Corticosteroid resistant interstitial pneumonitis in dermatomyositis/polymyositis: prediction and treatment with cyclosporine

J Rheumatol. 1999 Jul;26(7):1527-33.


Objective: To determine the characteristics of corticosteroid resistant interstitial pneumonitis (IP) in dermatomyositis (DM) and polymyositis (PM), and to evaluate the effect of cyclosporine on corticosteroid resistant IP in DM/PM.

Methods: We analyzed retrospectively the incidence, clinical features, and corticosteroid responses of IP in 111 patients with DM (56) or PM (55). All patients with DM/PM were treated with prednisolone, and corticosteroid resistant IP was defined as a progression of IP despite administration of 1 mg/kg/day prednisolone for more than 4 weeks. We also evaluated the effect of cyclosporine on corticosteroid resistant IP in patients with DM/PM.

Results: IP occurred in 24 of 56 DM and 12 of 55 PM patients. We then classified IP in DM/PM according to serum CPK levels at the onset of IP; IP associated with high CPK levels (type I) (19) and IP associated with normal CPK levels (type II) (17). Only 2 of 19 (11%) type I IP were resistant to prednisolone therapy, while 14 of 17 (82%) type II IP were resistant to prednisolone therapy. Thus, patients with type II IP showed poorer prognosis than those with type I IP (one year survival rate: type I 89% vs type II 31%). Cyclosporine was effective in all 5 cases with corticosteroid resistant IP in DM/PM (one year survival rate 80%).

Conclusion: (1) Corticosteroid resistant IP develops mostly in patients with DM/PM without CPK elevation at the onset of IP (type II IP), and (2) cyclosporine is effective for the corticosteroid resistant IP in DM/PM and significantly prolongs survival of patients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / drug therapy*
  • Autoimmune Diseases / physiopathology
  • Cyclosporine / therapeutic use*
  • Dermatomyositis / complications
  • Dermatomyositis / drug therapy*
  • Drug Resistance
  • Female
  • Glucocorticoids / therapeutic use*
  • Humans
  • Lung Diseases, Interstitial / drug therapy*
  • Lung Diseases, Interstitial / etiology
  • Lung Diseases, Interstitial / physiopathology
  • Male
  • Middle Aged
  • Polymyositis / complications
  • Polymyositis / drug therapy*
  • Prednisolone / therapeutic use*
  • Survival Rate


  • Glucocorticoids
  • Cyclosporine
  • Prednisolone