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Interferon-alpha for maintenance of remission in Churg-Strauss Syndrome: a long-term observational study

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2010 Vol.28, N°1 ,Suppl.57
PI 0024, PF 0030
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PMID: 20412698 [PubMed]

Received: 30/06/2009
Accepted : 12/10/2009
In Press: 22/04/2010
Published: 22/04/2010


BACKGROUND: Interferon-α has been successfully used for induction of remission in patients with Churg-Strauss syndrome, but data on its ability to prevent relapses and its safety during long-term use are lacking. OBJECTIVES:
To examine the safety and efficacy of interferon-α for mainten-ance of remission in Churg-Strauss syndrome. PATIENTS AND
In a prospective open-label long-term observational study, 13 patients with CSS in stable remission received interferon-α (3 x 3 Mio. I.U/week s.c.) for maintenance of remission. Primary end point was the incidence of relapses. Secondary end points were the doses of concomitant prednisolone and the frequency adverse events.
After a median follow up of 64 month three patients were still on treatment with interferon-α all with a dose of 9 million units/week. In nine patients, interferon-α was discontinued for lack of efficacy (n=5), due to adverse events (n=2), or both (n=2) after median of 63 months (15–153) of therapy. A total of 3 major and 18 minor relapses occurred in 10 of the 13 patients with a median time to first relapse of 17 months (range 5–46). Sera of relapsing patients did not contain antibodies against interferon-α. In 6 relapsing patients treatment was switched to cyclophosphamide (n=4) or methotrexate (n=2). Four episodes of worsened asthmatic symptoms associated with a mild rise of blood eosinophils occurred in 3 patients and resolved following a transient increase of the oral prednisolone dosage. After 49 months one patient died probably due to a relapse. IFN-α was ceased prematurely, because of autoimmune-thyreoiditis in one, depression in another and cerebral leukoencephalopathy in two patients. Overall, 18 infectious episodes with need of antimicrobial treatment were observed.CONCLUSIONS. Recombinant interferon-α appears to be partially effective in the prevention of major relapses in patients with Churg-Strauss syndrome. Due to numerous side effects and infections during long-term administration its use should be restricted to patients with contraindications against conventional immunosuppressive therapies.

Rheumatology Article