Looking into the future of sarcoidosis: what is next for treatment?

Curr Opin Pulm Med. 2020 Sep;26(5):598-607. doi: 10.1097/MCP.0000000000000709.

Abstract

Purpose of review: Sarcoidosis is a complex granulomatous disease of unknown cause. Corticosteroids and immune suppressants are often given long term in chronic disease, which may result in substantial toxicity. Validated strategies for selecting patients at risk for disease progression, who might benefit from early and targeted treatment, are lacking. Consequently, the unmet need for new treatment options in sarcoidosis is high. In this review, we critically discuss potential therapeutic targets and ongoing clinical trials in sarcoidosis.

Recent findings: Despite the heterogeneous clinical manifestations and the lack of a reliable animal model, our knowledge and understanding of the pathogenesis of sarcoidosis has improved in recent years, which has resulted in the identification of several potential therapeutic strategies. They include the inhibition of cytokines involved in maturation of macrophages, activation of dendritic cells, and maturation and activation of pathogenic T-lymphocytes. The inflammasome and the autophagy are additional areas for future research. Antifibrotic therapy might also be a reasonable choice in selected patients, although the best treatment strategy in progressive fibrotic sarcoidosis remains undetermined.

Summary: In this article, we review novel approaches to sarcoidosis treatment and potential therapeutic targets.

Publication types

  • Review

MeSH terms

  • Adrenocorticotropic Hormone / therapeutic use
  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use
  • Autophagy / immunology
  • Biological Products / therapeutic use
  • Cytokines / immunology
  • Dendritic Cells / immunology
  • Fibrosis
  • Glucocorticoids / therapeutic use*
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Inflammasomes / antagonists & inhibitors
  • Inflammasomes / immunology
  • Macrophages / immunology
  • Molecular Targeted Therapy
  • Piperidines / therapeutic use
  • Protein Kinase Inhibitors / therapeutic use
  • Pyridones / therapeutic use
  • Pyrimidines / therapeutic use
  • Sarcoidosis / drug therapy*
  • Sarcoidosis / immunology*
  • T-Lymphocytes / immunology

Substances

  • Anti-Inflammatory Agents, Non-Steroidal
  • Biological Products
  • Cytokines
  • Glucocorticoids
  • Immunosuppressive Agents
  • Inflammasomes
  • Piperidines
  • Protein Kinase Inhibitors
  • Pyridones
  • Pyrimidines
  • tofacitinib
  • Adrenocorticotropic Hormone
  • pirfenidone