New approaches to modulating idiopathic pulmonary fibrosis.

Until recently, idiopathic pulmonary fibrosis (IPF) has been a devastating and generally fatal disease with no effective therapeutic. New developments in understanding the biology of the disease include a growing consensus that the lesions are mainly composed of cells that originated from resident fibroblasts. New developments in therapeutics include recommendations against several treatment regimes that have been previously used. On a positive note, the orally available drug pirfenidone has been approved for use in IPF in China, Japan, India, and the European Union, but not yet in the United States. Other possibilities for managing IPF include managing gastrointestinal reflux, and limiting excessive salt intake. A variety of potential therapeutics for IPF are in clinical trials; for instance, in a Phase 1b trial, intravenous injections of a recombinant version of the normal human serum protein Serum Amyloid P (SAP, also known as PTX2) improved lung function in IPF patients.