Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose: Orbital mass is a rare ophthalmologic manifestation of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) that remains a therapeutic challenge. This study aimed to describe clinical presentation, therapeutic management and outcome of orbital mass in the setting of AAV.
Methods: We conducted a nationwide retrospective study including 59 patients with AAV fulfilling the American College of Rheumatology criteria or Chapel Hill Consensus Conference definitions and orbital mass based on clinical and radiological features. Clinical, biological, radiological and histological characteristics, treatment use and efficacy, and outcome, were analyzed.
Results: Fifty nine patients (33 women) were included. Fifty-six (95%) patients had granulomatosis with polyangiitis (GPA), two eosinophilic granulomatosis with polyangiitis, and one microscopic polyangiitis, with histological evidence of vasculitis found in 52 (90%) patients. Fifty-one (86%) patients were ANCA-positive, with a specificity against PR3 in 71%. Median age at AAV diagnosis and orbital mass onset were 46 and 49 years, respectively, with 46% patients presenting orbital mass concomitantly to AAV diagnosis, 41% patients during AAV course, and 13% previous to AAV diagnosis. Orbital mass was unilateral in 80% patients. Exophthalmia revealed orbital mass in 54 (93%) patients, while loss of visual acuity was noted in 20 (35%) and diplopia in 21 (36%). Fifty-six (95%) patients had a systemic disease at the time of orbital mass, with a median BVAS at 9. Orbital biopsy was performed in 32 (54%) patients, showing lymphoplasmocytic infiltrate in 65%, extravascular granulomas in 48% and necrotizing vasculitis in 36%. Four patients had histological evidence of IgG4-related disease. All but one patient received corticosteroids as first-line therapy, in combination with immunosuppressive agents in 82%. First-line immunosuppressive agents comprised cyclophosphamide in 24 patients (42%), rituximab in 11 (19%), methotrexate in 5 (9%), mycophenolate mofetil in 4 (7%), azathioprine in 2 (4%), and TNF antagonist in one (2%). Complete response was noted in 15 (27%) cases, partial response in 27 (48%), stabilization in 10 (18%) cases, and worsening in 4 (7%) cases. Among patients with complete response, seven (47%) were MPO-ANCA positive. Twenty seven patients (47%) required a second line of treatment because of relapse in 16 patients (59%) after a median delay of 13 months and refractory course in 11 cases (41%). Six of these 27 patients (22%) received corticosteroids alone as first line. All patients were treated with corticosteroids and one or combined immunosuppressive agent(s). After a median follow-up of 68 months after orbital mass diagnosis, ophthalmologic sequelae included visual impairment in 28% with blindness in 17%, orbital muscular extension on MRI with diplopia in 36%, and naso-sinusal bone defect on MRI in 22%.
Conclusion: Orbital mass represents a therapeutic challenge during AAV, especially during GPA. This rare manifestation is associated with refractory course and high-frequency of sequelae, in particular blindness. Histological findings could help physicians to manage orbital mass.
To cite this abstract in AMA style:Durel CA, Hot A, Trefond L, Aumaître O, Pugnet G, Samson M, Abad S, Belot A, Blanchard-Delaunay C, Cathebras P, Cohen P, Cohen F, Cottin V, Crestani B, Dumonteil S, de Moreuil C, Durupt S, Fuzibet JG, Garzaro-regard M, Girszyn N, Godeau B, Hachulla E, Jamilloux Y, Jego P, Lazaro E, Le Gallou T, Liozon E, Martin T, Papo T, Perlat A, Pillet P, Sacre K, Sève P, Guillevin for the French Vasculitis Study Group L, Terrier B. Clinical Presentation and Outcome of Orbital Mass in Antineutrophil Cytoplasmic Antibody-Associated Vasculitides [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/clinical-presentation-and-outcome-of-orbital-mass-in-antineutrophil-cytoplasmic-antibody-associated-vasculitides/. Accessed July 15, 2020.
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