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Coagulation disorders and the IPF puzzle
  1. David B Coultas
  1. Correspondence to Dr David B Coultas, Department of Medicine, The University of Texas Health Science Center-Tyler, Tyler, TX 75708 USA; david.coultas{at}

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While idiopathic pulmonary fibrosis (IPF) remains a frustrating mystery for patients and their physicians, growing evidence from a number of different sources provides potentially clinically relevant clues to the puzzle of this largely untreatable and highly fatal disorder.1 ,2 Answers to frequently asked questions from patients of ‘why me?’ ‘what is the cause?’ ‘what can be done?’ and ‘what can I expect?’ are gradually emerging from studies of pathogenesis, clinical trials and epidemiological investigations.

For over three decades, the majority of IPF research has focused on pathogenesis and clinical trials of various therapeutic agents.2 Available evidence on pathogenic mechanisms suggests that pulmonary fibrosis results from a number of exposures that cause alveolar epithelial injury (eg, cigarette smoke, asbestos fibres, viruses) followed by activation of a complex set of abnormal, genetically determined biological pathways that result in fibrosis.3 Altering the course of the disease remains a challenge partly because of the complexity of the pathogenesis of IPF, and time needed for discovery and clinical trials of new agents to determine efficacy. Moreover, despite the growing number of clinical trials among patients with symptomatic IPF the results have been disappointing. Therefore, parallel research approaches are needed on interventions to prevent the occurrence (ie, primary prevention) or progression of subclinical pulmonary fibrosis (ie, secondary prevention).

For any disease, the ultimate goal is primary prevention through elimination of exposures that cause the disease. While epidemiological evidence suggests this may be a possible approach for IPF, there are also major challenges. Exposure to a number of environmental and occupational agents may cause IPF,4 and control of these exposures offers the potential to prevent the development of IPF. For example, based …

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