What are the most likely signs and symptoms?
Recognising the symptoms of idiopathic pulmonary fibrosis (IPF) is not always easy because they are very similar to other lung conditions, as well as cardiovascular disease.1,2 A 2018 survey by national charity Action for Pulmonary Fibrosis (APF) found that 35% of people with IPF in the UK had been diagnosed with other conditions such as asthma, chronic obstructive pulmonary disease (COPD) or a heart problem, with 54% waiting more than 6 months to be diagnosed correctly with IPF and 20% waiting more than 2 years.3
Knowing the clinical features of IPF and referring patients where the disease is suspected to a specialist could mean a patient has access to appropriate specialist care early in their disease, with a potential positive impact on their prognosis.4 However, the symptoms of IPF are very common and so it would be inappropriate to refer all patients presenting with breathlessness, dry cough and fatigue.1,2 Therefore, it is important for GPs to understand the specific clinical features of IPF.
The key clinical features of IPF to be aware of when assessing/testing a patient include:
- Persistent breathlessness on exertion.5 You may notice this when the patient walks into the consulting room, but you could also ask the patient to walk a short distance around the building to observe their breathing
- A persistent, often dry cough5,6
- The sound of distinctive bilateral inspiratory crackles when listening to the basal areas of the lung.5 These are sometimes described as ‘Velcro-like’ crackles, and it is suggested that the assessment of these crackles is currently the only realistic means for the earlier diagnosis of IPF.7 Examples can be heard here:
- Clubbed fingers where the tips become rounded and swollen and the nails curve around the top of the finger5 Note: Clubbing may be associated with various clinical conditions – for full list see Sarkar M, 20128
- Normal or impaired spirometry, usually with a restrictive pattern but sometimes with an obstructive pattern, may also be a red flag for IPF5
Example of clubbed fingers.
There is increasing evidence that patients with pulmonary fibrosis (PF) also present with emphysema, a form of COPD.9,10 Patients with both IPF and emphysema face a higher mortality compared to those with IPF without emphysema.11
“IPF symptoms are similar to those in other lung conditions, which makes it a lot harder for GPs to disentangle them. For example, in both IPF and COPD, patients typically present with a cough – usually dry in IPF and variable in COPD – and breathlessness, which could also be linked to other underlying conditions, such as anxiety, obesity, heart failure, atrial fibrillation and aortic stenosis.
In both cases, patients are likely to be over 40 year old and have a history of smoking, although this tends to be more common in COPD. However, many patients with IPF may have basal ‘Velcro-like’ crackles bilaterally and quite often a chest X-ray may be abnormal. The degree of breathlessness and hypoxia can be more in IPF than COPD. If a patient is being treated for COPD, but actually has IPF, they won’t respond to inhalers.
As you can see, the picture can be very complicated, so if you think a patient might have a different lung condition, if the diagnosis is not clear and not fitting a familiar pattern or if symptoms and current treatments do not follow what you would expect, my advice is to refer the patient to a respiratory specialist as early as possible.”
1Hayen A, Herigstad M, Pattinson KTS. Understanding dyspnea as a complex individual experience. Maturitas 2013;76(1):45–50
2National Institute for Health and Care Excellence (NICE). Chronic obstructive pulmonary disease in over 16s: diagnosis and management [NG115]. Last accessed November 2019
3Action for Pulmonary Fibrosis. Patient survey report 2018: Giving patients a voice. Available at: www.actionpulmonaryfibrosis.org/wp-content/uploads/2019/03/APF-Report-Final-070319-crop.pdf. Last accessed November 2019
4du Bois RM. An earlier and more confident diagnosis of idiopathic pulmonary fibrosis. Eur Respir Rev 2012;21(124):141-146
5National Institute for Health and Care Excellence (NICE). Idiopathic pulmonary fibrosis in adults: diagnosis and management. CG163. Available at: https://www.nice.org.uk/guidance/cg163/resources/idiopathic-pulmonary-fibrosis-in-adults-diagnosis-and-management-pdf-35109690087877. Last accessed November 2019
6Senanayake S et al. Patients’ experiences of coping with Idiopathic Pulmonary Fibrosis and their recommendations for its clinical management. PLoS ONE 2018;13(5): e0197660
7Cottin V, Cordier JF. Velcro crackles: the key for early diagnosis of idiopathic pulmonary fibrosis? Eur Respir J 2012;40(3):519-521
8Sarkar M, Mahesh DM, Madabhavi I. Digital clubbing. Lung India 2019;29(4):354–362
9Jancowich MD, Rounds SIS. Combined pulmonary fibrosis and emphysema syndrome: a review. Chest 2012; 141(1):222-231
10NHS. Overview - Chronic obstructive pulmonary disease (COPD). Available at: https://www.nhs.uk/conditions/chronic-obstructive-pulmonary-disease-copd/. Last accessed November 2019
11Mejía M et al. Idiopathic Pulmonary Fibrosis and Emphysema: Decreased Survival Associated With Severe Pulmonary Arterial Hypertension. Chest 2009;136(1):10-15