title: A severe form of Churg-Strauss syndrome complicated with acute cardiac failure and rapidly progressive peripheral neuropathy--a possible effect of intravenous immunoglobulin therapy.
abstract: We report a case of Churg-Strauss syndrome (CSS) complicated by severe cardiac failure and peripheral neuropathy. Two courses of methylprednisolone pulse therapy were unable to control the disease activity. Repeated intravenous administration of high-dose human immunoglobulin (IVIg) was added together with intravenous cyclophosphamide pulse therapy (IVCY), and the patient's cardiac function and neurological symptoms were gradually ameliorated without any adverse event. Although glucocorticoid and cyclophosphamide comprise the standard therapy for patients with CSS, a number of patients with severe complications appear to be resistant to such conventional treatment. IVIg is thought to be an effective therapeutic option for such patients.
authors.affiliation: Division of Clinical Nephrology and Rheumatology, Department of Medicine II, Niigata University Graduate School of Medical and Dental Sciences, Japan. joifa602@hotmail.com
authors.forename: Daisuke
authors.initials: D
authors.lastname: Kobayashi
authors.forename: Yoko
authors.initials: Y
authors.lastname: Wada
authors.forename: Takuma
authors.initials: T
authors.lastname: Takata
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Koya
authors.forename: Shuichi
authors.initials: S
authors.lastname: Murakami
authors.forename: Takuya
authors.initials: T
authors.lastname: Ozawa
authors.forename: Yoshifusa
authors.initials: Y
authors.lastname: Aizawa
authors.forename: Takeshi
authors.initials: T
authors.lastname: Kuroda
authors.forename: Masaaki
authors.initials: M
authors.lastname: Nakano
authors.forename: Ichiei
authors.initials: I
authors.lastname: Narita
mesh_terms: D000208:Acute Disease; D015267:Churg-Strauss Syndrome / Q000150:complications* / Q000188:drug therapy / Q000628:therapy*; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D018450:Disease Progression; D004359:Drug Therapy, Combination; D005260:Female; D006333:Heart Failure / Q000209:etiology* / Q000628:therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000209:etiology* / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous; D003520:Cyclophosphamide; D008775:Methylprednisolone