A Systemic Review of Factors Associated with Systemic Sclerosis-associated Pulmonary Arterial Hypertension (SSc-PAH)

Yuxuan Jiang ¹, Matthew Turk ¹ and Janet Pope², ¹University of Western Ontario, London, ON, Canada, ²Western University, London, ON, Canada

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: Systemic sclerosis

Session Information

Date: Sunday, November 10, 2019

Title: Systemic Sclerosis & Related Disorders – Clinical Poster I

Session Type: Poster Session (Sunday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) is a lethal complication affecting approximately 8-15% of patients. Screening methods including echocardiography and pulmonary function tests exist to triage patients for definitive diagnosis by right heart catheterization. Improving our understanding of SSc-PAH associated risk factors could help stratify high-risk patients for regular screening.

Methods: A systematic review was performed in SCOPUS, Medline, EMBASE, Web of Science, and the Cochrane Library from their inception to February 22, 2019. Terms included phrases related to pulmonary arterial hypertension, systemic sclerosis, and prevalence. Studies were included if they reported on the frequency of a risk/association factor related to SSc-PAH. Studies were included if they determined PAH with right heart catheterization, compared SSc patients with and without PAH, and had sample size larger than 20.

Results: The search found 2654 articles of which, 984 were duplicates and 1578 were excluded due to irrelevant title. After the remaining 92 were screened, 37 articles met eligibility criteria. Forty-three risk/association factors for SSc-PAH were identified and placed into seven categories. The most frequently mentioned categories included: patient characteristics, pulmonary physiology, antibody profiles, and genetics/epigenetics factors. In contrast, biomarkers and other labs featured the fewest distinct risk factors. Specific risk factors found for patients were lowered diffusing capacity of the lungs for carbon monoxide, older age, and longer disease duration among others. Interstitial lung disease and anti-centromere antibody (ACA) were also frequently associated (Figure 1 and 2).

Conclusion: Presence of ILD, ACA, older age, and disease duration are consistently identified risk factors in PAH-SSc. Risk factors for SSc-PAH such as limited-SSc, ACA, older age, longer disease duration and presence of ILD may enrich screening programs. Patterns in genotypes and antibody profiles are inconsistent and requires further validation. Understanding these risk factors may give insight into which patients will need further screening but some risks are associated with Class I PAH (ex. ACA) and others with Class III (ILD, hypoxia) which may be why risk factors are inconsistent in the literature.

Figure 1. Pulmonary physiology measurements related to literature reported SSc-PAH

Fig.2 All clinical parameters associated with SSc_PAH as reported in the literature

Disclosure: Y. Jiang, None; M. Turk, None; J. Pope, AbbVie, 5, Abbvie, 5, Actelion, 5, Actellion, 5, Amgen, 2, 5, AstraZeneca, 2, Astra-Zeneca, 2, Bayer, 2, 5, BMS, 2, 5, Eicos Sciences, 5, Eli Lilly & Company, 5, Eli Lilly and Company, 5, EMERALD, 5, Emerald, 5, Genzyme, 5, Janssen, 5, Lilly, 5, Merck, 2, 5, Novartis, 5, Pfizer, 2, 5, Roche, 2, 5, Sandoz, 5, Sanofi, 5, Seattle Genetics, 2, UCB, 2, 5, 8.

To cite this abstract in AMA style:

Jiang Y, Turk M, Pope J. A Systemic Review of Factors Associated with Systemic Sclerosis-associated Pulmonary Arterial Hypertension (SSc-PAH) [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/a-systemic-review-of-factors-associated-with-systemic-sclerosis-associated-pulmonary-arterial-hypertension-ssc-pah/. Accessed .

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