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Updated: Oct 3, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Screening Strategies for Pulmonary Hypertension in Patients With Interstitial Lung Disease: A Multidisciplinary
Franck F Rahaghi1, Nicholas A Kolaitis2, Ayodeji Adegunsoye3
1Advanced Lung Disease Clinic, Cleveland Clinic Florida, Weston, FL.
Expert consensus identified key triggers for screening pulmonary hypertension (PH) in interstitial lung disease (ILD) patients. Early PH evaluation is crucial due to new therapies and worse outcomes in ILD.
Area of Science:
- Pulmonology
- Cardiology
- Critical Care Medicine
Background:
- Pulmonary hypertension (PH) is a frequent complication of interstitial lung disease (ILD), significantly increasing mortality.
- Standardized screening for PH in ILD patients is lacking, despite its association with poor outcomes.
- Effective therapies for PH in ILD are now available, underscoring the need for routine screening.
Purpose of the Study:
- To establish expert consensus on screening strategies for PH in patients with ILD.
- To provide guidance for identifying ILD patients who may have PH.
- To facilitate timely diagnosis and management of PH in ILD.
Main Methods:
- A modified Delphi consensus process involving 16 pulmonologists expert in PH and ILD.
- Three rounds of surveys were used to gather and refine expert opinions on PH screening.
- Panelists rated statements on PH screening strategies using a Likert scale.
Main Results:
- Consensus was reached on multiple PH suspicion triggers: symptoms, clinical signs, imaging (CT), pulse oximetry, natriuretic peptides (BNP/NT-proBNP), and pulmonary function/6-min walk test changes.
- Echocardiography and natriuretic peptides (BNP/NT-proBNP) were identified as key screening tools.
- Right heart catheterization remains the gold standard for PH confirmation.
Conclusions:
- Early PH evaluation in ILD patients is warranted, especially with available therapies.
- Existing ILD evaluation protocols can be adapted for PH risk assessment.
- This consensus provides a framework to aid physicians in identifying and evaluating ILD patients for PH, guiding timely right heart catheterization.
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