Parenchymal lung disease is an important complication of autoimmune rheumatic disease. There are a variety of patterns recognised; non-specific interstitial pneumonia (NSIP) and organising pneumonia (OP) being most prevalent but other processes include usual interstitial pneumonia (UIP), diffuse alveolar damage (DAD), reactive pulmonary lymphoid hyperplasia, desquamative interstitial pneumonia (DIP) and respiratory bronchiolitis-associated interstitial lung disease (RBILD). Pulmonary involvement is the leading cause of morbidity and mortality in Systemic Sclerosis. A variety of immunosuppressive agents have been evaluated as potential disease-modifying therapies in systemic sclerosis related interstitial lung disease (SSc-ILD). Two double-blind randomised controlled trials examined the efficacy of cyclophosphamide in SSc-ILD; the Scleroderma lung study (SLS) and the Fibrosing Alveolitis in Scleroderma Trial (FAST). Although each trial compared cyclophosphamide to placebo, there were important differences in the route of administration and the types of concurrent treatment in the active therapy arm. There are emerging data supporting mycophenolate mofetil (MMF) in SSc-ILD, which is both safe and well tolerated. A number of case reports have highlighted a potential role for rituximab in SSc-ILD. Cases will be presented that reflect the diversity of interstitial lung disease seen in the rheumatology clinic and current approaches to therapy reviewed including treatments of gastro-oesophageal reflux disease (GORD), potential of antioxidant therapies and the place for second line immunosuppression. Finally consideration of future approaches such as autologous stem cell transplantation and targeted anti-fibrotic agents such as pirfenidone or selective signalling inhibitors such as nintedanib will be discussed.
Disclosure of Interest None Declared