[Interstitial lung diseases and pulmonary hypertension]

Area of Science:

• Pulmonology
• Cardiology
• Pathophysiology

Context:

• Interstitial lung disease (ILD) frequently co-occurs with pulmonary hypertension (PH), affecting 30-40% of patients.
• PH in ILD can manifest even without severe lung dysfunction or hypoxia, carrying a poorer prognosis.
• Pathogenic mechanisms include oxidative stress, cytokines, endothelin system, hypoxic vasoconstriction, vascular destruction, and fibrosis.

Purpose:

• To highlight the high prevalence and diagnostic challenges of pulmonary hypertension in interstitial lung disease.
• To discuss the underlying mechanisms contributing to PH in ILD.
• To review current diagnostic modalities and limited treatment options for PH in ILD.

Summary:

• Pulmonary hypertension (PH) affects a significant portion of patients with interstitial lung disease (ILD), often leading to delayed diagnosis and worse prognosis.
• Pathological processes in ILD, including fibrosis and vascular remodeling, contribute to the development of PH.
• Diagnosis relies on non-specific clinical signs, imaging, and biomarkers, with right heart catheterization being the gold standard; treatment options are limited.

Impact:

• Emphasizes the need for earlier recognition and diagnosis of PH in ILD patients.
• Underscores the limited therapeutic strategies and the necessity for further clinical trials on novel treatments.
• Highlights lung transplantation as a potential option for end-stage disease.