Pulmonary hypertension secondary to interstitial lung disease

Abstract

Interstitial lung diseases (ILDs) may be complicated by the development of pulmonary hypertension (PH), which is associated with worse functional impairment and a poorer prognosis. This article reviews the current state of knowledge on the prevalence, pathogenesis, diagnosis and prognosis of ILD-related PH. Whether the treatment of ILD-related PH changes clinical outcomes is currently unknown, but the current studies are summarized and the authors’ perspective is offered.

Keywords::

• catheterization
• histiocytosis
• hypertension
• interstitial
• Langerhans cell
• lung diseases
• lymphangioleiomyomatosis
• pulmonary
• pulmonary fibrosis
• research design
• sarcoidosis
• scleroderma
• Swan–Ganz
• systemic

Financial & competing interests disclosure

Steven D Nathan has served as a consultant for, and received research funding from, Intermune, Gilead, United Therapeutics and Actelion. Oksana A Shlobin has served as a speaker for United Therapeutics. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.