Diagnosis

Diagnosis of idiopathic pulmonary fibrosis (IPF) is challenging and requires careful evaluation of clinical, laboratory, radiological and/or pathological data and careful exclusion of other disease aetiologies.1

Characteristic IPF inspiratory crackles by lung auscultation are valuable in early diagnosis. Fine crackles are already detected in early stages of disease and are present in more than 80% of IPF patients.2 

In most cases, when family and medical history, physical examination, physiological testing and laboratory data are available, ILD experts can diagnose IPF when high-resolution computed tomography (HRCT) reveals a usual interstitial pneumonia (UIP) pattern.1

The accuracy of IPF diagnosis improves with multidisciplinary discussion between pulmonologists, radiologists, and pathologists, particularly if the radiological and histopathological data are inconsistent.1

References

  1. Raghu G, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788–824.
  2. Borchers AT, et al. Idiopathic pulmonary fibrosis-an epidemiological and pathological review. Clin Rev Allergy Immunol 2011;40:117–134.

Related video

Luca Richeldi and Vincent Cottin discuss the importance of early diagnosis.

Luca Richeldi and Vincent Cottin discuss the importance of early diagnosis.