Interstitial lung disease in systemic sclerosis

Interstitial lung disease is an early and serious complication of systemic sclerosis (SSc). Because it may be asymptomatic for a long period, and only the early flogistic phase is at present susceptible to treatment, early diagnosis and identification of risk are critical to the outcome. However, identifying SSc patients at risk for developing interstitial lung disease is at present difficult; therefore, a strict monitoring of the disease, especially in the first years, is mandatory. Treatment strategy is aimed at suppressing inflammation. Unfortunately, optimal therapy has not yet been established. Combination of corticosteroids and cyclophosphamide is considered the best therapeutic approach available so far, but doses and duration of treatment need to be determined. Future research should focus on new anti-inflammatory or immunosuppressive agents.