Pathogenesis, Clinical Manifestations and Treatment of Sarcoidosis Associated Pulmonary Hypertension
Pulmonary Diseases Department, Cerrahpasa Medical Faculty, Istanbul Cerrahpasa University
Dr. Cuneyt Tetikkurt, Pulmonary Diseases Department, Cerrahpasa Medical Faculty, Istanbul Cerrahpasa University.
Keywords: Pulmonary Hypertension; Sarcoidosis; Pulmonary Hypertension Targeted Treatment; Sarcoidosis Associated Pulmonary Hypertension; Immunosuppressive Treatment
Pulmonary hypertension is a well-recognized and a serious complication of sarcoidosis. The exact prevalance is unknown due to the different measurement techniques and diagnostic modalities used with distinct criteria for patient selection but may occur in up to 74% of the sarcoidosis patients. The frequency usually depends on the severity of parenchymal involvement while pulmonary hypertension may also occur in the early stages of the disease. Pulmonary hypertension causes a substantial morbidity and adversely affects the survival causing a major impact on the sarcoidosis patients leading to major functional and prognostic consequences. In advanced disease it appears to be a crucial and a major factor for patient death causing up to ten fold increase in mortality. Therefore, screening, diagnosis and treatment of pulmonary hypertension in sarcoidosis carries the upmost importance. Lack of current treatment guidelines for sarcoidosis associated pulmonary hypertension leaves the clinician to design an individual treatment protocole for each patient consisting of imunosuppressive and pulmonary hypertension targeted agents. Pulmonary hypertension targeted therapy may improve short-term pulmonary hemodynamics without enhancing exercise capacity while steroids or immunosuppressive therapy may improve hemodynamics in selected patients. This review incorporates the risk factors, pathogenesis, clinical manifestations, diagnosis, treatment and prognostic sequela of sarcoidosis associated pulmonary hypertension in regard to current literature. We have also analyzed the clinical charactersitics of the patients, pitfalls in diagnosis and the potential treatment options. Although the prognosis is dreadful, treatment may alleviate patient symptoms, improve quality of life and prolong life expectancy in these patients.
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).