New insights on IgA vasculitis with underlying solid tumor: a nationwide French study of 30 patients

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ORIGINAL ARTICLE

New insights on IgA vasculitis with underlying solid tumor: a nationwide French study of 30 patients Antoine Hankard 1 & Jean-Marie Michot 2 & Benjamin Terrier 3,4,5 & Benoît Brihaye 6 & Johan Chanal 7 & Christian Combe 8 & Alexandre Karras 9 & Geoffrey Urbanski 10 & Zahir Amoura 11 & Anne-Sophie Darrigade 12 & Alban Deroux 13 & Florent Guerville 8 & Le Sébastien Burel 14 & Gwénola Maigné 1 & Arsène Mekinian 15 & Guillaume Moulis 16 & Etienne Riviere 17 & Carole Vandamme-Giard 18 & Francois Maillot 19,20 & Hubert De Boysson 1 & Achille Aouba 1 & Alexandra Audemard-Verger 19,20 & on behalf of the French Vasculitis Study Group (FVSG) Received: 7 May 2020 / Revised: 29 September 2020 / Accepted: 6 October 2020 # International League of Associations for Rheumatology (ILAR) 2020

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.

* Alexandra Audemard-Verger [email protected] 1

2

Department of Internal Medicine, Normandie University, UNICAEN, CHU de Caen Normandie, 14000 Caen, France 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

10

Department of Internal Medicine, CHU, Angers, France

11

Department of Internal Medicine 2, Pitie-Salpetriere