RT Journal Article
SR Electronic
T1 Long-term follow-up in Churg-Strauss syndrome following IFN-α-induced remission
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P2392
VO 40
IS Suppl 56
A1 Seeliger, Benjamin
A1 Foerster, Martin
A1 Moeser, Anne
A1 Happe, Janett
A1 Kehler, Nils
A1 Reißig, Angelika
A1 Bartuschka, Brigitte
A1 Kroegel, Claus
YR 2012
UL http://erj.ersjournals.com/content/40/Suppl_56/P2392.abstract
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.