Therapeutic strategies for pulmonary sarcoidosis

ABSTRACT

Introduction: The treatment of pulmonary sarcoidosis is not standardized. Treatment involves a multi-step decision process beginning with whether treatment is warranted, determining initial therapy, then assessing when therapy requires modifications or can be discontinued.

Areas covered: This manuscript will address the following issues concerning the treatment of pulmonary sarcoidosis: Treatment indications, initial anti-granulomatous therapy, therapy for chronic and fibrotic disease, glucocorticoid therapy, alternative therapy to glucocorticoids, non-granulomatous therapies, and managing complications of disease. Information was obtained through a literature search of PubMed and Web of Science databases.

Expert opinion: Although glucocorticoids are highly effective for pulmonary sarcoidosis, their potential to cause significant side effects often mandates consideration of alternative agents. As the most common indication for the treatment of pulmonary sarcoidosis is quality of life impairment, traditional objective tests of lung function and radiographic imagining often have a minor role in therapeutic decision-making. The development of pulmonary fibrosis from sarcoidosis often causes major morbidity and mortality and should be a major focus of concern.

KEYWORDS:

• Sarcoidosis
• pulmonary
• treatment
• quality of life
• pulmonary fibrosis
• glucocorticoids

Article highlights

• Treatment of pulmonary sarcoidosis is not mandatory, as the disease may have no long-term sequelae and potential toxicity of therapy is common and often significant.

• The two major indications for the treatment of sarcoidosis are the development/risk of a dangerous clinical situation and a significant impairment in quality of life.

• As impairment of quality of life is a major concern of pulmonary sarcoidosis patients and a major indication for treatment, effective therapy for pulmonary sarcoidosis incorporates treatment beyond the management of granulomatous inflammation including improving exercise performance and managing depression and anxiety.

• The initial drug of choice for the treatment of acute flares of pulmonary sarcoidosis, usually at a dose of 20 mg/day of prednisone equivalent.

• Because glucocorticoid side effects are common and significant, additional therapies for pulmonary sarcoidosis that are glucocorticoid-sparing or glucocorticoid-replacing should be considered if the need for treatment is prolonged.

• Most of the serious complications and death from pulmonary sarcoidosis occur in those with the fibrotic disease.

• Fibrosis in pulmonary sarcoidosis is a by-product of the granulomatous inflammation.

• Common potentially life-threatening complications of fibrotic pulmonary complications include sarcoidosis-associated pulmonary hypertension, bronchiectasis, and pulmonary mycetoma. These conditions will not respond to anti-granulomatous therapy and require alternative approaches.

Declaration of interest

MA Judson has received institution grant support from Mallinckrodt, aTyr, and Novartis. EW James has received institution grant support from Mallinckrodt. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.