Cécile-Audrey Durel1, Arnaud Hot1, Ludovic Trefond2, Olivier Aumaitre2, Gregory Pugnet3, Maxime Samson4, Sébastien Abad5, Alexandre Belot6, Claire Blanchard-Delaunay7, Pascal Cohen8, Fleur Cohen-Aubard9, Vincent Cottin10, Bruno Crestani11, Claire De Moreuil12, Stéphane Durupt13, Margaux Garzaro14, Nicolas Girszyn15, Bertrand Godeau16, Eric Hachulla17, Yvan Jamilloux18, Patrick Jego19, Martin Killian20, Estibaliz Lazaro21, Thomas Le Gallou19, Eric Liozon22, Thierry Martin23, Thomas Papo24, Antoinette Perlat19, Pascal Pillet25, Loïc Guillevin9,26,27, Benjamin Terrier9,26,27. 1. Department of Internal Medicine, Hôpital Edouard Herriot, Lyon. 2. Department of Internal Medicine, Hôpital Gabriel Montpied, Clermont-Ferrand. 3. Department of Internal Medicine, Hôpital Purpan, Toulouse. 4. Department of Internal Medicine and Clinical Immunology, CHU Dijon Bourgogne, Dijon. 5. AP-HP, Hôpital Avicennes, Department of Internal Medicine, Université Paris 13, Sorbonne, Paris Cité, Faculté de Médecine Bobigny, Paris. 6. Department of Pediatric Nephrology, Rheumatology, Dermatology, National Referral Center for Inflammatory Rheumatism and Autoimmune Diseases, Hôpital Femme Mère Enfant, Bron. 7. Department of Internal Medicine, Centre Hospitalier de Niort, Niort. 8. Department of Internal Medicine, Hôpital Cochin, Paris. 9. Department of Internal Medicine, National Referral Center for Systemic and Autoimmune Diseases, Hôpital de la Pitié-Salpêtrière, Paris. 10. Department of Respiratory Medicine, National Reference Center for Rare Pulmonary Diseases, Lyon. 11. Department of Pneumology, Hôpital Bichat, Paris. 12. Department of Internal Medicine, Hôpital de la Cavale Blanche, Brest. 13. Department of Internal Medicine, Centre Hospitalier Lyon Sud, Lyon. 14. Department of Internal Medicine, CHU l'Archet, Nice. 15. Department of Internal Medicine, Hôpital Charles Nicolle, Rouen. 16. Department of Internal Medicine, Referral Center for Adult Autoimmune Cytopenias, Hôpital Henri Mondor, Créteil. 17. Department of Internal Medicine, Hôpital Claude Huriez, National Referral Center for Systemic Autoimmune Diseases North and North-West of France, Université de Lille, Lille. 18. Department of Internal Medicine, Hôpital de la Croix-Rousse, Lyon. 19. Department of Internal Medicine, Hôpital Sud, Rennes. 20. Department of Internal Medicine, Hôpital Nord, Saint-Etienne. 21. Department of Internal Medicine, Hôpital Haut-Lévêque, CHU de Bordeaux, Pessac. 22. Department of Internal Medicine, Hôpital de Limoges, Limoges. 23. Department of Clinical Immunology, HIV, and Internal Medicine, National Referral Center for Systemic and Autoimmune Diseases RESO, Nouvel Hôpital Civil, Strasbourg. 24. Department of Internal Medicine, Hôpital Bichat, Paris. 25. Department of Medical Pediatrics, CHU de Bordeaux - GH Pellegrin, Bordeaux. 26. Hôpital Cochin, National Referral Center for Systemic and Autoimmune Diseases, Paris. 27. Faculté de Médecine Paris Descartes, Université Paris Descartes, Paris, France.
Abstract
OBJECTIVE: Orbital mass is a rare and sight-threatening manifestation of ANCA-associated vasculitides, which remains a therapeutic challenge. We aimed to describe the presentation, therapeutic management and outcome of ANCA-associated vasculitides-related orbital mass. METHODS: We conducted a French nationwide retrospective study of patients with orbital mass in the setting of ANCA-associated vasculitides according to ACR criteria and/or Chapel Hill Consensus Conference definitions. RESULTS: Fifty-nine patients [33 women, median age 46 (range 7-90) years] were included. Fifty-six (95%) patients had granulomatosis with polyangiitis, two eosinophilic granulomatosis with polyangiitis and one microscopic polyangiitis. Orbital mass was unilateral in 47 (80%) cases, and seemed to develop from ENT involvement in most cases. Orbital mass biopsy was available in 32 (54%) patients, showing lymphoplasmacytic infiltration in 65%, fibrosis in 55%, granulomas in 48% and vasculitis in 36%. All patients but one received glucocorticoids as first-line therapy associated with immunosuppressive agents in 82%, mainly cyclophosphamide. Response to therapy was noted in 52% of patients treated with cyclophosphamide compared with 91% of those treated with rituximab. Twenty-seven (46%) patients required a second-line therapy because of relapse (59%) or refractory course (41%). Sequelae included visual impairment in 28%, with definitive blindness in 17%. Refractory course was associated with PR3-ANCA positivity, visual loss and contiguous pachymeningitis. CONCLUSION: Orbital mass is associated with refractory course and high frequency of sequelae, especially blindness. Refractory course is associated with PR3-ANCA positivity, visual loss and contiguous pachymeningitis.
OBJECTIVE: Orbital mass is a rare and sight-threatening manifestation of ANCA-associated vasculitides, which remains a therapeutic challenge. We aimed to describe the presentation, therapeutic management and outcome of ANCA-associated vasculitides-related orbital mass. METHODS: We conducted a French nationwide retrospective study of patients with orbital mass in the setting of ANCA-associated vasculitides according to ACR criteria and/or Chapel Hill Consensus Conference definitions. RESULTS: Fifty-nine patients [33 women, median age 46 (range 7-90) years] were included. Fifty-six (95%) patients had granulomatosis with polyangiitis, two eosinophilic granulomatosis with polyangiitis and one microscopic polyangiitis. Orbital mass was unilateral in 47 (80%) cases, and seemed to develop from ENT involvement in most cases. Orbital mass biopsy was available in 32 (54%) patients, showing lymphoplasmacytic infiltration in 65%, fibrosis in 55%, granulomas in 48% and vasculitis in 36%. All patients but one received glucocorticoids as first-line therapy associated with immunosuppressive agents in 82%, mainly cyclophosphamide. Response to therapy was noted in 52% of patients treated with cyclophosphamide compared with 91% of those treated with rituximab. Twenty-seven (46%) patients required a second-line therapy because of relapse (59%) or refractory course (41%). Sequelae included visual impairment in 28%, with definitive blindness in 17%. Refractory course was associated with PR3-ANCA positivity, visual loss and contiguous pachymeningitis. CONCLUSION: Orbital mass is associated with refractory course and high frequency of sequelae, especially blindness. Refractory course is associated with PR3-ANCA positivity, visual loss and contiguous pachymeningitis.