Clinical phenotyping: role in treatment decisions in sarcoidosis

Eur Respir Rev. 2020 Mar 20;29(155):190145. doi: 10.1183/16000617.0145-2019. Print 2020 Mar 31.

Abstract

A variety of phenotypic categorisations have been developed for sarcoidosis. Phenotyping has been used for genetics studies and to guide treatment selection. The authors participated in a Delphi expert consensus panel to develop a proposed phenotype categorisation and treatment recommendations for pulmonary sarcoidosis patients. Panellists reached consensus that asymptomatic patients with normal pulmonary function and adenopathy alone or normal chest imaging do not require therapy, while symptomatic patients with impaired pulmonary function or infiltrates should be treated. The panel did not reach consensus on asymptomatic patients with abnormal chest imaging or reduced pulmonary function, or symptomatic patients with normal chest imaging and pulmonary function. The proposed phenotype categories and associated treatment recommendations are asymptomatic (no therapy), acute (disease duration <1-2 years, apparently self-limited, corticosteroids), chronic (antimetabolites and other second-line therapies) and advanced (biologics). Some clinical settings, such as dyspnoea/hypoxaemia at rest, severely impaired or rapidly decreasing pulmonary function tests, and severe cardiac, neurologic, ocular or renal involvement warrant immediate therapy.

Publication types

  • Review

MeSH terms

  • Acute Disease
  • Adrenal Cortex Hormones / therapeutic use
  • Asymptomatic Diseases
  • Biological Products / therapeutic use
  • Chronic Disease
  • Clinical Decision-Making*
  • Genetic Predisposition to Disease
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lung / drug effects
  • Lung / physiopathology*
  • Phenotype
  • Predictive Value of Tests
  • Prognosis
  • Sarcoidosis, Pulmonary / diagnosis*
  • Sarcoidosis, Pulmonary / drug therapy
  • Sarcoidosis, Pulmonary / genetics
  • Sarcoidosis, Pulmonary / physiopathology
  • Severity of Illness Index

Substances

  • Adrenal Cortex Hormones
  • Biological Products
  • Immunosuppressive Agents