Recent advances in the diagnosis and treatment of interstitial lung disease in systemic sclerosis (scleroderma): a review.

Interstitial lung disease (ILD) in systemic sclerosis (scleroderma) is a rare and potentially lethal and devastating autoimmune disease, and yet the cause of it remains poorly understood. It is associated with tissue fibrosis not only in the lungs, but also other organs like the heart and kidneys. Although ILD can manifest itself in various disease presentations, ILD in systemic sclerosis is particularly and especially worrying because it has the highest case-specific mortality among all autoimmune rheumatic conditions. The unsatisfactory clinical outcome and prognosis for ILD in systemic sclerosis has, unsurprisingly, fuelled an intense search for early and accurate diagnosis, as well as new therapeutic strategies. Recent advances in diagnostic techniques and treatment interventions represent a significant step forward in our understanding and management of ILD in systemic sclerosis. Here, we review the current knowledge pertaining to the treatment of ILD in systemic sclerosis, and also address the various challenges involved as well as implications for the future.