Giard18, Francois Maillot19,20, Hubert De Boysson1, Achille Aouba1, Alexandra Audemard-Verger21,22. 1. Department of Internal Medicine, Normandie University, UNICAEN, CHU de Caen Normandie, 14000, Caen, France. 2. Department of Drug Development (DITEP), Gustave Roussy, Villejuif, France. 3. Université Paris Descartes, Paris, France. 4. Department of Internal Medicine, Hôpital Cochin, Paris, France. 5. National Referral Center for Systemic and Autoimmune Diseases, Hôpital Cochin, Paris, France. 6. Department of Internal Medicine, CH, Saint-Quentin, France. 7. Department of Dermatology, Hôpital Cochin, AP-HP, Paris, France. 8. Department of Nephrology, CHU, Bordeaux, France. 9. Department of Nephrology, Hôpital Européen Georges Pompidou, AP-HP, Paris, France. 10. Department of Internal Medicine, CHU, Angers, France. 11. Department of Internal Medicine 2, Pitie-Salpetriere Hospital, Paris, France. 12. Department of Dermatology, CHU, Bordeaux, France. 13. Department of Internal Medicine, CHU, Grenoble, France. 14. Department of Internal Medicine, Hôpital Bicêtre, AP-HP, Paris, France. 15. Department of Internal Medicine, Hôpital Saint-Antoine AP-HP, Paris, France. 16. Department of Internal Medicine, CHU, Toulouse, France. 17. Department of Internal Medicine, CHU, Bordeaux, France. 18. Department of Dermatology, CHU, Angers, France. 19. Department of Internal Medicine and Clinical Immunology, CHRU Tours, Tours, France. 20. University of Tours, Tours, France. 21. Department of Internal Medicine and Clinical Immunology, CHRU Tours, Tours, France. a.audemard-verger@chu-tours.fr. 22. University of Tours, Tours, France. a.audemard-verger@chu-tours.fr.
Abstract
OBJECTIVE: IgA vasculitis (IgAV) frequently occurs during or after a mucosal infection; it also rarely occurs in patients with cancer. We hypothesized that cancer could impact the baseline characteristics and/or outcome of vasculitis. We aimed to describe the presentation of IgAV in patients with cancer (IgAV ca+) compared to patients without cancer. METHODS: We conducted a nationwide retrospective study of adult patients in France who presented with both IgAV and cancer. Baseline characteristics were described and compared with those of the 260 patients included in a nationwide French IgAV study. RESULTS: Thirty patients were included. The mean age was 69 ± 12 years; 80% were men. Compared to patients without underlying cancer, IgAV ca+ patients were older (69 ± 12 vs. 50 ± 18 years; p < 0.0001) and they presented more frequently with necrotic purpura (53 vs. 26%; p < 0.002) and intra-alveolar hemorrhage (10 vs. 0.5%; p < 0.0001). IgAV ca+ patients frequently had elevated serum IgA levels (79 vs. 53%; p < 0.034); most (n = 22, 73%) had adenocarcinoma or urothelial carcinoma involving the large intestines (n = 6), bladder (n = 5), and lung (n = 5). Most IgAV ca+ patients had progressive cancer (n = 21); a minority had metastatic disease (n = 2) at IgAV diagnosis. After a median follow-up of 3 months, 8 deaths were observed but none was related to IgAV. CONCLUSION: Compared to their noncancer counterpart, patients with IgAV related to cancer were older and more frequently presented with necrotizing purpura, intra-alveolar hemorrhage, and elevated serum IgA levels. Adult patients with IgAV and these latter characteristics should be carefully screened for cancer. Key Points • Clinical and biological characteristics of patients presenting with IgAV are distinct depending on the underlying cause of vasculitis related to cancer. • Patients with IgAV related to cancer are older, and compared to their counterparts without IgAV, they present more frequently with necrotic purpura, alveolar hemorrhage, and elevated serum IgA levels. • All adult patients with IgAV should be screened for cancer, and there should be a focus on elderly male patients presenting with necrotic purpura and/or alveolar hemorrhage.
OBJECTIVE:IgAvasculitis (IgAV) frequently occurs during or after a mucosal infection; it also rarely occurs in patients with cancer. We hypothesized that cancer could impact the baseline characteristics and/or outcome of vasculitis. We aimed to describe the presentation of IgAV in patients with cancer (IgAV ca+) compared to patients without cancer. METHODS: We conducted a nationwide retrospective study of adult patients in France who presented with both IgAV and cancer. Baseline characteristics were described and compared with those of the 260 patients included in a nationwide French IgAV study. RESULTS: Thirty patients were included. The mean age was 69 ± 12 years; 80% were men. Compared to patients without underlying cancer, IgAV ca+ patients were older (69 ± 12 vs. 50 ± 18 years; p < 0.0001) and they presented more frequently with necrotic purpura (53 vs. 26%; p < 0.002) and intra-alveolar hemorrhage (10 vs. 0.5%; p < 0.0001). IgAV ca+ patients frequently had elevated serum IgA levels (79 vs. 53%; p < 0.034); most (n = 22, 73%) had adenocarcinoma or urothelial carcinoma involving the large intestines (n = 6), bladder (n = 5), and lung (n = 5). Most IgAV ca+ patients had progressive cancer (n = 21); a minority had metastatic disease (n = 2) at IgAV diagnosis. After a median follow-up of 3 months, 8 deaths were observed but none was related to IgAV. CONCLUSION: Compared to their noncancer counterpart, patients with IgAV related to cancer were older and more frequently presented with necrotizing purpura, intra-alveolar hemorrhage, and elevated serum IgA levels. Adult patients with IgAV and these latter characteristics should be carefully screened for cancer. Key Points • Clinical and biological characteristics of patients presenting with IgAV are distinct depending on the underlying cause of vasculitis related to cancer. • Patients with IgAV related to cancer are older, and compared to their counterparts without IgAV, they present more frequently with necrotic purpura, alveolar hemorrhage, and elevated serum IgA levels. • All adult patients with IgAV should be screened for cancer, and there should be a focus on elderly male patients presenting with necrotic purpura and/or alveolar hemorrhage.
Entities:
Keywords:
Cancer; Henoch-Schönlein purpura; IgA vasculitis; Malignancy; Neoplasia; Solid tumor
Authors: Brenna G Kelly; Delaney B Stratton; Iyad Mansour; Bekir Tanriover; Keliegh S Culpepper; Clara Curiel-Lewandrowski Journal: JAAD Int Date: 2022-06-13