The diagnosis of idiopathic pulmonary fibrosis and its complications

Expert Opin Med Diagn. 2008 Dec;2(12):1317-31. doi: 10.1517/17530050802549484.


Background: Idiopathic pulmonary fibrosis (IPF) is a devastating, progressive condition with a median survival of 2.8 - 4 years from diagnosis. Clinicians confronted with a patient with fibrosing lung disease need to be reliably able to distinguish IPF from other diffuse parenchymal lung diseases. Furthermore, they need to be able to gauge prognosis, evaluate timing of interventions including referral for transplant, assess reliably the effectiveness of treatment and be able to detect rapidly the development of disease complications.

Objective/method: This paper provides an overview of currently available diagnostic tests for IPF and its complications and evaluates the possible future role of candidate biomarkers in the diagnosis and assessment of patients with IPF. A literature search was performed for papers evaluating diagnostic tests in the diagnosis of IPF and its complications.

Conclusion: Computed tomography combined with clinical data is sufficient for diagnosing IPF in approximately two-thirds of patients with the condition. For the remaining patients, histological assessment is important in achieving a precise diagnosis. Serial measurements of carbon monoxide diffusing capacity and forced vital capacity provide the best prognostic indicator in IPF. Potential biomarkers for diagnosing IPF include KL-6, MMP1 and MMP7. Brain naturetic peptide shows promise as a non-invasive screening tool for the diagnosis of IPF-associated pulmonary hypertension.