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. 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.
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.
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.
Authors: A Bersano; M Kraemer; A Burlina; M Mancuso; J Finsterer; S Sacco; C Salvarani; L Caputi; H Chabriat; S Lesnik Oberstein; A Federico; E Tournier Lasserve; D Hunt; M Dichgans; M Arnold; S Debette; H S Markus Journal: J Neurol Date: 2020-04-21 Impact factor: 4.849