Patients with sarcoidosis and precapillary pulmonary hypertension should be treated with therapies such as phosphodiesterase inhibitors and prostacyclin analogues. Up to 70% of patients with advanced pulmonary sarcoidosis develop precapillary pulmonary hypertension, which is associated with a 5-year mortality rate of approximately 40%. Worldwide, more than 60% of deaths from sarcoidosis are due to pulmonary involvement however, more than 70% of deaths from sarcoidosis are due to cardiac involvement in Japan. Overall, the mortality rate for sarcoidosis is approximately 7% within a 5-year follow-up period. However, lung disease progression occurs in more than 10% of patients and can result in fibrocystic architectural distortion of the lung, which is associated with a mortality rate of 12% to 18% within 5 years. Observation Among patients with pulmonary sarcoidosis, the rate of spontaneous remission without serious sequelae ranges from 10% to 82%. Approximately 10% to 30% of patients with sarcoidosis develop progressive pulmonary disease. Importance Sarcoidosis is an inflammatory granulomatous disease of unknown cause that affects an estimated 2 to 160 people per 100 000 worldwide and can involve virtually any organ.

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