Incidence of pulmonary hypertension and its clinical relevance in patients with interstitial pneumonias: comparison between idiopathic and collagen vascular disease associated interstitial pneumonias

Intern Med. 2007;46(12):831-7. doi: 10.2169/internalmedicine.46.6342. Epub 2007 Jun 15.


Object: This study was undertaken to investigate the frequency of pulmonary hypertension (PH), and clinical parameters associated with PH in patients with idiopathic interstitial pneumonias (IIPs) and collagen vascular disease-associated interstitial pneumonias (CVD-IPs).

Patients and methods: Retrospective analyses were performed in 163 consecutively enrolled patients (78 IIPs and 85 CVD-IPs) who were being evaluated for PH by Doppler echocardiography. PH was defined as an estimated systolic pulmonary artery pressure (sPAP) of > or = 40 mmHg. The frequency of PH was evaluated and clinical parameters were compared between patients with PH and those without PH.

Results: Among patients successfully evaluated for sPAP by echocardiography, 20 of 70 (28%) patients with IIPs and 17 of 80 (21%) patients with CVD-IPs had PH. Among patients with IIP and CVD-IP, those with PH were more frequently treated with supplemental oxygen. Patients with IIP who had PH tended to be older and had decreased % diffusion capacity of carbon monoxide (%DLCO). Additionally, in patients with IIP, sPAP was negatively correlated with %DLCO. However, this association was not found in patients with CVD-IP. Patients with CVD-IP who had PH were predominantly male.

Conclusion: PH was frequently observed in patients with both IIPs (28%) and CVD-IPs (21%) in the chronic phase of the disease. PH should be evaluated in patients with IPs who need supplemental oxygen, IIP patients with decreased diffusing capacity, and in patients with CVD-IP.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Aged
  • Causality
  • Collagen Diseases / diagnosis
  • Collagen Diseases / epidemiology*
  • Comorbidity
  • Echocardiography, Doppler
  • Female
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / epidemiology*
  • Incidence
  • Japan / epidemiology
  • Lung Diseases, Interstitial / diagnosis
  • Lung Diseases, Interstitial / epidemiology*
  • Male
  • Middle Aged
  • Pulmonary Artery / diagnostic imaging
  • Respiratory Function Tests
  • Retrospective Studies
  • Risk Factors