Among patients with
pulmonary sarcoidosis, the rate of spontaneous remission without serious sequelae ranges from 10% to 82%. 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. Overall, the mortality rate for
sarcoidosis is approximately 7% within a 5-year follow-up period. 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. 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%. Patients with
sarcoidosis and precapillary
pulmonary hypertension should be treated with
therapies such as
phosphodiesterase inhibitors and
prostacyclin analogues. Although optimal doses of oral
glucocorticoids for
pulmonary sarcoidosis are unknown, oral
prednisone typically starting at a dose of 20 mg/d to 40 mg/d for 2 to 6 weeks is recommended for patients who are symptomatic (
cough,
dyspnea, and
chest pain) and have parenchymal infiltrates and abnormal pulmonary function test results. Oral
glucocorticoids can be tapered over 6 to 18 months if symptoms, pulmonary function test results, and radiographs improve. Prolonged use of oral
glucocorticoids may be required to control symptoms and stabilize disease. Patients without adequate improvement while receiving a dose of
prednisone of 10 mg/d or greater or those with adverse effects due to
glucocorticoids may be prescribed
immunosuppressive agents, such as
methotrexate,
azathioprine, or an anti-
tumor necrosis factor medication, either alone or with
glucocorticoids combined with appropriate microbial prophylaxis for Pneumocystis jiroveci and
herpes zoster. Effective treatments are not available for advanced fibrocystic
pulmonary disease.
Conclusions and Relevance: