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

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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.

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Authors and Affiliations

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

    Antoine Hankard, Gwénola Maigné, Hubert De Boysson & Achille Aouba

  2. Department of Drug Development (DITEP), Gustave Roussy, Villejuif, France

    Jean-Marie Michot

  3. Université Paris Descartes, Paris, France

    Benjamin Terrier

  4. Department of Internal Medicine, Hôpital Cochin, Paris, France

    Benjamin Terrier

  5. National Referral Center for Systemic and Autoimmune Diseases, Hôpital Cochin, Paris, France

    Benjamin Terrier

  6. Department of Internal Medicine, CH, Saint-Quentin, France

    Benoît Brihaye

  7. Department of Dermatology, Hôpital Cochin, AP-HP, Paris, France

    Johan Chanal

  8. Department of Nephrology, CHU, Bordeaux, France

    Christian Combe & Florent Guerville

  9. Department of Nephrology, Hôpital Européen Georges Pompidou, AP-HP, Paris, France

    Alexandre Karras

  10. Department of Internal Medicine, CHU, Angers, France

    Geoffrey Urbanski

  11. Department of Internal Medicine 2, Pitie-Salpetriere Hospital, Paris, France

    Zahir Amoura

  12. Department of Dermatology, CHU, Bordeaux, France

    Anne-Sophie Darrigade

  13. Department of Internal Medicine, CHU, Grenoble, France

    Alban Deroux

  14. Department of Internal Medicine, Hôpital Bicêtre, AP-HP, Paris, France

    Le Sébastien Burel

  15. Department of Internal Medicine, Hôpital Saint-Antoine AP-HP, Paris, France

    Arsène Mekinian

  16. Department of Internal Medicine, CHU, Toulouse, France

    Guillaume Moulis

  17. Department of Internal Medicine, CHU, Bordeaux, France

    Etienne Riviere

  18. Department of Dermatology, CHU, Angers, France

    Carole Vandamme-Giard

  19. Department of Internal Medicine and Clinical Immunology, CHRU Tours, Tours, France

    Francois Maillot & Alexandra Audemard-Verger

  20. University of Tours, Tours, France

    Francois Maillot & Alexandra Audemard-Verger

Authors
  1. Antoine Hankard
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  2. Jean-Marie Michot
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  3. Benjamin Terrier
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  4. Benoît Brihaye
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  5. Johan Chanal
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  6. Christian Combe
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  7. Alexandre Karras
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  8. Geoffrey Urbanski
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  9. Zahir Amoura
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  10. Anne-Sophie Darrigade
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  11. Alban Deroux
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  12. Florent Guerville
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  13. Le Sébastien Burel
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  14. Gwénola Maigné
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  15. Arsène Mekinian
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  16. Guillaume Moulis
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  17. Etienne Riviere
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  18. Carole Vandamme-Giard
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  19. Francois Maillot
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  20. Hubert De Boysson
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  21. Achille Aouba
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  22. Alexandra Audemard-Verger
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Consortia

on behalf of the French Vasculitis Study Group (FVSG)

Corresponding author

Correspondence to Alexandra Audemard-Verger.

Ethics declarations

The study was performed in accordance with ethical standards of the Helsinki Declaration and was approved by the Institutional Review Board.

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Hankard, A., Michot, JM., Terrier, B. et al. New insights on IgA vasculitis with underlying solid tumor: a nationwide French study of 30 patients. Clin Rheumatol 40, 1933–1940 (2021). https://doi.org/10.1007/s10067-020-05455-z

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  • DOI: https://doi.org/10.1007/s10067-020-05455-z

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