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.