Interní Med. 2009; 11(10): 463-465

Churg-Strauss syndrome

MUDr. Monika Žurková¹, prof. MUDr. Vítězslav Kolek DrSc¹, MUDr. Kateřina Musilová²

¹ Klinika plicních nemocí a tuberkulózy, FN a LF UP Olomouc

² Ústav patologie LF UP Olomouc

Churg-Strauss syndrome is a very rare disease. The incidence is about 2.4/1,000,000 and men are involved more frequently. It usually has

three phases. The first phase is characterized by allergic manifestations, allergic rhinitis, nasal polyps, and bronchial asthma. In the second

phase, eosinophils appear in the blood and tissues; in the respiratory system eosinophilic lung infiltrates and pleural effusion, mostly exudate,

with a high eosinophil count are described, in the gastrointestinal tract eosinophilic gastroenteritis occurs. The third phase is the stage of

systemic vasculitis with manifestations of individual organ damage. The biochemical markers include high erythrocyte sedimentation rate,

high level of IgE antibodies, anaemia, and a positive rheumatoid factor. An eosinophil count above 1.5 x 109 in the peripheral blood is very

common. ANCA antibodies are a typical finding Mostly, they include p-ANCA (perinuclear) autoantibodies, less frequently c-ANCA (cytoplasmic).

Histopathological findings are characterized by necrotising vasculitis of small and medium vessels, granulomas, and infiltration of

tissue by eosinophils. The mainstay of treatment are glucocorticoids, namely prednisone in a dose of 40-60 mg/day, usually in combination

with other immunosuppressive drugs. At present, more than 75% of patients can survive 5 years thanks to combination therapy. In the case

report section, a case of a 38-year-old man with interstitial lung disease is presented. The diagnosis was made by histology from VATS biopsy

and supported by typical clinical, laboratory, and radiological findings. Continuous therapy with steroids was successful.

Keywords: Churg-Strauss syndrome, ANCA antibodies, diagnostics, treatment.

Published: November 1, 2009  Show citation