PT  - JOURNAL ARTICLE
AU  - Benjamin Seeliger
AU  - Martin Foerster
AU  - Anne Moeser
AU  - Janett Happe
AU  - Nils Kehler
AU  - Angelika Reißig
AU  - Brigitte Bartuschka
AU  - Claus Kroegel
TI  - Long-term follow-up in Churg-Strauss syndrome following IFN-α-induced remission
DP  - 2012 Sep 01
TA  - European Respiratory Journal
PG  - P2392
VI  - 40
IP  - Suppl 56
4099  - http://erj.ersjournals.com/content/40/Suppl_56/P2392.short
4100  - http://erj.ersjournals.com/content/40/Suppl_56/P2392.full
SO  - Eur Respir J2012 Sep 01; 40
AB  - Churg-Strauss syndrome (CSS) is a small vessel systemic vasculitis associated with asthma, eosinophilia, and involvement of other organs. Interferon (IFN) represents a immunomodulatory cytokine that induces remission in CSS. Herein, we evaluated the long-term effect of IFN-α-induced remission after discontinuation of IFN-α treatment.We conducted a single-center, open-label pilot study using pegylated IFN-α (135 µg/week s.c.) for induction of remission in p-ANCA-negative CSS patients with predominant pulmonary involvement (defined as severe corticosteroid-dependent asthma, chronic rhinosinusitis, peripheral eosinophilia). Written informed consent was obtained from all individuals. A total of 8 patients were treated with IFN-α over more than 2 yrs leading to full remission without immunosuppressive therapy. In three patients (2 females, 1 male; ages 50, 51 and 60, respectively) treatment was discontinued due to side effects (neuropathia, autoimmune hepatitis, anaemia) after 3, 4, and 10 years. At this time-point, IFN-α treatment had induced full normalisation of initially elevated (38%, 25% 23%) eosinophil counts and reduction of total IgE serum-levels (797 to 55, 377 to 233; 1170 to 133 kU/L; min/max values) in all patients. Following discontinuation of IFN-α-treatment, side effects disappeared. In addition, IgE-levels and eosinophil counts did not change and patients remained in remission without immunosuppression during follow-up for up to 4 years.CONCLUSIONS: Although reversible side effects occur, IFN-α is successful in inducing long-lasting remission even after discontinuation of therapy in patients with CSS.