Symptoms of pulmonary fibrosis

Nurses should be conscious of the common symptoms of ILD in their patients1–6

Diagnosis of pulmonary fibrosis


Certain symptoms, including dry cough and shortness of breath, are common to most ILDs that can develop pulmonary fibrosis.1,3,5,8–10

Patients should be made aware that pulmonary fibrosis symptoms may gradually develop over months or years,7 and can vary from person to person,1 so their symptoms may be different to other patients.1,11–15

Symptoms common to most ILDs1,9


A dry cough that doesn’t get better


Shortness of breath, especially with mild physical activity

Icon of patient with finger clubbing.

For IPF, finger clubbing* (widening and rounding of the tips of fingers)

*Finger clubbing is a sign of IPF and other fibrotic ILDs;3,16 patients with SSc-ILD may experience symptoms on their hands, for example puffy hands and skin thickening17 and may find this symptom confusing.

Ensure your patients are aware of the symptoms that may indicate pulmonary fibrosis1,3,5,8–10 and confirm that they are prepared to talk to their doctor about them4,6,12,13


Pulmonary fibrosis symptoms can be non-specific and shared with other more common conditions, such as asthma and chronic obstructive pulmonary disease (COPD).1,9,21–24

The Velcro-like crackles of fibrotic ILDs on lung auscultation are distinct from coarse crackles of other lung conditions, such as chronic bronchitis and severe pulmonary edema25,26 and predicts the presence of pulmonary fibrosis.18

Initial symptoms of fibrotic ILDs can be similar to some common conditions1,9,21–24


Listen to the sound of:

Fibrotic ILDs recording 1
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Fibrotic ILDs recording 2
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Fibrotic ILDs recording 4
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Fibrotic ILDs recording 6
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Fibrotic ILDs recording 8
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Healthy recording 1
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Healthy recording 2
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COPD recording 1
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COPD recording 2
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Initial symptoms of fibrotic ILDs, such as dry cough and dyspnea, can be nonspecific,3,10 promptly refer your patients to specialist doctors for further investigations when pulmonary fibrosis is suspected10,28


It may be difficult for patients with underlying systemic diseases to identify whether their symptoms are caused by pulmonary fibrosis, or by the underlying disease and associated treatment.1,30 For example, it may be useful to explain the differences between finger clubbing, which is a symptom of pulmonary fibrosis for patients with IPF and other fibrotic ILDs,3,16 and the puffy hands and skin thickening that patients with SSc-ILD may experience.17

Patients rarely describe breathlessness as ‘shortness of breath’ or ‘breathlessness’, so it is important to understand patients’ language and listen out for hints that may help you identify fibrotic ILDs in your patients.29



*Survey of 6 multicenter focus groups including 45 patients with CTD-ILDs (IIM-ILD (n=11), RA-ILD (n=13), SSc-ILD (n=17), and various other CTD diagnoses (n=4)). Traditional descriptors such as ‘shortness of breath’ or ‘breathlessness’ were seldom voiced, instead phrases such as these were used.29

Help your patients understand and recognize the symptoms of fibrotic ILD with the Living with Pulmonary Fibrosis ebook


Listen out for subtle hints of fibrotic ILD symptoms when talking to patients29 and promptly refer to specialist doctors for further investigations when pulmonary fibrosis is suspected10,28

What other resources can help you support your patients in their journey with fibrotic ILD?

  • COPD, chronic obstructive pulmonary disease; CTD, connective tissue disease; CTD-ILD, connective tissue disease-associated interstitial lung disease; IIM-ILD, idiopathic inflammatory myopathy; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; PPF, progressive pulmonary fibrosis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SSc-ILD, systemic sclerosis-associated interstitial lung disease.  

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  17. Young A, Namas R, Dodge C, et al. Hand impairment in systemic sclerosis: Various manifestations and currently available treatment. Curr Treatm Opt Rheumatol. 2016;2:252–269.
  18. Sgalla G, Walsh SLF, Sverzellati N, et al. “Velcro-type” crackles predict specific radiologic features of fibrotic interstitial lung disease. BMC Pulm Med. 2018;18(1):103.
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  22. Johnson MJ, Bland JM, Gahbauer EA, et al. Breathlessness in elderly adults during the last year of life sufficient to restrict activity: prevalence, pattern, and associated factors. J Am Geriatr Soc. 2016;64(1):73–80.
  23. Theander K, Hasselgren M, Luhr K, et al. Karlsson I. Symptoms and impact of symptoms on function and health in patients with chronic obstructive pulmonary disease and chronic heart failure in primary health care. Int J Chron Obstruct Pulmon Dis. 2014;9:785–794.
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  26. Sarkar M, Madabhavi I, Niranjan N, et al. Auscultation of the respiratory system. Ann Thorac Med. 2015;10(3):158–168.
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Resources for patients