Treatment for Churg-Strauss syndrome: induction of remission and efficacy of intravenous immunoglobulin therapy.
Churg-Strauss syndrome (CSS) is characterized by the presence of asthma, eosinophilia, and small-vessel vasculitis with granuloma. It is a distinct entity, as determined from all classifications of systemic vasculitis. The poor prognostic factors in CSS are renal insufficiency, cardiomyopathy, severe gastrointestinal (GI) tract, and central nervous systems (CNS) involvement. The initial management of CSS should include a high dose of a corticosteroid: prednisone at 1 mg/kg/day or its equivalent for methylprednisolone with tapering over 6 months. In patients with severe or rapidly progressing CSS, the administration of methylprednisolone pulse at 1 g/body/day for 3 days is recommended. When corticosteroid therapy does not induce remission, or when patients have poor prognostic factors, immunosuppressive cytotoxic therapy is indicated. However, some patients with severe CSS often show resistance to conventional treatment. We think that IVIG therapy is a hopeful candidate for second-line treatment for CSS patients, particularly in the case of neuropathy and/or cardiomyopathy, which are resistant to conventional therapy. However, there is not much evidence supporting the effectiveness of IVIG in CSS, and the mechanisms underlying the action of IVIG remain unclear. Now we are performing clinical trials of IVIG therapy for CSS patients who are resistant to conventional treatment, through a nationwide double-blinded placebo-controlled study in Japan.
Taniguchi
Masami
M
National Hospital Organization, Sagamihara National Hospital, Kanagawa, Japan. m-taniguchi@sagamihara-hosp.gr.jp
Tsurikisawa
Naomi
N
Higashi
Noritaka
N
Saito
Hiroshi
H
Mita
Haruhisa
H
Mori
Akio
A
Sakakibara
Hiroki
H
Akiyama
Kazuo
K
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D015415:Biomarkers / Q000652:urine; D015267:Churg-Strauss Syndrome / Q000188:drug therapy* / Q000276:immunology / Q000652:urine; D003520:Cyclophosphamide / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016898:Interferon-alpha / Q000627:therapeutic use; D017999:Leukotriene E4 / Q000652:urine; D008775:Methylprednisolone / Q000627:therapeutic use; D010951:Plasma Exchange; D011239:Prednisolone / Q000627:therapeutic use; D012074:Remission Induction; D016879:Salvage Therapy; D017211:Treatment Failure; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal; D015415:Biomarkers; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D016898:Interferon-alpha; D017999:Leukotriene E4; D003520:Cyclophosphamide; D011239:Prednisolone; D008775:Methylprednisolone