The current state-of-the-art in pharmacotherapy for pulmonary sarcoidosis.

INTRODUCTION: Sarcoidosis is a chronic granulomatous of unknown etiology that mostly affects lungs with an heterogenous clinical presentation and prognosis. Therefore, therapeutic management of the disease is challenging. The goals of treatment are to prevent or to minimize organ damage, to relieve symptoms, and to improve the patient's quality of life.

AREAS COVERED: The present review covers current pharmacotherapy options for pulmonary sarcoidosis. Corticosteroids are still the first-line treatment option, however, for those patients with prolonged expectation of treatment, undesirable side effects and refractory disease, immunosuppressive drugs are preferred options. Biological drugs are promising third line therapies. Recent evidence shows that antifibrotic agents, such as nintedanib, have a role in fibrotic lung disease, as well as efzofitimob, which has shown promising results in controlling inflammatory lung disease.

EXPERT OPINION: Sarcoidosis treatment is evolving as new molecules are available. The number of studies of therapies for pulmonary sarcoidosis has increased in recent years, however, the information available is still limited and there is no consensus on how to monitor the activity of the disease.