Pulmonary hypertension due to interstitial lung disease or chronic obstructive pulmonary disease: a patient experience study of symptoms and their impact on quality of life

Abstract

Pulmonary hypertension (PH) resulting from chronic lung disease such as chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) is categorized by the World Health Organization (WHO) as Group 3 PH. To identify the symptoms and impacts of WHO Group 3 PH and to capture data related to the patient experience of this disease, qualitative research interviews were undertaken with 3 clinical experts and with 14 individuals with PH secondary to COPD or ILD. Shortness of breath, fatigue, cough, and swelling were the most frequently reported symptoms of PH due to COPD or ILD, and shortness of breath was further identified as the single most bothersome symptom for most patients (71.4%). Interview participants also described experiencing a number of impacts related to PH and PH symptoms, including limitations in the ability to perform activities of daily living and impacts on physical functioning, family life, and social life as well as emotional impacts, which included frustration, depression, anxiety, isolation, and sadness. Results of these qualitative interviews offer an understanding of the patient experience of PH due to COPD or ILD, including insight into the symptoms and impacts that are most important to patients in this population. As such, these results may help guide priorities in clinical treatment and assist researchers in their selection of patient-reported outcome measures for clinical trials in patients with PH due to COPD or ILD.

Screening Strategies for Pulmonary Hypertension in Patients With Interstitial Lung Disease

“…1 PH associated with ILD is associated with worse outcomes, including an approximately threefold increased risk of mortality, a heightened propensity for acute exacerbations, impaired quality of life, decreased exercise capability, and increased need for supplemental oxygen. [2][3][4][5][6][7][8][9][10] The reported prevalence of PH in ILD varies based on the underlying population studied, the disease severity, and the method used for diagnosing PH. This has resulted in a wide range of reported prevalence rates, anywhere from 3% to 86% in different studies.…”

Pulmonary hypertension in interstitial lung disease and in chronic obstructive pulmonary disease: different entities?

“…The clinical picture of PH may be similar in COPD and in ILD: severe dyspnea [60] especially on exertion [66], desaturation, palpitations [67 ] are frequent in severe PH, although in COPD peripheral edema may exist with preserved right heart function, due to the activation of the renin-angiotensin-aldosterone pathway [68]. Worsening dyspnea, hypoxemia or desaturation on exertion with stable lung function and/or imaging should prompt PH suspicion [67 & ,69].…”

“…2). These clinical manifestations may significantly impact quality of life [66], although patient-reported outcomes have not yet been adequately studied in Group 3 PH.…”

“…The clinical picture of PH may be similar in COPD and in ILD: severe dyspnea [60] especially on exertion [66], desaturation, palpitations [67 ▪ ], dizziness or chest pain. Symptoms and sign of right heart failure [67 ▪ ] are frequent in severe PH, although in COPD peripheral edema may exist with preserved right heart function, due to the activation of the renin-angiotensin-aldosterone pathway [68].…”

Interstitial lung disease-associated pulmonary hypertension – what the future holds

“…Pulmonary hypertension has a dramatic impact on the outcome of patients with fILD including quality of life [16]. Patients with WHO Group 3 pulmonary hypertension have a worse prognosis than those with pulmonary hypertension from other groups including those with Group 1 pulmonary arterial hypertension (PAH) [17][18][19][20].…”