title: Intravenous immunoglobulin may be an effective therapy for refractory, active diffuse cutaneous systemic sclerosis.
abstract: OBJECTIVE
  We sought to retrospectively review a single-center experience using intravenous immunoglobulin (IVIG) for the treatment of refractory, active diffuse cutaneous systemic sclerosis (dcSSc).
  
  
  METHODS
  The mean modified Rodnan Skin score (mRSS) at baseline was compared to the mRSS at 6, 12, 18, and 24 months post-IVIG initiation by the paired Student t test. Changes in mRSS at 6 and 12 months were also compared to data from historical controls of 3 large, negative, multicenter, randomized clinical trials of other medications [D-penicillamine (D-pen), recombinant human relaxin (relaxin), and oral bovine type I collagen (collagen)] and to patients treated with mycophenolate mofetil (MMF) alone using the Student t test.
  
  
  RESULTS
  Thirty patients were treated with adjunctive IVIG (2 g/kg/mo) for refractory, active dcSSc. The mean baseline mRSS of our cohort was 29.6 ± 7.2, and this significantly decreased to 24.1 ± 9.6 (n = 29, p = 0.0011) at 6 months, 22.5 ± 10.0 (n = 25, p = 0.0001) at 12 months, 20.6 ± 11.8 (n = 23, p = 0.0001) at 18 months, and 15.3 ± 6.4 (n = 15, p < 0.0001) at 24 months. The mean change in mRSS at 6 months was not significantly different in the IVIG group (-5.3 ± 7.9) compared to the relaxin trial (-4.8 ± 6.99, p = 0.74) or MMF group (-3.4 ± 7.4, p = 0.26); however, at 12 months, the mean change in mRSS was significantly better in the IVIG group (-8 ± 8.3) than in the D-pen (-2.47 ± 8.6, p = 0.005) and collagen (-3.4 ± 7.12, p = 0.005) groups, and was comparable to the group of primary MMF responders (-7.1 ± 9, p = 0.67).
  
  
  CONCLUSIONS
  Our observational study suggests that IVIG may be an effective adjunctive therapy for active dcSSc in patients failing other therapies.
authors.affiliation: From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.C.L. Poelman, BA; L.K. Hummers, MD, ScM, Associate Professor of Medicine; F.M. Wigley, MD, Professor of Medicine; C. Anderson, MS; F. Boin, MD, Assistant Professor of Medicine; A.A. Shah, MD, MHS, Assistant Professor of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine.
authors.forename: Corrie L
authors.initials: CL
authors.lastname: Poelman
authors.affiliation: From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.C.L. Poelman, BA; L.K. Hummers, MD, ScM, Associate Professor of Medicine; F.M. Wigley, MD, Professor of Medicine; C. Anderson, MS; F. Boin, MD, Assistant Professor of Medicine; A.A. Shah, MD, MHS, Assistant Professor of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine.
authors.forename: Laura K
authors.initials: LK
authors.lastname: Hummers
authors.affiliation: From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.C.L. Poelman, BA; L.K. Hummers, MD, ScM, Associate Professor of Medicine; F.M. Wigley, MD, Professor of Medicine; C. Anderson, MS; F. Boin, MD, Assistant Professor of Medicine; A.A. Shah, MD, MHS, Assistant Professor of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine.
authors.forename: Fredrick M
authors.initials: FM
authors.lastname: Wigley
authors.affiliation: From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.C.L. Poelman, BA; L.K. Hummers, MD, ScM, Associate Professor of Medicine; F.M. Wigley, MD, Professor of Medicine; C. Anderson, MS; F. Boin, MD, Assistant Professor of Medicine; A.A. Shah, MD, MHS, Assistant Professor of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine.
authors.forename: Cynthia
authors.initials: C
authors.lastname: Anderson
authors.affiliation: From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.C.L. Poelman, BA; L.K. Hummers, MD, ScM, Associate Professor of Medicine; F.M. Wigley, MD, Professor of Medicine; C. Anderson, MS; F. Boin, MD, Assistant Professor of Medicine; A.A. Shah, MD, MHS, Assistant Professor of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine.
authors.forename: Francesco
authors.initials: F
authors.lastname: Boin
authors.affiliation: From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.C.L. Poelman, BA; L.K. Hummers, MD, ScM, Associate Professor of Medicine; F.M. Wigley, MD, Professor of Medicine; C. Anderson, MS; F. Boin, MD, Assistant Professor of Medicine; A.A. Shah, MD, MHS, Assistant Professor of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine. ashah32@jhmi.edu.
authors.forename: Ami A
authors.initials: AA
authors.lastname: Shah
mesh_terms: D000328:Adult; D018450:Disease Progression; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D045743:Scleroderma, Diffuse / Q000188:drug therapy*; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents
keywords: DIFFUSE SCLERODERMA; INTRAVENOUS IMMUNOGLOBULINS; SYSTEMIC SCLEROSIS