Background Few studies with small patients numbers (n=15) [1, 2] have addressed the disease course of orbital masses in GPA. These studies suggest an aggressive course of orbital masses in spite of intense immunosuppression including biologics .
Objectives To identify and characterize GPA patients with orbital masses in our monocentric cohort of 1142 GPA patients followed from 1990 to the end of 2010 with respect to disease stage, orbital masses and other disease manifestations, course of disease and outcome including chronic damage. Furthermore, to describe efficacy of immunosuppressive treatment, surgical procedures and side effects of therapy.
Methods All patients who fulfilled the American College of Rheumatology criteria or the Chapel Hill Consensus Conference Definition of GPA or had localised GPA , developed orbital masses and had a follow-up >6 months were included. Patients were assessed in 4-6 monthly intervals by a standardized interdisciplinary setting with respect to orbital masses (evaluated by MRI and ophthalmology opinion), all other clinical manifestations, disease stages and activity, ANCA-status, immunosuppressive treatment and effect, surgical procedures, relapse rate, chronic organ damage and side effects of treatment.
Results During the follow-up period, 1142 patients with GPA were seen, 51 (4.5%) of whom with orbital masses. 40 patients fulfilled the inclusion criteria (44% females, median age 44 years, range 20-74, 85% ANCA positive) and had an overall median follow-up period of 101.5 months (23-255 months). 37 patients had a follow-up of >6 months after first manifestation of orbital masses and were included into the analysis. 40.5% (n=15) had a refractory course (disease progression of orbital mass) under standard remission induction with cyclophosphamide and glucocorticoids, 24.3% showed unchanged disease activity, 24.3% achieved a response and 8.1% a complete remission. 44.1% had relapses of orbital masses. 72% of patients developed visual impairment, 19% suffered from blindness of at least one eye. Patients who went blind had a significantly longer time to remission compared to patients who did not (median time to remission 24 vs. 9 months, p=0.0008), and had significantly more often a refractory course (5 out of 6 vs. 7 out of 26, p=0.01) and relapses of orbital masses (5 out of 6 vs. 7 out of 26, p=0.01).
Conclusions Orbital masses are a rare manifestation of GPA and occurred in 4.5% of patients in this cohort. Orbital masses are characterized by a refractory course in spite of highly-potent immunosuppression, high rates of relapse and local damage. Patients with a delayed treatment response, a refractory and/or relapsing course are at higher risk to develop blindness.
Fechner FP, Faquin WC, Pilch BZ. Wegener’s granulomatosis of the orbit: a clinicopathological study of 15 patients. The Laryngoscope 2002; 112: 1945-1950.
Holle JU, Dubrau C, Herlyn K, Heller M, Ambrosch P, Noelle B, et al. Rituximab for refractory granulomatosis with polyangiitis (Wegener’s granulomatosis): comparison of efficacy in granulomatous versus vasculitic manifestations. Ann Rheum Dis 2011 Oct 21 [Epub ahead of print].
Disclosure of Interest None Declared
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