Proptosis as a Presenting Symptom of Systemic Wegener's Granulomatosis: A Diagnostic Challenge!!

Abstract

Wegener's Granulomatosis (WG) is a multisystem autoimmune disease of unknown etiology. Isolated orbital presentation of WG is rare and becomes a diagnostic challenge. We report a 37 yr old male with recurrent episodes of right sided proptosis for 2 years and remittance with systemic steroids. He presented to us with a relapse of proptosis over one month associated with sudden loss of vision. Investigations including biopsies were inconclusive done elsewhere. Clinical examination revealed                   non-axial proptosis with necrotizing scleritis and cilioretinal artery occlusion in the right eye. His systemic evaluation was normal. Imaging and orbital biopsy revealed chronic inflammation. ESR, CRP, cANCA and IgG4 were deranged and pointed towards WG. He was initiated on high dose steroids and later augmented with immune modulators with which he responded favorably. High index of suspicion is needed to diagnose WG since the ocular features and markers are non specific. Remittance and relapses in WG need a multi departmental approach of management and any delay in the diagnosis can lead to permanent visual disability.