Use of Selective JAK Inhibitor in ILD and Skin Tightness of Systemic Sclerosis Versus MMF

Systemic sclerosis (SSc) is a systemic autoimmune disease with heterogeneous clinical presentation and prognosis. JAK inhibitors reduced cutaneous and pulmonary fibrosis in mice models of SSc. Clinical data regarding the efficacy and safety of JAK inhibitors in SSc patients are scarce. Upadacitinib mechanism of action JAK-STAT pathways are composed of four JAK kinases and seven STATs (STAT1-6, including homologs STAT5a and STAT5b). A cytokine binding to its receptor initiates the signaling cascade as well as the subsequent association/rearrangement of the receptor subunits. This rearrangement enables JAK activation by transphosphorylation and, upon activation, JAKs phosphorylate the receptors.

Inclusion criteria

• all patients are systemic sclerosis disease duration more than 3 years limited and diffuse types ground glass at HRCT DLCO< 80% decline in FVC >10% in last 12months

Exclusion criteria

• associated other CT diseases disease duration < 3 years kidney disease