Advances in predicting patient survival in pulmonary sarcoidosis

ABSTRACT

Introduction: Pulmonary disease remains the most common reason for death in sarcoidosis with pulmonary fibrosis and pulmonary hypertension the two most common causes of mortality. Although most sarcoidosis patients with pulmonary fibrosis have a benign outcome, up to 25% of these patients will die from respiratory failure. Unfortunately, over a third of sarcoidosis patients with pre-capillary pulmonary hypertension die within three years.

Areas covered: A dedicated search of articles regarding sarcoidosis and survival up to 1 December 2020 formed the basis of the review. Several factors including the extent of pulmonary fibrosis on high-resolution computer tomography (HRCT), the severity of lung impairment on pulmonary function testing including reduction of diffusion of carbon monoxide in the lung (DLCO), and the presence of pulmonary hypertension are associated with an increased risk of death from pulmonary sarcoidosis. A recent composite score has been developed and verified as a useful tool to identify patients at higher mortality risk.

Expert opinion: Several features from pulmonary function testing and chest imaging may identify those patients with increased risk for death. Information available from either the HRCT or DLCO alone may provide useful screening data.

KEYWORDS:

• Mortality
• sarcoidosis
• pulmonary hypertension
• pulmonary fibrosis
• hrct
• echocardiogram

Article highlights

• Overall, only 5% of sarcoidosis die from their disease, but the mortality is four times higher for sarcoidosis patients with significant pulmonary fibrosis.

• Patients with greater than 20% fibrosis by high-resolution CT scan have an increased risk of death.

• CT scan can also screen for the presence of pulmonary hypertension.

• A reduced DLCO can occur because of fibrosis or pulmonary hypertension.

• A composite score of both pulmonary function and HRCT may identify those at highest risk for death.

This box summarizes key points contained in the article.

Declaration of interest

R Baughman has received grants from Genentech, Mallinckrodt, Bayer, Novartis, Atyr, Gilead, Bellephron, Actelion; is a consultant for Novartis, Actelion, Mallinckrodt, Methial and Bellephron. E Lower has received grants from Genentech, Mallinckrodt, Bayer, Novartis, Atyr and Gilead. 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 disclosure

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

Additional information

Funding

This paper was not funded.