Title :

Orbital mass in ANCA-associated vasculitides: data on clinical, biological, radiological and histological presentation, therapeutic management, and outcome from 59 patients.

Author(s) :

Durel, Cecile-Audrey [Auteur]
Hot, Arnaud [Auteur]
Trefond, Ludovic [Auteur]
Aumaitre, Olivier [Auteur]
Pugnet, Gregory [Auteur]
Samson, Maxime [Auteur]
Abad, Sebastien [Auteur]
Belot, Alexandre [Auteur]
Blanchard-Delaunay, Claire [Auteur]
Cohen, Pascal [Auteur]
Cohen-Aubart, Fleur [Auteur]
Cottin, Vincent [Auteur]
Crestani, Bruno [Auteur]
Moreuil, Claire De [Auteur]
Durupt, Stephane [Auteur]
Garzaro, Margaux [Auteur]
Girszyn, Nicolas [Auteur]
Godeau, Bertrand [Auteur]
Hachulla, Eric [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Jamilloux, Yvan [Auteur]
Jego, Patrick [Auteur]
Killian, Martin [Auteur]
Lazaro, Estibaliz [Auteur]
Le Gallou, Thomas [Auteur]
Liozon, Eric [Auteur]
Martin, Thierry [Auteur]
Papo, Thomas [Auteur]
Perlat, Antoinette [Auteur]
Pillet, Pascal [Auteur]
Guillevin, Loic [Auteur]
Terrier, Benjamin [Auteur]

Journal title :

Rheumatology (Oxford, England)

Abbreviated title :

Rheumatology (Oxford)

Publication date :

2019-03-18

ISSN :

1462-0332

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

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 ...
Show more >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. CONCLUSIONS: 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.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

CHU Lille
Inserm
Université de Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2019-10-22T07:44:28Z