Intravenous immunoglobulin therapy for refractory interstitial lung disease associated with polymyositis/dermatomyositis

Lung. 2009 May-Jun;187(3):201-6. doi: 10.1007/s00408-009-9146-6. Epub 2009 Apr 22.


Interstitial lung disease (ILD) associated with polymyositis/dermatomyositis (ILD-PM/DM), including amyopathic dermatomyositis (ADM), is recognized as an important condition because it frequently causes death, despite intensive therapy with high-dose corticosteroid and immunosuppressive agents, such as cyclosporine A and cyclophosphamide. Intravenous immunoglobulin therapy (IVIG) has shown efficacy for myopathy associated with PM/DM, but its usefulness for ILD-PM/DM is unclear. This study was designed to investigate the efficacy of IVIG for refractory ILD-PM/DM. A review was made of medical charts of five patients (2 men and 3 women) who were treated with IVIG for refractory ILD-PM/DM resistant to high-dose corticosteroid and cyclosporine A and/or cyclophosphamide. One patient had acute ILD-PM and four patients had acute ILD-ADM. Of the five patients, one patient with ILD-PM and one patient with ILD-ADM survived. No adverse reactions were seen due to IVIG treatment. There were no critical differences in the clinical parameters and clinical courses between survivors and nonsurvivors. IVIG treatment is safe and could be an effective salvage therapy for refractory ILD-PM/DM in certain cases, suggesting that further controlled trials are worthwhile.

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Aged
  • Bronchoalveolar Lavage Fluid / immunology
  • Cyclosporine / therapeutic use
  • Dermatomyositis / complications
  • Dermatomyositis / diagnosis
  • Dermatomyositis / drug therapy*
  • Dermatomyositis / mortality
  • Drug Resistance*
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use*
  • Immunologic Factors / therapeutic use*
  • Immunosuppressive Agents / therapeutic use
  • Lung Diseases, Interstitial / diagnosis
  • Lung Diseases, Interstitial / drug therapy*
  • Lung Diseases, Interstitial / etiology
  • Lung Diseases, Interstitial / mortality
  • Male
  • Methotrexate / therapeutic use
  • Middle Aged
  • Retrospective Studies
  • Salvage Therapy
  • Tomography, X-Ray Computed
  • Treatment Outcome


  • Adrenal Cortex Hormones
  • Immunoglobulins, Intravenous
  • Immunologic Factors
  • Immunosuppressive Agents
  • Cyclosporine
  • Methotrexate