Janet Pope, Canada

St. Joseph's Health Care Rheumatology
Dr. Janet Pope is a Professor of Medicine and head of the Division of Rheumatology at the University of Western Ontario (UWO), Schulich School of Medicine, London, Ontario, Canada. She has published over 500 peer-reviewed articles and mentored more than 125 trainees. Research expertise includes scleroderma, SLE and RA. She has been inducted into the Canadian Academy of Health Sciences.

Presenter of 1 Presentation

 Conference Calendar

A SYSTEMIC REVIEW OF FACTORS ASSOCIATED WITH PULMONARY ARTERIAL HYPERTENSION (PAH) IN SYSTEMIC SCLEROSIS (SSC)

Session Name
PS44 - THE HEART AND LUNG IN AUTOIMMUNE CONDITIONS
Session Type
PARALLEL SESSIONS
Date
31.05.2021, Monday
Session Time
10:00 - 12:00
Room
HALL E
Lecture Time
11:00 - 11:10
Presenter
Session Icon
Pre Recorded

Abstract

Background and Aims

Pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) is a lethal complication affecting 8-15% of patients. Screening tests such as echocardiography and pulmonary function tests allow for triaging patients for diagnosis by right heart catheterization. Understanding risk factors of SSc-PAH could help differentiate high-risk patients. The aims were to determine risk factors of PAH in SSc.

Methods

A systematic review was conducted to determine associations with SSc-PAH, including clinical/disease characteristics, antibodies, labs and biomarkers. The frequencies of publications featuring each risk/association were reported.

Results

Among 2654 articles, 984 duplicates and 1578 irrelevant articles were removed, leaving 92 articles for manual screening. After excluding 55 papers with small sample sizes, publications from identical cohorts, not English language, or PAH not ascertained by RHC, 37 articles were eligible. A total of 43 factors for SSc-PAH were identified within seven categories. Several associations were due to PAH and risk factors such as dynpnea, right heart failure, and short 6-minute walk distance. Patient characteristics (14), pulmonary physiology (6), antibody profiles (6) and genetics/epigenetics (6) had the most numerous and diverse factors, while biomarkers (4) and other labs (2) features were infrequent. Low carbon monoxide (DLCO) (6), older age (4), longer disease duration (4), positive anticentromere antibodies (ACA) (4), telangiectasias (4), high brain natriuretic peptide (4) were frequent associations.

Conclusions

Risk factors for SSc-PAH such as ACA, older age, longer disease duration limited cutaneous SSc subset and presence of ILD may enrich screening programs. Genes and other antibody profiles are inconsistent and requires further validation.

Hide