Introduction: Idiopathic Pulmonary Fibrosis (IPF) is a unique type of interstitial pneumonia in which progressive fibrosis can ultimately result in respiratory failure and death. The median survival of IPF remains dismal despite newer anti-fibrotic therapies. Lung transplantation is the only modality currently known to enhance survival for patients with IPF. Areas covered: Since IPF is predominantly a disorder of the elderly, determination of the impact of co-morbidities is crucial for risk stratification of the individual patient. We review the potential effect of anti-fibrotic therapy in the pre and post-transplant period; and also discuss the factors that need to be considered in deciding between single lung and bilateral lung transplantation. Expert commentary: Early referral to a transplant center is recommended for patients with IPF due to the high waiting list mortality. Evaluation of the transplant candidate should also be directed specifically at identifying co-morbidities that portend higher risk. While there has been a universal trend favoring bilateral lung transplantation over single lung transplantation for IPF, there are inherent pros and cons for both strategies and decisions should be individualized. Further studies are required to deduce the efficacy and safety of anti-fibrotic drugs in the immediate pre-and post-lung transplantation period.

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.