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A Retrospective Comparison of Pulmonary Arterial Hypertension (PAH) Patients with Myositis-Related Autoantibodies to Patients with Idiopathic PAH and PAH Associated with Systemic Sclerosis

S Si

Sophia Weinmann

VN Despotovic

C McEvoy

Murali Chakinala


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Conference: 2018 PHA International PH Conference & Scientific Sessions

Release Date: 07.28.2018

Presentation Type: Abstracts

File Download: Conference 2018_1014

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2018 International PH Conference and Scientific SeAbstract presented at the 2018 International PH Conference and Scientific Sessions in Orlando, Fla., June 28-July 1, 2018.


Pulmonary arterial hypertension (PAH) is a heterogeneous disorder that can arise from a number of causes. Distinct forms of PAH, most notably PAH related to connective tissue diseases or systemic sclerosis (SSc-PAH), have differing characteristics and are associated with worse outcomes relative to idiopathic PAH (IPAH). We are not aware of studies that have specifically investigated a group of patients with myositis autoantibodies and PAH (MA-PAH) in comparison to other cohorts of PAH patients.


We carried out a single-center, retrospective cohort study to compare the phenotypic, laboratory, functional, hemodynamics, and outcomes of patients with myositis autoantibodies and PAH to IPAH and SSc-PAH patients. Individuals with PAH confirmed by cardiac catheterization were eligible for inclusion into the study, and patients were enrolled from August 2012 to January 2017. MyoMarker Panel 3 (RDL Reference Laboratories, Inc.) assay was used for myositis autoantibody testing. Patient characteristics were compared between groups using the Chi-square test and one-way ANOVA with Tukey post hoc test. Survival analysis was performed utilizing the Kaplan-Meier method and the log-rank test.


37 MA-PAH patients were compared to 47 IPAH and 19 SSc-PAH patients. The median age of the subjects was 61 years (range 30 to 80 years) and 81 (79%) patients were female. MA-PAH patients did not differ with respect to any tested characteristic when compared to SSc-PAH patients. Of note, subjects with IPAH were significantly younger, had superior exercise capacity, and demonstrated better lung function relative to both MA-PAH patients and SSc-PAH patients. Hemodynamically, IPAH patients had more elevated mean pulmonary artery pressures (mPAP) and pulmonary vascular resistances (PVR) in comparison to MA-PAH patients and SSc-PAH patients. There were no significant differences in PAH-specific therapies between groups. Estimated survival from enrollment for the overall cohort was 94%, 89%, and 81% at one year, two years, and four years, respectively, with no differences in mortality between any of our three cohorts.


Our results demonstrate that MA-PAH patients are clinically similar to SSc-PAH patients and significantly dissimilar to IPAH patients. Differences in response to treatment and long-term outcomes for this novel sub-group of PAH patients needs further investigation.


Table 1. Baseline Clinical, Functional, and Hemodynamic Characteristics
Chi-square test or one-way ANOVA with Tukey post hoc test. *P<.05, **P<.01, ***P<.001, ****P<.0001.