Scleroderma-related interstitial lung disease: principles of management.

INTRODUCTION: Interstitial lung disease (ILD) is the most common cause of mortality in systemic sclerosis; accounting for approximately 35% of deaths. Although immunosuppression is currently opted as first line therapy for scleroderma-related ILD (SSc-ILD), the benefits from it remain modest with concerns for systemic toxicity from long term use. Areas covered: We review the important facets in monitoring a patient with SSc-ILD, including recognizing various patterns of ILD, identifying those at risk for disease progression and discuss the strength of evidence for immunosuppressant drugs and lung transplantation. We also discuss the potential role of anti-fibrotic agents and the existing evidence for myeloablative stem-cell transplantation. Expert commentary: Non-specific interstitial pneumonia (NSIP) is the most common radiologic and histopathologic pattern seen, but other forms of ILD may also be appreciated. Mycophenolate mofetil and cyclophosphamide are most commonly used as first line therapy for SSc-ILD; however, the efficacy of mycophenolate is comparable to cyclophosphamide with a better tolerability profile. Selected patients with SSc-ILD may be candidates for lung transplantation, although meticulous assessment for co-morbidities is crucial. Further studies are required to deduce the role of anti-fibrotic medications, biologic agents and effects of myeloablative stem cell transplantation in SSc.