Orbital inflammatory pseudotumor and ischemic vasculitis in Churg-Strauss syndrome

Orbital inflammatory disease: a diagnostic and therapeutic challenge

“…65,66 Systemic 67,68 Multiple organ system involvement is common in this disease and may include the heart, central nervous system, liver, colon, gall bladder, kidney, peripheral nerves, brain, and musculoskeletal system. Laboratory evaluation may also reveal increased circulating IgE and identify the presence of p-ANCA antibodies.…”

Non-infectious orbital vasculitides

“…As CSS and orbital involvement is a relatively rare entity, most information regarding this in the literature is in the form of case reports. [36][37][38][39][40][41] For example, Pradeep et al 38 described two cases of bilateral orbital inflammation in which both patients presented with proptosis and restriction of extraocular movements.…”

“…The key on histology to identifying Churg-Strauss is a necrotizing vasculitis with extravascular infiltration of eosinophils. [38][39][40] The presence of systemic findings can also help to determine CSS as the cause. Laboratory studies include eosinophilia and positive p-ANCA; p-ANCA has been shown to be present in approximately 70% of patients with CSS.…”

“…31 CT and MRI results are less specific and demonstrate enlargement of the retro-orbital space with swelling of the lacrimal glands and extraocular muscles. 38,40 Treatment of orbital vasculitis secondary to CSS primarily consists of corticosteroids. Cases in the literature have shown an excellent response to oral prednisone with almost complete resolution of orbital inflammation.…”

“…Methotrexate and cyclophosphamide have also been used in addition to corticosteroids with similar success to prednisone monotherapy. 38,40 As orbital involvement is fairly infrequent, therapy is almost always in context with treatment of the systemic disease. There have been some successful reports on the use of biologics in refractory or relapsing CSS, although not specifically orbital involvement.…”