Background/Purpose: Large vessel vasculitis (LVV) comprises a spectrum of rare, potentially life-threatening disorders, including giant cell arteritis (GCA) and Takayasu’s arteritis (TAK), defined by granulomatous inflammation and destruction of the aorta and its primary branching arteries. GCA and TAK present with critical arterial stenosis, occlusion, or aneurysmal dilatation that may result in end-organ ischemia and infarction. Concomitant LVV with SSc is exceedingly rare yet carries high morbidity risk. Few cases describe pulmonary arterial hypertension (PAH) in these cases of overlap. In this study, we examine whether LVV is associated with the development of SSc. Then, we examine whether the presence of PAH in patients with LVV is associated with the development of SSc and anticentromere antibodies (ACA) and anti-DNA topoisomerase I antibodies (Scl-70). 

Methods: This retrospective analysis utilized real-world dynamic data from TriNetX clinical research platform, representing over 124 million patients in the Research network. We identified 62,027 patients with LVV and 124,255,508 patients without as controls. SSc prevalence and relative risk with 95% confidence interval were assessed between groups. Among patients with LVV, we identified 1,042 patients with PAH and 56,113 without as controls and assessed SSc, ACA, and Scl-70 prevalence and relative risk with 95% confidence interval. Chi-square test was used to compare SSc prevalence between groups and significance was defined as p-value < 0.05. Participants were identified based on ICD-10-CM diagnosis codes.

Results: Demographics of LVV cohort included: mean age 76, SD 15, female 67.54%, male 28.83%, unknown gender 3.63%. Demographics of controls without LVV cohort included: mean age 46, SD 25, female 52.31%, male 45.37%, unknown gender 2.32%. 
 
Patients with LVV showed SSc prevalence of 564.3/10,000 patients compared to in controls 0.0.042/10,000 patients (p-value < 0.0001). 52(0.050) of 1,042 patients with LVV and PAH had SSc compared to 194(0.003) of 56,113 in the control group(p-value < 0.0001). Among patients with LVV and PAH, 14(0.013) had positive ACA compared to 249(0.004) in the control group (p-value < 0.0001), while 15(0.014) had Scl-70 compared to 279(0.005) in the controls (p-value < 0.0001). Association between LVV and SSc in patients with PAH was 14 times higher compared to patients without (RR: 14.7; 95% CI: 10.9 – 19.8), while the association between LVV and SSc-associated antibodies was 3 times higher in patients with PAH for ACA (RR: 3; 95% CI: 1.8 – 5.2) and Scl-70 (RR: 2.9; 95% CI: 1.8 – 4.9), respectively.

Conclusion: Patients with LVV have a statistically significant association with the development of SSc. The presence of PAH in patients with LVV has statistically significant associations with the development of SSc, ACA and Scl-70. LVV and SSc in LVV portend worse prognosis with higher mortality risk compared to those without overlap. Prompt recognition and management of this unusual clinical overlap is crucial given the potential for life-threatening complications. Monitoring with transthoracic echocardiogram for the vascular manifestation of PAH in patients with LVV may be an effective screening tool for SSc, ACA and Scl-70.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/association-of-large-vessel-vasculitis-and-development-of-ssc-and-ssc-associated-antibodies-impact-of-pulmonary-arterial-hypertension/