Strategies for identifying pulmonary sarcoidosis patients at risk for severe or chronic disease

ABSTRACT

Introduction: Most of the morbidity and mortality resulting from pulmonary sarcoidosis relates to complications of fibrotic disease. Because the fibrosis related to pulmonary sarcoidosis is often of minimal clinical importance, pharmacotherapy is not mandated. However, a small fraction of pulmonary sarcoidosis patients develop significant lung fibrosis, and they could potentially benefit from anti-sarcoidosis therapy. A reliable algorithm to determine the likelihood of a pulmonary sarcoidosis patient developing fibrosis would minimize the toxicity of therapy and potentially prevent serious complications of the disease.

Areas covered: The mechanisms of fibrosis in pulmonary sarcoidosis are discussed. Granulomatous inflammation is the major cause of fibrosis in pulmonary sarcoidosis. Known risk factors for the development of persistent and fibrotic sarcoidosis, including genetic risk factors are explored.

Expert opinion/commentary: Currently, methods to determine the propensity of a pulmonary sarcoidosis to develop significant fibrosis are unreliable. This is an important unmet medical need.

KEYWORDS:

• Sarcoidosis
• fibrosis
• inflammation
• granuloma
• end stage lung disease
• genetics

Declaration of interest

MA. Judson is a consultant for Biogen and has received institutional grant support from Novartis and Mallinckrodt. The author has 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.

Additional information

Funding

This paper was not funded.