Christian D Becker, MD, PhD

Idiopathic pulmonary fibrosis (IPF) is a chronically progressive, irreversible and ultimately fatal fibrosing lung disease. The disease affects many patients worldwide without any geographical or racial predilection. The prevalence in the US is estimated at around 20 cases per 100,000 for males and 13 cases per 100,000 for females. The disease imposes both a high symptom burden and an ultimately fatal prognosis on affected patients and their families. The median survival time after diagnosis currently is only 2.5 to 3.5 years. Our understanding of this disease, its cause(s) and underlying pathogenetic mechanisms is poor. There is no effective treatment available to date. Secondary forms of pulmonary fibrosis affect a much higher number of patients, but exact numbers on prevalence, morbidity and mortality are difficult to estimate given the heterogeneity of the different underlying conditions. A characterization of how the different dendritic cell subsets are dysfunctional in pulmonary fibrosis will help to better understand the underlying pathogenetic mechanisms and may offer new insights into therapeutic options to halt the progression of the fibrotic remodeling process in patients afflicted with this condition.