Sarcoidosis-Associated Pulmonary Hypertension

Area of Science:

• Pulmonology
• Cardiology
• Rheumatology

Background:

• Sarcoidosis is a multisystem granulomatous disease of unknown cause.
• Pulmonary hypertension (PH) is a recognized complication of sarcoidosis, increasing patient morbidity and mortality.
• Sarcoidosis-associated PH (SAPH) is classified under Group 5 (miscellaneous) of PH classification.

Purpose of the Study:

• To review the current understanding of Sarcoidosis-associated Pulmonary Hypertension (SAPH).
• To highlight the unknown epidemiology and incompletely understood pathogenesis of SAPH.
• To discuss the variable clinical presentation and management challenges of SAPH.

Main Methods:

• Literature review of sarcoidosis and pulmonary hypertension.
• Analysis of classification and clinical characteristics of SAPH.
• Evaluation of current and potential therapeutic strategies for SAPH.

Main Results:

• The epidemiology and pathogenesis of SAPH remain largely unknown.
• SAPH presentation is variable and not always correlated with sarcoidosis severity.
• No established treatment algorithm exists for SAPH, though lung transplantation is an option for refractory cases.

Conclusions:

• SAPH is a significant complication of sarcoidosis requiring expert management.
• Pulmonary arterial hypertension-specific vasodilators targeting key pathways show potential benefits in SAPH.
• Further research is needed to clarify SAPH epidemiology, pathogenesis, and optimal treatment strategies.