Upadacitinib as a potential management option for diffuse cutaneous systemic sclerosis: A case report.

Area of Science:

• Rheumatology
• Immunology
• Dermatology

Background:

• Systemic sclerosis (SSc) is a multisystem autoimmune disease characterized by vasculopathy, immune dysfunction, and fibrosis.
• SSc presents with diverse clinical manifestations, complicating treatment standardization and affecting multiple organs.
• Diffuse cutaneous SSc (dcSSc) involves significant skin thickening and can lead to severe complications.

Observation:

• A 52-year-old woman with a 14-year history of dcSSc presented with severe, refractory disease.
• Manifestations included Raynaud's phenomenon with digital ulcers, sclerodactyly, telangiectasias, esophageal involvement, interstitial lung disease, and calcinosis.
• Previous treatments (nintedanib, sevelamer, colchicine, tadalafil, prednisone, mycophenolate mofetil) failed to control her multisystem disease.

Findings:

• A trial of upadacitinib was initiated for this treatment-resistant SSc patient.
• Upadacitinib treatment led to notable improvements in both vascular and cutaneous symptoms.
• These clinical benefits were observed despite the patient's refractory and multisystemic disease presentation.

Implications:

• Upadacitinib demonstrates potential as a therapeutic option for severe, treatment-resistant systemic sclerosis.
• Targeting specific inflammatory pathways may offer new avenues for managing complex autoimmune conditions like SSc.
• Further research is warranted to explore the efficacy and safety of upadacitinib in a broader SSc patient population.