Interstitial Lung Disease Due to Systemic Disease (Disorder)
Conditions
Keywords
ILD
Brief summary
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
Detailed description
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.
Interventions
oral tablet upadacitinib
DEVICECellcept Oral Product
active control group 2000mg MMF daily
Sponsors
Assiut University
Study design
Allocation
RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
TRIPLE (Subject, Investigator, Outcomes Assessor)
Intervention model description
Randomised double blinded clinical trial
Eligibility
Sex/Gender
ALL
Age
18 Years to 65 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| forced vital capacity | 52 week | FVC reduction of 15% |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| High resolution chest computed tomography | 52 week | HRCT chest pattern changes ground glass appearance |
| modefided Rodnan skin score | 52 week | skin score for tightness grade more than 14 is worse |
Countries
Egypt
Outcome results
None listed