Symptom prevalence of patients with fibrotic interstitial lung disease: a systematic literature review

Background: Those affected by advanced fibrotic interstitial lung diseases have limited treatment options and in
the terminal stages, the focus of care is on symptom management. However, quantitatively, little is known about
symptom prevalence. We aimed to determine the prevalence of symptoms in Progressive Idiopathic Fibrotic
Interstitial Lung Disease (PIF-ILD).
Methods: Searches on eight electronic databases including MEDLINE for clinical studies between 1966 and 2015
where the target population was adults with PIF-ILD and for whom the prevalence of symptoms had been
calculated.
Results: A total of 4086 titles were screened for eligibility criteria; 23 studies were included for analysis. The highest
prevalence was that for breathlessness (54–98%) and cough (59–100%) followed by heartburn (25–65%) and
depression (10–49%). The heterogeneity of studies limited their comparability, but many of the symptoms present
in patients with other end-stage disease were also seen in PIF-ILD.
Conclusions: This is the first quantitative review of symptoms in people with Progressive Idiopathic Fibrotic
Interstitial Lung Diseases. Symptoms are common, often multiple and have a comparable prevalence to those
experienced in other advanced diseases. Quantification of these data provides valuable information to inform the
allocation of resources.
Keywords: Pulmonary fibrosis, Symptom prevalence and interstitial lung disease