Diffuse bilateral orbital inflammation in Churg-Strauss Syndrome

Abstract

A 33-year-old man was diagnosed with asthma and within 5 weeks developed bilateral periocular swelling. Examination revealed bilateral axial proptosis with conjunctival nodules. His blood tests revealed a positive p-antineutrophil cytoplasmic autoantibody with significant eosinophilia. MRI of the orbit showed enlarged extraocular muscles, lacrimal glands, and infiltrative changes in the orbital fat. Biopsy demonstrated granulomatous inflammation with eosinophil predominance. A diagnosis of diffuse bilateral inflammation in Churg-Strauss syndrome was made and the patient responded dramatically to prednisolone with resolution of systemic and orbital findings. The second case was a 72-year-old woman with a prolonged prodromal phase of asthma, paranasal sinus disease, and bilateral orbital involvement by a process consistent with reactive lymphoid hyperplasia on initial biopsy. Three years later she developed rapidly worsening orbital disease, marked peripheral eosinophilia, and orbital biopsy showed evidence of granulomatous inflammation with marked eosinophil infiltration and vasculitic changes, and a weakly positive antineutrophil cytoplasmic autoantibody. Hence, diffuse bilateral orbital inflammation occurring in the setting of asthma and peripheral eosinophilia should raise the possibility of Churg-Strauss syndrome and warrants biopsy as early institution of therapy can reduce both systemic and ophthalmic complications.