Summary of the main areas of application of low-dose, long-term macrolides in diffuse interstitial lung diseases (ILDs)

Macrolide uses and functionsEvidence typeFirst author [ref.]
In ILDs, including idiopathic pulmonary fibrosis
 Macrolides may play an antifibrotic roleStill to be demonstrated in human models
  Promoters of autophagocytosis and clearance of intracellular   protein aggregatesOnly cellular modelsGuillot [1]
Thomson [8]
Stamatiou [9]
  Regulators of surfactant homeostasisOne case reportThouvenin [10]
 Macrolides may act as host–microbiota interaction modulators    and/or host immune response modulatorsStill to be demonstrated in human models
  Reduction of acute exacerbations rateRetrospective monocentric studyKuse [33]
In organising pneumonia
 Macrolides may exert an anti-inflammatory/immunomodulatory actionCase reports and case series
  As adjuvants to steroid therapy and steroid-sparing agentsOne case reportDing [51]
  As first-line agents as an alternative to steroidsCase reports and case seriesIchikawa [45]
Stover [46]
Hotta [49]
Radzikowska [44]
  As second-line agents when steroids are not tolerated or failedCase reports and case seriesChang [52]
Stover [46]
Pathak [53]