New advances in the management of pulmonary sarcoidosis

BMJ. 2019 Oct 22:367:l5553. doi: 10.1136/bmj.l5553.

Abstract

Sarcoidosis is a highly variable granulomatous multisystem syndrome. It affects individuals in the prime years of life; both the frequency and severity of sarcoidosis are greater in economically disadvantaged populations. The diagnosis, assessment, and management of pulmonary sarcoidosis have evolved as new technologies and therapies have been adopted. Transbronchial needle aspiration guided by endobronchial ultrasound has replaced mediastinoscopy in many centers. Advanced imaging modalities, such as fluorodeoxyglucose positron emission tomography scanning, and the widespread availability of magnetic resonance imaging have led to more sensitive assessment of organ involvement and disease activity. Although several new insights about the pathogenesis of sarcoidosis exist, no new therapies have been specifically developed for use in the disease. The current or proposed use of immunosuppressive medications for sarcoidosis has been extrapolated from other disease states; various novel pathways are currently under investigation as therapeutic targets. Coupled with the growing recognition of corticosteroid toxicities for managing sarcoidosis, the use of corticosteroid sparing anti-sarcoidosis medications is likely to increase. Besides treatment of granulomatous inflammation, recognition and management of the non-granulomatous complications of pulmonary sarcoidosis are needed for optimal outcomes in patients with advanced disease.

Publication types

  • Review

MeSH terms

  • Biomarkers / blood
  • Bronchoscopy / methods
  • Endoscopic Ultrasound-Guided Fine Needle Aspiration / methods
  • Fluorodeoxyglucose F18 / administration & dosage
  • Glucocorticoids / therapeutic use*
  • Humans
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / mortality
  • Hypertension, Pulmonary / prevention & control*
  • Immunosuppressive Agents / therapeutic use*
  • Incidence
  • Lung / diagnostic imaging
  • Lung / pathology
  • Positron-Emission Tomography / methods
  • Prognosis
  • Pulmonary Fibrosis / etiology
  • Pulmonary Fibrosis / mortality
  • Pulmonary Fibrosis / prevention & control*
  • Sarcoidosis, Pulmonary / complications
  • Sarcoidosis, Pulmonary / diagnosis*
  • Sarcoidosis, Pulmonary / drug therapy
  • Sarcoidosis, Pulmonary / epidemiology
  • Treatment Outcome

Substances

  • Biomarkers
  • Glucocorticoids
  • Immunosuppressive Agents
  • Fluorodeoxyglucose F18