Pulmonary sarcoidosis is the most common form of sarcoidosis, accounting for the initial presentation in over 70% patients and with eventual presence in 90% of patients with sarcoidosis. However, the course of the disease is often unpredictable; its manifestations can be highly variable and its treatment may not be effective in all patients. As such, the optimized treatment of pulmonary sarcoidosis often requires a thoughtful personalized approach with the need to get the patient involved in decisions of management. In many patients with pulmonary sarcoidosis, the disease is self-limited and nonprogressive, thus treatment is not necessary. In other patients, the presence of significant symptoms or functional limitation often associated with worsening radiological changes and pulmonary function tests warrants treatment. Corticosteroids are the first-line treatment for pulmonary sarcoidosis; antimetabolites are second-line agents, with methotrexate being most commonly employed. Antitumor necrosis alpha antibodies, especially infliximab, are emerging as potential third-line agents. A high index of suspicion should be held for pulmonary hypertension and other comorbidities that may complicate the course of patients with advanced sarcoidosis. Lung transplantation may be the only option for patients who have refractory disease despite maximal medical therapy.
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