In patients with ANCA-associated vasculitides, refractory orbital mass was associated with proteinase-3 antineutrophil cytoplasmic antibody (PR3-ANCA) positivity, visual loss, and contiguous pachymeningitis, according to a study published in Rheumatology. Additionally, biopsies should be considered at the early stage of the disease to aid in stratifying treatment.

This nationwide, retrospective, multicenter study of French patients (n=59, 33 women, median age 46 years) was designed to describe the presentation, therapeutic management, and treatment outcomes for ANCA-associated vasculitides-related orbital mass. Biological, clinical, radiological, and histological data were collected retrospectively via a standard physician case report form at diagnosis and follow-up. Baseline disease activity was retrospectively assessed using the Birmingham Vasculitis Activity Score, version 3. Therapy response was defined according to European League Against Rheumatism recommendations, which include complete response (remission), partial response, and refractory disease. Complete response was defined as the total disappearance of orbital symptoms attributable to active disease, and partial response was defined as a 50% reduction of orbital symptoms attributable to active disease as well as an absence of new manifestations.

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Of included patients, 95% (n=56) had granulomatosis with polyangiitis. Two patients had eosinophilic granulomatosis with polyangiitis and one had microscopic polyangiitis. Unilateral orbital mass was found in 80% (n=47) of patients, and in most cases appeared to develop from ear, nose, and throat involvement. Among the 32 patients for whom orbital mass biopsies were available, 65% showed lymphoplasmacytic infiltration, 55% showed fibrosis, 48% showed granulomas, and 36% showed vasculitis. Of the 57 patients with therapeutic data available, 56 received first-line glucocorticoids (median dose 60 mg/d), with 54% of these being preceded by intravenous pulses of methylprednisolone. Of all patients, 18% (n=10) received glucocorticoids only, and 82% (n=47) received glucocorticoids in combination with immunosuppressive agents, mainly cyclophosphamide. One patient received immunosuppressive agents only, 1 had orbital radiotherapy, and 2 had concomitant surgical procedures (orbital mass resection and enucleation).

Therapeutic response was observed in 52% of patients receiving cyclophosphamide and 91% of those receiving rituximab. Of all patients, 46% (n=27) received second-line therapy due to relapse or refractory course (59% and 41%, respectively). Clinical ophthalmologic sequelae included visual impairment (28%), definitive blindness (17%), and diplopia (19%). Orbital refractory course was associated with visual loss, PR3-ANCA positivity, and contiguous pachymeningitis.


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Limitations include a retrospective design, the absence of centralized reviewing of data, and heterogeneous management due to a multicenter design.

Investigators conclude, “Current therapeutic approaches provide disappointing results, and our findings suggest that treatments should be probably stratified according to ANCA positivity, initial severity and pathological features. Also, using more aggressive combination therapies should be evaluated in the future.”

Reference

Durel CA, Hot A, Trefond L, et al. Orbital mass in ANCA-associated vasculitides: data on clinical, biological, radiological and histological presentation, therapeutic management, and outcome from 59 patients [published online March 18, 2019]. Rheumatology. doi: 10.1093/rheumatology/kez071