History: Two patients, 60 (pat. 1; female) and 30 years of age (pat. 2; male), respectively, suffering from a histologically confirmed Churg-Strauss-syndrome and receiving immunosuppressive therapy were treated with Interferon-alpha.
Investigations: Clinical complaints, disease activity, blood eosinophil counts, and lung function were monitored. In patient 1 the differential cell counts and immunocytology of bronchoalveolar lavage cells were assessed using flow cytometry.
Treatment and course: Both patients were treated with interferon-alpha in dosages of 3 million units of IFN-alpha 2b or an equivalent dosage of interferon-acon thrice weekly subcutaneously. The patients were observed for a period of up to 24 months. Interferon-alpha induced remission of disease and allowed discontinuation of oral glucocorticoid therapy in both patients. Treatment also improved the peripheral polyneuropathia in patient 1 as well as the hemorrhagic cystitis and reduction of the Cushing syndrome (weight reduction of 19 kg) in patient 2. In addition, blood eosinophil counts normalised. After 12 months of treatment, the number of bronchoalveolar eosinophils decreased from 61,5% (5.7 x 106 cells/ml) to 7% (1.1 x 106 cells/ml). In addition, the proportion of CD4+ T-lymphocytes and B-cells increased, while CD8+ T-cells and NK cells decreased (pat. 1).
Conclusion: Interferon-alpha may represent an effective alternative to the current treatment of Churg-Strauss syndrome consisting of corticosteroids and immunosuppressives.