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Updated: Jul 2, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary hypertension in interstitial lung disease
1Advanced Lung Disease and Transplant Program, Inova Heart and Vascular Institute, Inova Fairfax Hospital, 3300 Gallows Road, Falls Church, VA 22042, USA. steven.nathan@inova.org
Pulmonary hypertension (PH) complicates advanced interstitial lung disease (ILD), worsening patient outcomes. The causes of PH in ILD are complex and require further research to determine if therapies can improve the condition.
Area of Science:
- Pulmonology
- Cardiology
- Internal Medicine
Background:
- Pulmonary hypertension (PH) is a serious complication in advanced interstitial lung disease (ILD).
- PH significantly worsens prognosis for patients with ILD.
- The underlying causes of PH in ILD are multifactorial and vary across different ILD subtypes.
Purpose of the Study:
- To explore the complex relationship between pulmonary hypertension and interstitial lung disease.
- To identify the common ILD conditions associated with PH.
- To investigate the potential for therapeutic modification of PH in ILD.
Main Methods:
- Literature review of studies investigating PH in ILD.
- Analysis of etiological factors contributing to PH in various ILD types.
- Identification of common ILD conditions associated with PH, including idiopathic pulmonary fibrosis, sarcoidosis, connective tissue disease-related ILD, and pulmonary Langerhans cell histiocytosis.
Main Results:
- Pulmonary hypertension frequently complicates advanced interstitial lung disease.
- Common ILD types associated with PH include idiopathic pulmonary fibrosis, sarcoidosis, CTD-ILD, and PLCH.
- The etiology of PH in ILD is multifactorial, with varying contributions from different factors.
Conclusions:
- PH is a significant negative prognostic factor in advanced ILD.
- Understanding the multifactorial etiology of PH in ILD is crucial.
- Further research is needed to determine if therapeutic interventions can alter the course and impact of PH in these conditions.
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