Author(s): Duarte, Ana Catarina ; Santos, Maria José ; Cordeiro, Ana
Date: 2018
Persistent ID: http://hdl.handle.net/10400.26/30503
Origin: Egas Moniz - Cooperativa de Ensino Superior, CRL
Subject(s): Anti-fibrotic therapy; Interstitial lung disease; Nintedanib; Systemic sclerosis
Systemic sclerosis is a connective tissue disease characterized by progressive skin thickening and a wide spectrum of internal organ involvement. Pathogenesis includes vasculopathy, inflammation, and fibrosis. Although immunosuppressants such as cyclophosphamide and mycophenolate mofetil have shown some benefit in interstitial lung disease management, it is still a major cause of morbi-mortality in these patients. Therefore, there is a current need for new therapies. Here, we report a 65-year-old female patient with limited cutaneous systemic sclerosis, anti-topoisomerase-positive and extensive lung disease. The patient developed progressive lung fibrosis under several immunosuppressants and was started on nintedanib, with clinical and functional stabilization. Nintedanib is a tyrosine-kinase inhibitor that blocks several profibrotic pathways, inhibiting proliferation and migration of fibroblasts and decreasing the synthesis of extracellular matrix proteins. It is approved for idiopathic lung fibrosis and has demonstrated good results in inhibiting migration and proliferation of systemic sclerosis dermal fibroblasts, constituting a promising agent for systemic sclerosis-associated lung fibrosis.
