Orbital inflammatory pseudotumor and ischemic vasculitis in Churg-Strauss syndrome: report of two cases and review of the literature

Abstract

Objective: To clarify the characteristics of ocular manifestations in Churg-Strauss syndrome (allergic granulomatosis and angiitis).

Design: Two interventional case reports and literature review.

Participants: Two patients with Churg-Strauss syndrome with ocular manifestations are described; 15 previously reported cases and the present 2 cases of Churg-Strauss syndrome with ocular manifestations are reviewed.

Intervention: Ocular manifestations were divided into two groups: orbital inflammatory pseudotumor and ischemic vasculitis.

Main outcome measures: The onset, conjunctival involvement, orbital imaging, antineutrophil cytoplasmic antibodies (ANCA), and visual prognosis were evaluated.

Results: The characteristics of the orbital inflammatory pseudotumor type (eight cases) are chronic onset, positive conjunctival involvement, abnormalities in orbital imaging studies, negative ANCA, and good visual prognosis. The ischemic type (nine cases) is characterized by sudden onset, no conjunctival involvement or abnormalities in imaging studies, positive ANCA, and occasional poor visual prognosis.

Conclusions: Orbital inflammatory pseudotumor and ischemic vasculitis may represent two essential characteristics of Churg-Strauss syndrome, granulomatosis and angiitis, respectively. The clinical features of the two types are so distinct that differentiation may be meaningful for diagnosis and treatment of Churg-Strauss syndrome with ocular manifestations.