[From renal crisis to lung fibrosis - systemic sclerosis in the course of time]

Systemic sclerosis (SSc) is a connective tissue disease of multifactorial origin in which autoimmune inflammatory reactions lead to fibrosis of multiple tissues. In the past, renal crisis was a common complication with a very high mortality. Due to the recommendation for a more cautious use of corticosteroids and the use of ACE inhibitors as an acute treatment reduced the incidence of a renal crisis and improved overall survival since the 1980s. However, lung involvement including pulmonary arterial hypertension, interstitial lung disease and lung fibrosis is now the most common cause of death in SSc. An early detection, including the use of HR-CT screening, and adequate treatment of interstitial lung disease are therefore of the utmost importance. Mycophenolate mofetil (MMF) has proven to be an effective therapeutic agent for the pulmonary manifestation. Nintedanib is the only drug approved in Germany for SSc-associated progressive lung fibrosis. Studies have shown the best prognostic improvements with early combination therapy of MMF in combination with Nintedanib.