title: [Churg-Strauss syndrome: diagnosis and treatment].
abstract: Few randomized clinical trials (RCTs) have been performed in patients with Churg-Strauss syndrome. Therefore, it is recommended to treat patients with this syndrome in accordance with the current practice guidelines for therapy for patients with ANCA-associated vasculitis. Standard therapy is prednisolone in combination with cyclophosphamide. Cyclophosphamide is stopped 3-6 months after diagnosis and subsequently azathioprine is started. Recently, it was demonstrated in two RCTs that B cell depletion by rituximab can be used as therapy for moderate to severe ANCA-associated vasculitis. Remission rates and adverse events were comparable with those of treatment with cyclophosphamide. It is pivotal to start therapy in patients with Churg-Strauss syndrome as soon as possible after the diagnosis is made.
authors.affiliation: Academisch Ziekenhuis Maastricht, afd. Interne Geneeskunde/Klinische en Experimentele Immunologie, Maastricht, Netherlands. secretariaat-immuno@immuno.unimaas.nl
authors.forename: J W
authors.initials: JW
authors.lastname: Cohen Tervaert
authors.forename: C A
authors.initials: CA
authors.lastname: Stegeman
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D015267:Churg-Strauss Syndrome / Q000175:diagnosis* / Q000188:drug therapy*; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D019317:Evidence-Based Medicine; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017410:Practice Guidelines as Topic; D011239:Prednisolone / Q000627:therapeutic use; D012074:Remission Induction; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone; D001379:Azathioprine