Factors affecting treatment outcome in patients with idiopathic nonspecific interstitial pneumonia: a nationwide cohort study

Respir Res. 2017 Dec 6;18(1):204. doi: 10.1186/s12931-017-0686-7.

Abstract

Background: The effects of corticosteroid-based therapy in patients with idiopathic nonspecific interstitial pneumonia (iNSIP), and factors affecting treatment outcome, are not fully understood. We aimed to investigate the long-term treatment response and factors affecting the treatment outcome in iNSIP patients from a multi-center study in Korea.

Methods: The Korean interstitial lung disease (ILD) Study Group surveyed ILD patients from 2003 to 2007. Patients were divided into two groups to compare the treatment response: response group (forced vital capacity (FVC) improves ≥10% after 1 year) and non-response group (FVC <10%). Factors affecting treatment response were evaluated by multivariate logistic regression analysis.

Results: A total of 261 patients with iNSIP were enrolled, and 95 patients were followed-up for more than 1 year. Corticosteroid treatment was performed in 86 patients. The treatment group showed a significant improvement in lung function after 1-year: FVC, 10.0%; forced expiratory volume (FEV1), 9.8%; diffusing capacity of the lung for carbon monoxide (DLco), 8.4% (p < 0.001). Sero-negative anti-nuclear antibody (ANA) was significantly related with lung function improvement. Sero-positivity ANA was significantly lower in the response group (p = 0.013), compared to that in the non-response group. A shorter duration of respiratory symptoms at diagnosis was significantly associated with a good response to treatment (p = 0.018).

Conclusion: Treatment with corticosteroids and/or immunosuppressants improved lung function in iNSIP patients, which was more pronounced in sero-negative ANA and shorter symptom duration patients. These findings suggest that early treatment should be considered in iNSIP patients, even in an early disease stage.

Keywords: Non-specific interstitial pneumonia; Pulmonary lung function; Treatment.

Publication types

  • Multicenter Study

MeSH terms

  • Adrenal Cortex Hormones / administration & dosage
  • Aged
  • Cohort Studies
  • Female
  • Follow-Up Studies
  • Humans
  • Idiopathic Interstitial Pneumonias / diagnosis
  • Idiopathic Interstitial Pneumonias / drug therapy*
  • Idiopathic Interstitial Pneumonias / epidemiology*
  • Immunosuppressive Agents / administration & dosage
  • Male
  • Middle Aged
  • Republic of Korea / epidemiology
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Immunosuppressive Agents