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Pulmonary hypertension associated with interstitial lung disease

Jay H Ryu, MD
Robert P Frantz, MD
Section Editors
Talmadge E King, Jr, MD
Jess Mandel, MD
Deputy Editor
Geraldine Finlay, MD


Pulmonary hypertension (PH) is increasingly recognized as a complication of interstitial lung disease (ILD) [1-3]. It can exist when ILD is mild, but is more common when hypoxemia and severe pulmonary dysfunction exist. The definition, classification, prevalence, pathogenesis, and clinical manifestations of ILD-associated PH are reviewed here. In addition, diagnosis, treatment, and prognosis are discussed. A specific type of ILD-associated PH, PH associated with systemic sclerosis (formerly called scleroderma), and diagnosis of non-ILD-associated PH are presented separately. (See "Pulmonary arterial hypertension in systemic sclerosis (scleroderma): Definition, classification, risk factors, screening, and prognosis" and "Clinical features and diagnosis of pulmonary hypertension in adults" and "Overview of pulmonary hypertension in adults" and "Treatment of pulmonary hypertension in adults".)


Interstitial lung disease (ILD)-associated pulmonary hypertension (PH) is considered present when PH exists in a patient who has ILD, and no alternative cause of the PH can be identified.

PH is defined as a mean pulmonary artery pressure (mPAP) greater than 25 mmHg at rest, as measured by right heart catheterization [4]. PH is considered severe if cardiac index is <2 L/min/m2 or if mPAP is ≥35 mmHg [5].

Previous definitions that are no longer used include an mPAP greater than 30 mmHg with exercise (measured by right heart catheterization) [6] and a systolic pulmonary artery pressure (sPAP) greater than 40 mmHg (measured by Doppler echocardiography) [7]. (See "Clinical features and diagnosis of pulmonary hypertension in adults", section on 'Diagnostic criteria'.)

ILD is inferred when the history, physical examination, imaging, and pathology suggest diffuse parenchymal lung disease. Common manifestations include dyspnea with exertion, cough, auscultatory crackles on chest examination, diffuse bilateral reticular or nodular opacities on chest imaging studies, and interstitial inflammation or fibrosis on lung biopsy. (See "Approach to the adult with interstitial lung disease: Clinical evaluation" and "Approach to the adult with interstitial lung disease: Diagnostic testing".)


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Literature review current through: Jan 2017. | This topic last updated: Tue Jul 12 00:00:00 GMT 2016.
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