First author [ref.] | Treatment | Disease | Lung involvement | Effect on IFN signature | Clinical response/outcome | Side-effects |

Frémond [97] | Ruxolitinib | SAVI (n=3) | ILD (n=2) | Partial inhibition | Improvement | None |

Chia [119] | Baricitinib | SAVI (n=1) | ILD (n=1) | ND | Improvement | NA |

Sanchez [100] | Baricitinib | SAVI (n=4) CANDLE (n=10) | ILD (n=4) PAH (n=1) | Inhibition | Improvement | ND |

Buchbinder [104] | Tofacitinib | CANDLE (n=1) | PAH (n=1) | ND | Initial improvement | None |

Saldanha [103] | Ruxolitinib | SAVI (n=1) | PAH (n=1) | Inhibition | Improvement | None |

Yu [102] | Tofacitinib | SAVI (n=1) | PAH (n=1) | ND | Partial improvement/sudden death (unknown cause) | NA |

Boyadzhiev [133] | Baricitinib | CANDLE (n=1) | ND (n=1) | ND | Improvement | None |

Trombetta [123] | Ruxolitinib | DNAse II deficiency (n=1) | PAH (n=1) | Inhibition | Improvement | None |

Volpi [101] | Ruxolitinib | SAVI (n=3) | ILD (n=3) PAH (n=1) | Partial inhibition | Improvement (transitory: n=2; persistent: n=1) | Severe viral infection (n=1) |

Frémond [134] | Ruxolitinib | COPA (n=1) | DAH (n=1) | Inhibition | Partial improvement | None |

SAVI: STING-associated vasculopathy with onset in infancy; STING: stimulator of interferon genes; ND: not determined; ILD: interstitial lung disease; NA: not available; CANDLE: chronic atypical dermatosis with lipodystrophy and elevated temperatures; PAH: pulmonary arterial hypertension; COPA: coatomer protein complex, subunit-α; DAH: diffuse alveolar haemorrhage.