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

Abstract

BackgroundThose 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).MethodsSearches 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.ResultsA 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.ConclusionsThis 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.Electronic supplementary materialThe online version of this article (10.1186/s12890-018-0651-3) contains supplementary material, which is available to authorized users.

Investigation burden for patients with fibrotic interstitial lung disease at the end of life

“…Numerous clinical audits and research studies have confirmed the high burden of skin infections and hypothesized that early treatment may reduce the risk and subsequent morbidity and mortality associated with acute rheumatic fever (ARF), acute post-streptococcal glomerulonephritis (APSGN) and skin related sepsis. 3 There is a gap in our understanding of the overall incidence of complicated skin infections across all age groups.…”

“…2 People with IPF also experience a significant symptom burden with dyspnoea and cough. 3 Despite the terminal nature of the disease, people with fibrotic ILD (f-ILD) are often unaware of their prognosis and unprepared for end-of-life care decisions. 4 While the American Thoracic Society and British Thoracic Society ILD guidelines recommend early palliative care involvement, 1,4,5 some studies from overseas demonstrate that few people with f-ILD access such care and those who do are referred only late at the very end of life.…”

Palliative care for patients with pulmonary fibrosis: symptom relief is essential

“…Symptom burden plagues patients with progressive fibrotic interstitial lung disease (PF-ILD) [1]; this symptom burden, in combination with poor prognosis and uncertainty of disease course heavily impacts quality of life, both for patients and family members [2,3]. Patients with PF-ILD are confronted with a wide range of diagnoses and prognoses; some may live many years with a disease that is responsive to treatment, while others with PF-ILD often have a disease trajectory similar to and/or worse than that of lung cancer [4,5].…”

“…Patients suffer common symptoms related to their chronic lung disease which contributes greatly to their morbidity and impacts their quality of life [1]. Symptoms patients most frequently experience include dyspnoea, cough, fatigue, anxiety and depression, and patients experience varying degrees of symptom burden and functional decline as their PF-ILD progresses [1,2].…”

Clinical significance of self-reported cough intensity and frequency in patients with interstitial lung disease: a cross-sectional study

“…Nonproductive cough is a prominent symptom of interstitial lung disease (ILD) [1], and it has been suggested that cough associated with ILD should be assessed in clinical trials [2]. The prevalence of cough has been reported to be as high as 84% in patients with idiopathic pulmonary fibrosis (IPF) [3], 73% in those with scleroderma-related ILD [4], and 83% in those with chronic hypersensitivity pneumonitis (CHP) [5].…”