Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome

François-Xavier Danlos; Giovanni Maria Rossi; Daniel Blockmans; Giacomo Emmi; Andreas Kronbichler; Stéphane Durupt; Claire Maynard; Luminita Luca; Cyril Garrouste; Bertrand Lioger; Rachel Mourot-Cottet; Robin Dhote; Jean-Benoit Arlet; Thomas Hanslik; Philippe Rouvier; Mikael Ebbo; Xavier Puéchal; Dominique Nochy; Agnès Carlotti; Luc Mouthon; Loïc Guillevin; Augusto Vaglio; Benjamin Terrier; French Vasculitis Study Group

doi:10.1016/j.autrev.2017.07.020

Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome

Autoimmun Rev. 2017 Oct;16(10):1036-1043. doi: 10.1016/j.autrev.2017.07.020. Epub 2017 Aug 2.

Authors

François-Xavier Danlos¹, Giovanni Maria Rossi², Daniel Blockmans³, Giacomo Emmi⁴, Andreas Kronbichler⁵, Stéphane Durupt⁶, Claire Maynard⁷, Luminita Luca⁸, Cyril Garrouste⁹, Bertrand Lioger¹⁰, Rachel Mourot-Cottet¹¹, Robin Dhote¹², Jean-Benoit Arlet¹³, Thomas Hanslik¹⁴, Philippe Rouvier¹⁵, Mikael Ebbo¹⁶, Xavier Puéchal¹, Dominique Nochy¹⁷, Agnès Carlotti¹⁸, Luc Mouthon¹⁹, Loïc Guillevin¹, Augusto Vaglio², Benjamin Terrier²⁰; French Vasculitis Study Group

Affiliations

¹ Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
² Nephrology Unit, University Hospital, Parma, Italy.
³ Clinical Department of General Internal Medicine Department, Research Department of Microbiology and Immunology, Laboratory of Clinical Infectious and Inflammatory Disorders, University Hospitals Leuven, Leuven, Belgium.
⁴ Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
⁵ Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, Anichstraße 35, 6020 Innsbruck, Austria.
⁶ Department of Internal and Vascular Medicine, Hospices Civils de Lyon, Groupe Hospitalier Sud, Université de Lyon, Pierre-Bénite, France.
⁷ Department of Nephrology, Centre Hospitalier Métropole Savoie, Chambery, France.
⁸ Department of Internal Medicine, Hôpital de Poitiers, Poitiers, France.
⁹ Department of Nephrology, Clermont-Ferrand University Hospital, 58, rue Montalembert, 63000 Clermont-Ferrand, France.
¹⁰ Department of Internal Medicine, University Medical Center Tours, Tours, France.
¹¹ Department of Internal Medicine, CHRU de Strasbourg, 67000 Strasbourg, France.
¹² Department of Internal Medicine, Avicenne University Hospital, Bobigny, France.
¹³ Department of Internal Medicine, Hôpital Européen Georges Pompidou, AP-HP, Paris, France.
¹⁴ Department of Internal Medicine, University Hospital, AP-HP, Versailles Saint Quentin University, Boulogne-Billancourt, France.
¹⁵ Department of Pathology, Groupe Hospitalier Pitié Salpétrière, AP-HP, Paris, France.
¹⁶ Department of Internal Medicine, CHU Timone, Assistance Publique-Hôpitaux de Marseille, Marseille, France; Aix-Marseille Université, France.
¹⁷ Department of Pathology, Hôpital Européen Georges Pompidou, AP-HP, Paris, France.
¹⁸ Department of Pathology, Hôpital Cochin, AP-HP, Paris, France.
¹⁹ Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Université Paris Descartes, Paris 5, Paris, France.
²⁰ Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Université Paris Descartes, Paris 5, Paris, France. Electronic address: benjamin.terrier@aphp.fr.

PMID: 28780079
DOI: 10.1016/j.autrev.2017.07.020

Abstract

Objective: Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap.

Methods: We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria.

Results: Eighteen patients were included (median age 55.5years, 13 men). AAV and IgG4-RD were diagnosed concomitantly in 13/18 (72%) patients; AAV preceded IgG4-RD in 3/18 (17%) while IgG4-RD preceded AAV in 2/18 (11%). AAV diagnoses included granulomatosis with polyangiitis in 14 (78%), microscopic polyangiitis in 3 (17%), and eosinophilic granulomatosis with polyangiitis in one case. IgG4-RD diagnosis included definite IgG4-RD in 5 (28%) cases, probable IgG4-RD in 5 (28%) and possible IgG4-RD in 8 (44%). IgG4-RD manifestations were chronic periaortitis in 9/18 (50%) patients, orbital mass and tubulointerstitial nephritis in 4 (22%) cases, prevertebral fibrosis in 3 (17%), pachymeningitis and autoimmune pancreatitis in 2 (11%) cases. Patients required median number of 2 (range 0-4) lines of immunosuppressants in combination with glucocorticoids. During the follow-up (median 49,8months, range 17,25-108months), AAV manifestations relapsed in 10/18 (56%) cases and IgG4-RD lesions in 5/18 (28%). When used, mainly for relapses, rituximab showed response in all cases.

Conclusion: AAV and IgG4-RD may overlap. Clinicians should consider that atypical manifestations during AAV could be related to IgG4-RD rather than to refractory granulomatous or vasculitic lesions.

Keywords: ANCA-associated vasculitis; Granulomatosis with polyangiitis (GPA); IgG4-related disease.

Publication types

Multicenter Study
Review

MeSH terms

Antibodies, Antineutrophil Cytoplasmic / immunology*
Female
Humans
Immunoglobulin G / immunology*
Male
Middle Aged
Retrospective Studies
Syndrome

Substances

Antibodies, Antineutrophil Cytoplasmic
Immunoglobulin G