Figure 4.

Possible role of antifibrotics in the management of IIM-ILD. Schematic of how antifibrotics might be employed in the management of IIM-ILD. Key outstanding questions are (1) efficacy of upfront antifibrotic therapy in all patients both in the mild-moderate category to improve outcomes, (2) efficacy of early initiation of antifibrotics to prevent ILD in high-risk patients (e.g. +MDA5 or Ro52), (3) efficacy in severe/rapidly progressing ILD to improve survival, and (4) at failure of induction or maintenance therapy for additive benefit and to slow progression.

*Tacrolimus could be considered as a first-line agent for the management of MDA5 + dermatomyositis with ILD in the outpatient setting; tofacitinib could be considered as early therapy in life-threatening MDA5+ disease.