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J Rheumatol. 2015 Feb;42(2):236-42. doi: 10.3899/jrheum.140833. Epub 2014 Nov 29.

Intravenous immunoglobulin may be an effective therapy for refractory, active diffuse cutaneous systemic sclerosis.

Author information

1
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.
2
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.

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).

CONCLUSION:

Our observational study suggests that IVIG may be an effective adjunctive therapy for active dcSSc in patients failing other therapies.

KEYWORDS:

DIFFUSE SCLERODERMA; INTRAVENOUS IMMUNOGLOBULINS; SYSTEMIC SCLEROSIS

PMID:
25433527
PMCID:
PMC4314512
DOI:
10.3899/jrheum.140833
[Indexed for MEDLINE]
Free PMC Article

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