Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small vessel vasculitis associated with asthma and eosinophilia. Optimal therapy for maintenance of remission is yet to be defined. We present a case-series of three patients with EGPA in whom IFN-α, an immunomodulatory cytokine induced remission, which was maintained even after discontinuation of the drug. In all patients (ages 60, 51, and 50 years), remission was associated with normalisation of eosinophil counts and IgE-levels. Moreover, the patients remained in remission for one to four years. Two patients did not need further immunosuppression, one patient required low dose maintenance therapy. Although reversible side effects occur, IFN-α-therapy induces long-term remission of EGPA even after discontinuation of treatment.
Keywords: ANCA, antineutrophil cytoplasmatic antibodies; Autoimmunity; BAL, bronchoalveolar lavage; DMARDs, disease modifying anti-rheumatic drugs; EGPA, eosinophilic granulomatosis with polyangiitis; Eosinophils; IFN-α, interferon-alpha; Interferon; MU, Million Units; PNP, polyneuropathy; Vasculitis.