TABLE 2

Effects of interferonopathy (IFN)-signalling blockade on lung involvement in monogenic interferonopathies

First author [ref.]TreatmentDiseaseLung involvementEffect on IFN signatureClinical response/outcomeSide-effects
Frémond [97]RuxolitinibSAVI (n=3)ILD (n=2)Partial inhibitionImprovementNone
Chia [119]BaricitinibSAVI (n=1)ILD (n=1)NDImprovementNA
Sanchez [100]BaricitinibSAVI (n=4)
CANDLE (n=10)
ILD (n=4)
PAH (n=1)
InhibitionImprovementND
Buchbinder [104]TofacitinibCANDLE (n=1)PAH (n=1)NDInitial improvementNone
Saldanha [103]RuxolitinibSAVI (n=1)PAH (n=1)InhibitionImprovementNone
Yu [102]TofacitinibSAVI (n=1)PAH (n=1)NDPartial improvement/sudden death (unknown cause)NA
Boyadzhiev [133]BaricitinibCANDLE (n=1)ND (n=1)NDImprovementNone
Trombetta [123]RuxolitinibDNAse II deficiency (n=1)PAH (n=1)InhibitionImprovementNone
Volpi [101]RuxolitinibSAVI (n=3)ILD (n=3)
PAH (n=1)
Partial inhibitionImprovement (transitory: n=2; persistent: n=1)Severe viral infection (n=1)
Frémond [134]RuxolitinibCOPA (n=1)DAH (n=1)InhibitionPartial improvementNone

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.