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

ABSTRACT

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.

KEYWORDS:

• Sarcoidosis
• corticosteroids
• immunosuppressants
• efzofitimob
• nintedanib

Article highlights

• Sarcoidosis is a systemic chronic granulomatous disease of unknown etiology which mostly affects lungs.

• Treatment aims to avoid permanent or progressive organ damage, reduce granulomatous inflammation, and stop or slow down pulmonary fibrosis progression.

• Identifying active disease is essential since established organ damage may result in reduced treatment options.

• Corticosteroids are still the first-line treatment option.

• Nintedanib and efzofitimob has recently shown positive results on fibrotic and inflammatory disease respectively.

Declaration of interests

The authors have no 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. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties

Reviewer disclosures

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

Additional information

Funding

This paper was supported by FEDER Funds [PI19/01152 and PI23/00294], SEPAR, SOCAP, FUCAP and the August Pi i Sunyer Biomedical Research Institute [IDIBAPS].