Long-term treatment with human immunoglobulin for antisynthetase syndrome-associated interstitial lung disease

Respir Med. Jul-Aug 2019;154:6-11. doi: 10.1016/j.rmed.2019.05.012. Epub 2019 May 21.

Abstract

Background: Interstitial lung disease-associated antisynthetase syndrome (AS-ILD) carries significant morbidity and mortality. Corticosteroids and immunosuppressive drugs are the mainstay of treatment. Human immunoglobulin (IVIg), an immunomodulator without immunosuppressive properties, is effective in myositis but the evidence supporting its use in ILD is scarce.

Objective: To describe clinical outcomes of AS-ILD patients receiving IVIg.

Methods: Retrospective analysis of AS-ILD patients. Linear mixed models using restricted maximum likelihood estimation was used to estimate the change in lung function and corticosteroid dose over time.

Results: Data from 17 patients was analyzed. Median follow-up was 24.6 months. Fourteen patients had refractory disease. The mean percent-predicted forced vital capacity (FVC%) (p = 0.048) and percent-predicted diffusing capacity of the lung for carbon monoxide (DLCO%) (p = 0.0223) increased over time, while the mean prednisone dose (p < 0.001) decreased over time. Seven patients achieved a >10% increase in FVC%, including two who used IVIg as initial treatment. Five patients showed a >10% increase in DLCO% and TLC%. Nine (53%) patients experienced side effects.

Conclusions: IVIg may be a useful complementary therapy in active progressive AS-ILD but is associated with potential side effects. Fssssurther investigation is required to determine the value of IVIg as an initial treatment in AS-ILD.

Keywords: Antisynthetase syndrome and refractory disease; Human intravenous immunoglobulin; Interstitial lung disease; Therapy.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Administration, Intravenous
  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Aged
  • Carbon Monoxide / metabolism
  • Female
  • Follow-Up Studies
  • Humans
  • Immunoglobulins, Intravenous / adverse effects
  • Immunoglobulins, Intravenous / therapeutic use*
  • Immunologic Factors / therapeutic use*
  • Immunosuppressive Agents / therapeutic use
  • Lung / physiopathology
  • Lung Diseases, Interstitial / complications
  • Lung Diseases, Interstitial / mortality
  • Lung Diseases, Interstitial / therapy*
  • Male
  • Middle Aged
  • Myositis / complications
  • Myositis / mortality
  • Myositis / therapy*
  • Prednisone / therapeutic use
  • Pulmonary Diffusing Capacity / drug effects
  • Retrospective Studies
  • Treatment Outcome
  • Vital Capacity / drug effects

Substances

  • Adrenal Cortex Hormones
  • Immunoglobulins, Intravenous
  • Immunologic Factors
  • Immunosuppressive Agents
  • Carbon Monoxide
  • Prednisone

Supplementary concepts

  • Antisynthetase syndrome