Session Information
Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud’s - Clinical Aspects and Therapeutics II
Session Type: Abstract Submissions (ACR)
Background/Purpose: Pulmonary arterial hypertension (PAH) is an intractable complication of connective tissue disease (CTD). Current guidelines recommend early detection and intervention for improvement of outcomes. To achieve this goal, it is imperative to identify subgroups with high risk for developing PAH. Patients with systemic sclerosis (SSc) are known to have the highest risk for PAH. Serum autoantibodies, such as anticentromere, anti-U1RNP, anti-U3 RNP, and anti-Th/To, were also associated with PAH in certain CTD subgroups. However, little information is available for utility of autoantibody profiles for predicting PAH risk in patients with CTD. Here, we examined a role of autoantibody testing in identifying patients with PAH risk using a large-scale database of a routine autoantibody laboratory.
Methods: This study enrolled 6,162 patients, whose sera were sent to our autoantibody laboratory between 1995 and 2008 because of clinical suspicion of CTD. They were selected from 9,872 consecutive patients based on observation period >3 years, and availability of full clinical information on medical charts. Indirect immunofluorescence, double immunodiffusion, and RNA immunoprecipitation assay were routinely conducted to identify autoantibodies to centromere, topoisomerase I, Sm, U1RNP, SSA, SSB, Th/To, U3RNP, SRP, aminoacyl tRNA synthetase, and ribosome.
Results: During 6.6 ± 5.6 years of follow-up, 71 patients (1.2%) were diagnosed as having PAH confirmed by right heart catheterization. Mixed connective tissue disease (MCTD), SSc, and systemic lupus erythematosus (SLE) were clinical diagnosis associated with PAH (unadjusted odds ratio [OR] 10.8, 8.4, and 2.0, respectively). PAH also occurred in a small population of patients with primary Sjögren’s syndrome (n = 7; 0.8%), rheumatoid arthritis (n = 3; 0.2%), or dermatomyositis (n = 1; 0.3%). Autoantibodies associated with PAH included those to centromere, U1RNP, Sm, SSA, SSB, and Th/To (unadjusted OR 4.5-6.6). Female gender and Raynaud’s phenomenon were also identified as PAH risk factors (unadjusted OR 8.0 and 10.5, respectively). Multivariate logistic regression analysis revealed that MCTD, SSc, and SLE were independent PAH risk, indicating that the overall PAH risk could be explained primarily by clinical diagnosis. Interestingly, anti-SSA antibody without diagnosis of MCTD, SSc, or SLE was another independent PAH risk, indicating limited utility of the autoantibody status. When we further developed a multivariate logistic regression model by combining the diagnosis and autoantibody profile, PAH risk can be explained by 6 independent variables, including SSc with anticentromere (OR 578, 95% confidence interval [CI] 119-10409), MCTD (OR 397, 95% CI 81-7174), SLE with anti-U1RNP (OR 150, 95% CI 29-2743), SSc without anticentromere (OR 103, 95% CI 18-1923), anti-SSA without diagnosis of MCTD, SSc, or SLE (OR 56, 95% CI 11-1028), and SLE without anti-U1RNP (OR 42, 95% CI 6.8-813).
Conclusion: A combination of clinical diagnosis and autoantibody profiles effectively stratifies PAH risk in patients suspected to have CTD, and may aid in selection of patients who benefit from active screening program for PAH detection.
Disclosure:
M. Kuwana,
None;
Y. Shirai,
None;
H. Yasuoka,
None;
T. Takeuchi,
None;
K. Masui,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/utility-of-autoantibody-testing-for-predicting-risk-of-pulmonary-arterial-hypertension-a-retrospective-analysis-in-routine-autoantibody-laboratory/