Purpose of review
To review the recent medical literature pertaining to interstitial lung disease found in association with systemic sclerosis remains a major contributor to morbidity and mortality. Significant progress is being made in terms of understanding the pathogenesis, the best approaches to clinical evaluation, and various options for therapy of systemic sclerosis patients whose disease course is complicated by interstitial lung disease.
Recent studies highlight the importance of microvascular disease, autoimmunity, and fibroblast differentiation/activation in the pathogenesis of systemic sclerosis-interstitial lung disease, particularly in the early phase of disease. It appears as if the balance between various pro-fibrotic/pro-inflammatory and anti-fibrotic/anti-inflammatory mediators may be central to interstitial lung disease pathogenesis, which presents potential opportunities for therapeutic intervention. The clinical approach to staging of disease activity remains controversial. High resolution computed tomography scans, bronchoalveolar lavage and various serum markers (e.g., surfactant protein D and KL-6) each may provide useful information about the degree of activity of the systemic sclerosis-interstitial lung disease. Currently, treatment recommendations are limited by a scarcity of well designed clinical trials, but the recently completed Scleroderma Lung Study is a model for future studies and is providing useful information about this important complication of systemic sclerosis.
Basic and clinical studies of systemic sclerosis patients with interstitial lung disease are yielding promising data that ultimately will be translated in to more effective diagnostic and therapeutic strategies.