[Churg-Strauss syndrome]

Abstract

Background: The Churg-Strauss syndrome, also known as allergic granulomatosis with angiitis, is a rare necrotizing vasculitis with unknown pathogenesis. The necrotizing granulomatous vasculitis involves small and medium-sized arteries, capillaries and veins, leading to the characteristic changes of intra- and extravascular, eosiniphilic granulomas, accompanied by clinically symptoms: bronchial asthma, hypereosinophilia and fever. Ocular manifestations are rarely reported.

Case report: A 53-year-old woman suffered from bronchial asthma, relapsing lung infiltrates and sinusites since 1994. In August 1997 she suddenly disclosed vasculitic skin manifestations on both legs and a mononeuritis of the left peroneus nerve. At the same time visual acuity of the right eye decreased, before she had shown some attacks of amaurosis fugax. The funduscopy showed a central retinal artery occlusion.

Laboratory findings: blood eosinophilia of 20%, elevated IgE value to 396 kU/l (normal value < 120 kU/l), and negative parameters for antineutrophil cytoplasmatic antibodies (p- and c-ANCA).

Conclusion: The clinical and laboratory findings are characteristic signs for the Churg-Strauss syndrome. Without such typical manifestations the histologic examination leads to the diagnosis and helps to differentiate this disease from other necrotizing vasculitides, e.g. panarteriitis nodosa or Wegener's granulomatosis.