Pulmonary hypertension and cardiac involvement in sarcoidosis

Abstract

Sarcoidosis is a multisystem inflammatory disease of unknown origin, characterized by the presence of non-caseating granulomas. Pulmonary hypertension (PH) is a known, but rare complication of sarcoidosis and is associated with increased morbidity and mortality. Cardiac involvement is another leading cause of death in sarcoidosis. This thesis evaluates the diagnosis, treatment and prognosis of both PH and cardiac involvement in sarcoidosis. Clinicians should be aware of the diverse underlying pathophysiological mechanisms of PH in sarcoidosis and subsequent prognosis and treatment options. As the treatment highly depends on the underlying cause, clinical phenotyping can be a first step towards personalised therapeutic decision-making. PH-specific therapies might benefit the individual patient, but there is no strong evidence for effectiveness in the whole population. Cardiac sarcoidosis (CS) is associated with heart failure, arrhythmias and sudden cardiac death, although the rate of adverse events during follow-up varies greatly between CS patients. Advanced cardiac imaging (CMR and FDG PET/CT) and multidisciplinary team evaluation are crucial for adequate diagnosis. The optimal medical therapy of CS is still not determined, but immunosuppressive treatment should be initiated in symptomatic patients and should be considered in patients with cardiac inflammation. Finally, risk stratification has to be performed in each CS patient to determine which patient benefits from cardiac implantable electronic devices. Risk stratification is the first step towards tailor made medicine in CS.