What are the signs and symptoms of IPF?

Symptoms of IPF are gradual in onset, initially presenting as dyspnoea (shortness of breath) upon exertion and a dry cough.1 These early, non-specific symptoms are commonly mistaken for ageing, cardiac disease, emphysema, bronchitis, asthma or COPD.2,3 Such misdiagnosis, or ignoring the early symptoms entirely, may explain why IPF is rarely diagnosed immediately. Often a correct diagnosis may take several months or even years.2,3

A common sign of IPF is bibasilar inspiratory ‘Velcro crackles’ on lung auscultation.4 These occur in >80% of patients and can be detected when a physician listens to the sounds with a stethoscope made by the lungs during inspiration. The crackles appear firstly in the basal areas of the lung, where the disease initiates, with further progression to the upper zones of the lungs.5

Another symptom of IPF is finger clubbing, where the fingertips and fingernails spread out and become rounder. Finger clubbing occurs in 25–50% of patients.1,4

Other extrapulmonary symptoms, i.e. those unrelated to the lungs, are rare, but weight loss, malaise, and fatigue may be seen in some patients.1

Symptoms, especially dyspnoea and cough, can cause significant impairment to overall quality of life for patients. Symptoms are also likely to get worse over time, with patients in the later stages of disease noting that dyspnoea curtails physical activity and is extremely distressing.6 Cough has been described by patients as being "dry and non-productive" or "hacking" and "occurring when I talk for long periods", with several mentioning "a nagging desire to cough constantly" and "never feeling relieved after coughing".6 Fatigue is another IPF symptom that patients mention as occurring and reducing their quality of life.6

It is important that IPF patients with worsening dyspnoea or other respiratory symptoms are identified and evaluated promptly.7 Clinical guidelines for the management of IPF developed by an international collaboration of major respiratory societies in the US (ATS), Europe (ERS), Japan (JRS) and Latin America (ALAT), state that these patients should be evaluated for progressive disease, assessed for oxygenation at rest and with exertion, with care taken to identify and address secondary complications, such as e.g. development of deep venous thrombosis and pulmonary embolus.7

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  1. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med 2000;161:646–664.
  2. Collard HR, et al. Patient experiences with pulmonary fibrosis. Respir Med 2007;101:1350–1354.
  3. Meltzer EB, et al. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis 2008;3:8.
  4. Borchers AT, et al. Idiopathic pulmonary fibrosis-an epidemiological and pathological review. Clin Rev Allergy Immunol 2011;40:117–134.
  5. Cottin V, et al. Velcro crackles: the key for early diagnosis of idiopathic pulmonary fibrosis? Eur Respir J 2012;40:519–521.
  6. Swigris JJ, et al. Patients’ perspectives on how idiopathic pulmonary fibrosis affects the quality of their lives. Health Qual Life Outcomes 2005;3:61.
  7. 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.