Prevalence and impact of WHO group 3 pulmonary hypertension in advanced idiopathic nonspecific interstitial pneumonia

Abstract

Pulmonary hypertension (PH) is associated with impaired quality of life, worsened functional status and increased mortality in idiopathic pulmonary fibrosis (IPF) [1]. The prevalence and clinical impact in other idiopathic interstitial pneumonias (IIPs) has not previously been reported. Given the prognostic significance of PH in IPF, we sought to determine the prevalence and severity of World Health Organization (WHO) group 3 PH in a cohort of patients with biopsy-proven idiopathic nonspecific interstitial pneumonia (NSIP). We recognised that the prevalence of PH in this population of patients with advanced NSIP would be an overestimation of the NSIP population at large, but felt that mandating surgical lung biopsy and right heart catheterisation (RHC) were the only way to ensure accurate diagnosis of NSIP and PH, respectively. A retrospective review of patients with idiopathic NSIP diagnosed between 2002 and 2016 at our centre was performed. All cases were reviewed at our multidisciplinary pulmonary pathology conference. Connective tissue disease (CTD) was rigorously excluded in all patients and RHC was required for confirmation of PH. RHCs are not performed standardly on all patients in our clinic, and would have been performed as part of lung transplant evaluation or to confirm PH suggested by clinical parameters or echocardiographic data. PH was defined as a resting mean pulmonary artery pressure (mPAP) ⩾25 mmHg and severe PH as mPAP ⩾35 mmHg. Patients with a pulmonary capillary wedge pressure >15 mmHg were excluded from the cohort as they were suspected to have left-heart disease associated (WHO group 2) PH. Chronic thromboembolic disease was excluded in all patients with ventilation/perfusion scanning. The primary endpoints of this study were the prevalence and impact of WHO Group 3 PH on patient outcomes in idiopathic NSIP. Secondary outcomes included the correlation of forced vital capacity (FVC) and mPAP in idiopathic NSIP patients, as well as the association between other demographics and PH in this population. Review of records from our multidisciplinary pulmonary pathology meeting identified 95 potential patients with biopsy proven NSIP. Of these patients, 35 met criteria for inclusion in the study. 11 (31.4%) out of 35 patients had WHO group 3 PH. The mean±SD mPAP for those with and without WHO group 3 PH were 32.0±10.3 mmHg and 18.1±3.1 mmHg, respectively. Of the 35 patients in the study, seven (20%) patients had a mPAP of 25-30 mmHg, two (5.7%) had a mPAP of 31-34 mmHg and two (5.7%) had a mPAP ⩾35 mmHg. Table 1 details the characteristics of the patients with PH versus those without. Per cent predicted FVC was similar between the WHO group 3 PH and no PH cohorts, with r= −0.12 suggesting poor correlation between FVC per cent predicted and mPAP (p=0.48). Median transplant-free survival was significantly lower in patients with WHO group 3 PH at 17.6 months as compared to 47.9 months in the cohort without PH (p=0.05). Severe PH (mPAP ⩾35 mmHg or mPAP ⩾25 mmHg with a low cardiac index) was only seen i...

Cell dichotomous role of STING in pulmonary hypertension

“…Pulmonary hypertension is a major global health issue, with Group 3 PH prevalence on the rise affecting up to 60% of patients with chronic lung diseases 2,3 . Currently there are no disease-modifying therapies for PH patients.…”

“…Pulmonary hypertension (PH) is a devastating disease, affecting approximately 15-50 million people wor World Health Organization (WHO) Group 3 PH -PH secondary to chronic lung disease and/or hypoxia -is the leading cause of PH, with rising incidence globally and the highest mortality rate among all types of pulmonary v disease [2][3][4] . Group 3 PH patients, likewise, utilize the health care system up to four times higher than patients va 1 , Elnaz (STING), ons related cribed.…”

Pathogenesis, clinical features, and phenotypes of pulmonary hypertension associated with interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative ‐ Group 3 Pulmonary Hypertension

“…However, in idiopathic NSIP there is evidence that PH may be present in about one‐third of patients. 111 In patients with chronic hypersensitivity pneumonitis (chronic HP), precapillary PH may also be quite common (44%) 112 and similarly appears to be associated with a worse prognosis. 113 Furthermore, one study of chronic HP patients demonstrated that an increased PA/aorta ratio on CT is associated with worse survival.…”

Group 3 Pulmonary Hypertension: From Bench to Bedside

“…PH was reported in 31% of a cohort of 35 patients with idiopathic nonspecific interstitial pneumonitis and 44% of a cohort of 50 patients with chronic hypersensitivity pneumonitis. 126,127 More advanced parenchymal lung disease is associated with worse hemodynamics [128][129][130] and even echocardiographic PH 131 or minimal elevations in mPAP (>17 mm Hg) 132 are associated with reduced survival in patients with IPF. Interestingly, the degree of parenchymal lung involvement, based on PFTs 133 and high-resolution CT fibrosis score, 131,[134][135][136][137] does not correlate with PH severity.…”