Background Retro-orbital granuloma is a rare and devastating component of granulomatosis with poliangiitis (GPA). Current medical treatment protocols are falling short and outcomes are poor [1-2].
Objectives To investigate the frequency, clinical features and treatment outcomes of retro-orbital granuloma in patients with GPA.
Methods This is a retrospective, multi-center study, which involves GPA cohorts from five different clinics. Data were extracted from patient charts including history, physical examination, radiologic-laboratory-histological findings and treatment protocols. Major clinical outcome measures were changes in the volume of the granuloma on comparative MRI, and visual acuity on repeated ophthalmologic examinations.
Results Among 153 GPA patients, 9 (5 female, 4 male) were diagnosed with a retro-orbital granuloma. Average duration of disease was 8 years. Proptosis and diplopia were the dominant presenting symptoms (77%), followed by orbital pain (55%). Three out of nine patients had isolated retro-orbital granulomas, without other organ involvement of GPA. Three patients had already been diagnosed with GPA, prior to the development of the retro-orbital granuloma.
Seven patients received conventional pulse steroid and pulse cyclophosphamide as the first line remission induction therapy. Six of these patients had progressive disease and one had stable disease.
Seven patients received rituximab (1 as the first-line, 6 as the second-line) as a remission induction therapy. None of these patients had progression following rituximab therapy. In four of these patients mass size on MRI remained stable while in the remaining three it decreased. 3 patients underwent orbital decompression surgery. The indication for the decision for surgery was either progressive loss of vision or intractable pain.
Conclusions Standard first-line chemotherapy (cyclophosphamide and steroids) was ineffective against retro-orbital granuloma associated with GPA. Rituximab could be an alternative in these cases. Surgical intervention may help to decrease the morbidity. Further prospective studies with greater patient numbers are needed to test the clinical efficiency of Rituximab as a first-line remission induction chemotherapy.
Pagnoux, C., et al., Wegener's granulomatosis strictly and persistently localized to one organ is rare: assessment of 16 patients from the French Vasculitis Study Group database. J Rheumatol, 2011. 38(3): p. 475-8.
Holle, J.U., et al., Orbital masses in granulomatosis with polyangiitis are associated with a refractory course and a high burden of local damage. Rheumatology (Oxford), 2013. 52(5): p. 875-82.
Disclosure of Interest None declared