title: An exploratory trial of intravenous immunoglobulin therapy for idiopathic pulmonary fibrosis: a preliminary multicenter report.
abstract: OBJECTIVE
  Idiopathic pulmonary fibrosis (IPF) is a fatal disorder without specific treatments. Although the efficacy of intravenous immunoglobulin (IVIG) therapy for autoimmune diseases has been reported, that for IPF remains unknown. This study aims to determine the efficacy and safety of IVIG for IPF.
  
  
  METHODS
  In an exploratory, multicenter, non-randomized and prospective trial, patients with progressive IPF were enrolled. Patients were treated with IVIG for five consecutive days (5-day IVIG) or once monthly for five consecutive months (5-month IVIG). Changes in the vital capacity (VC), diffusion capacity of the lung for carbon monoxide (DLCO), 6-min walk test (6MWT) and high-resolution computed tomography (HRCT) findings were evaluated.
  
  
  RESULTS
  A total of 10 patients with IPF were treated with IVIG: 6 were in 5-day IVIG and 4 were in 5-month IVIG group. In 5-day IVIG group, the treatment effects were temporal, and physiological and HRCT findings deteriorated in three of six patients. In 5-month IVIG group, changes in %VC, %DLCO and walk distance in 6MWT at 6 months were -0.9 ± 5.3%, 6.9 ± 12.6% and 79 ± 58 m (mean ± standard deviation), respectively, and the treatment effects were long lasting. The change in VC 6 months after starting IVIG was smaller than that of 6-12 months after starting IVIG (after cessation of IVIG) (-0.02 ± 0.15 vs -0.33 ± 0.14 L, P = 0.022). Ground glass opacities were diminished in two of four patients. Adverse events were mild and tolerable.
  
  
  CONCLUSIONS
  This preliminary study shows that once-monthly IVIG treatment may be effective and tolerable in patients with IPF.
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan. norieno@hama-med.ac.jp.
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Enomoto
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.|Department of Internal Medicine, Hamamatsu Toyooka Hospital, Hamamatsu, Japan.
authors.forename: Kingo
authors.initials: K
authors.lastname: Chida
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Takafumi
authors.initials: T
authors.lastname: Suda
authors.affiliation: Department of Internal Medicine, Enshu Hospital, Hamamatsu, Japan.
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Kaida
authors.affiliation: Clinical Research Center for Allergy and Rheumatology, National Hospital Organization, Sagamihara National Hospital, Sagamihara, Japan.
authors.forename: Masami
authors.initials: M
authors.lastname: Taniguchi
authors.affiliation: Internal Medicine, Department of Pulmonary Medicine/Infection and Oncology, Nippon Medical School, Tokyo, Japan.
authors.forename: Arata
authors.initials: A
authors.lastname: Azuma
authors.affiliation: Internal Medicine, Department of Pulmonary Medicine/Infection and Oncology, Nippon Medical School, Tokyo, Japan.
authors.forename: Hiroki
authors.initials: H
authors.lastname: Hayashi
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Ogura
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors.forename: Hideya
authors.initials: H
authors.lastname: Kitamura
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
authors.forename: Ou
authors.initials: O
authors.lastname: Yamaguchi
authors.affiliation: Omotesando Yoshida Hospital, Kumamoto, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Ando
authors.affiliation: Kyoto Preventive Medical Center, Kyoto, Japan.
authors.forename: Atsuhiko
authors.initials: A
authors.lastname: Sato
authors.affiliation: Fukujuji Hospital, Japan Anti-Tuberculosis Association, Tokyo, Japan.
authors.forename: Shoji
authors.initials: S
authors.lastname: Kudo
mesh_terms: D000368:Aged; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000503:physiopathology; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014797:Vital Capacity / Q000187:drug effects; D000070857:Walk Test
chemical_list: D016756:Immunoglobulins, Intravenous
keywords: IVIG; idiopathic pulmonary fibrosis; intravenous immunoglobulin therapy