ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1881

Discovery and Validation of Novel Disease Subsets in 806 Patients with Takayasu’s Arteritis across Four International Cohorts

Ruchika Goel1, Katherine B. Gribbons2, Kathleen Maksimowicz-McKinnon3, Gary S. Hoffman4, Sathish Kumar5, George Joseph6, Raheesh Ravindran7, Aswin Nair8, David Cuthbertson9, Simon Carette10, Nader A. Khalidi11, Curry L. Koening12, Carol Langford13, Carol A. McAlear14, Paul A. Monach15, Larry W. Moreland16, Christian Pagnoux17, Philip Seo18, Antoine G. Sreih19, Kenneth J. Warrington20, Steven R. Ytterberg20, Peter A. Merkel21, Debashish Danda22 and Peter C. Grayson2, 1Rheumatology, Christian Medical College, Vellore Tamilnadu, India, 2National Institute of Arthritis, Musculoskeletal and Skin Disease, National Institutes of Health, Bethesda, MD, 3Rheumatology, Henry Ford Hospital, Detroit, MI, 4Rheumatology, Cleveland Clinic, Cleveland, OH, 5Child Health, Christian Medical College, Vellore, India, 6Cardiology, Christian Medical College, Vellore, India, 7Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India, 8Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India, 9Biostatistics and Informatics, Department of Pediatrics, Department of Biostatistics and Informatics, Department of Pediatrics, University of South Florida, Tampa, FL, 10Division of Rheumatology, Mount Sinai Hospital, Toronto, ON, Canada, 11Rheumatology, McMaster University, Hamilton, ON, Canada, 12Rheumatology, Division of Rheumatology, University of Utah, Salt Lake City, UT, 13Rheumatic and Immunologic Diseases, Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, 14Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, 15Section of Rheumatology, Boston University School of Medicine, Boston, MA, 16Division of Rheumatology and Clinical Immunology, Division of Rheumatology, University of Pittsburgh, Pittsburgh, PA, 17Division of Rheumatology, Division of Rheumatology, Mount Sinai Hospital, Toronto, ON, Canada, 18Medicine, Division of Rheumatology, Johns Hopkins University, Baltimore, MD, 19Rheumatology, Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, 20Rheumatology, Division of Rheumatology, Mayo Clinic College of Medicine, Rochester, MN, 21University of Pennsylvania, Philadelphia, PA, 22Clinical Immunology & Rheumatology, Christian Medical College, Vellore, India, Vellore, India

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: , , ,

Session Information

Date: Monday, October 22, 2018

Title: 4M091 ACR Abstract: Vasculitis–Non-ANCA-Assocd & Rel D/Os I: Population-Based Studies(1881–1886)

Session Type: ACR Concurrent Abstract Session

Session Time: 2:30PM-4:00PM

Background/Purpose:

    Takayasu’s arteritis (TAK) is characterized by variable patterns of damage throughout the large arteries. This study aimed to develop and validate a novel disease classification system in TAK based on distribution of arterial lesions using data-driven methods.

    Methods:

    Data was used from patients with TAK from four independent cohorts: one in India and three in North America (NA). All patients underwent whole-body angiography of the aorta and branch vessels, with categorization of involvement (stenosis, occlusion, or aneurysm) in 13 arterial territories. K-means cluster analysis was performed to identify subgroups of patients based on pattern of angiographic involvement. Cluster groups were identified in the Indian cohort and independently validated in the NA cohorts. Recursive partitioning was used to develop a decision tree to predict cluster assignment.

    Results:

    581 and 225 patients with TAK were included from the Indian and NA cohorts, respectively. Three distinct clusters were identified in the Indian cohort and validated in the NA cohorts. Patients in Cluster 1 had significantly more disease in the abdominal aorta, renal, and mesenteric arteries (p < 0.01). Patients in Cluster 2 had significantly more bilateral disease in the carotid and subclavian arteries (p < 0.01). Compared to Clusters 1 and 2, patients in Cluster 3 had asymmetric disease with fewer involved territories (p < 0.01). When serial angiography was available for review, only 1 of 109 patients changed cluster assignment over time. There were more patients from India in Cluster 1 (41% vs 24%; p < 0.01) and more patients from NA in Cluster 3 (41% vs 32%; p = 0.03). Recursive partitioning predicted cluster assignment in the Indian cohort with 92% accuracy and cross-predicted cluster assignment of the NA cohorts with 87% accuracy based on involvement of the abdominal aorta, carotid, subclavian, mesenteric, and renal arteries. In the Indian and the NA cohorts, patients in Clusters 1 and 2 compared to Cluster 3 were more likely to have arterial occlusions (58% vs 82% vs 37%; p < 0.01) and a history of tuberculosis (8% vs 10% vs 3%; p = 0.03). Disease onset in childhood (28% vs 16% vs 19%; p < 0.01) and hypertension (71% vs 42% vs 39%; p < 0.01) were more common in Cluster 1. Stroke (0% vs 22% vs 5%; p = 0.03), vision loss (0% vs 33% vs 6%; p = 0.01), carotidynia (3% vs 26% vs 9%; p = 0.01) and persistent disease activity (46% vs 59% vs 44%; p = 0.02) were significantly more prevalent in Cluster 2.

    Conclusion:

    This large study in TAK identified and validated three novel subsets of patients based on patterns of arterial disease. The same subsets were seen in patients from India and NA; however, prevalence of patients within each subset differed by country. Angiographic-based disease classification may help identify causal disease factors and enable stratified clinical decision making in this complex, clinically heterogeneous disease.

    Disclosure: R. Goel, None; K. B. Gribbons, None; K. Maksimowicz-McKinnon, None; G. S. Hoffman, None; S. Kumar, None; G. Joseph, None; R. Ravindran, None; A. Nair, None; D. Cuthbertson, None; S. Carette, None; N. A. Khalidi, None; C. L. Koening, None; C. Langford, Bristol-Myers Squibb, 2,GlaxoSmithKline, 2,ChemoCentryx, 2,Genentech, Inc., 2,Bristol-Myers Squibb, 9,AbbVie Inc., 9; C. A. McAlear, None; P. A. Monach, None; L. W. Moreland, None; C. Pagnoux, None; P. Seo, None; A. G. Sreih, None; K. J. Warrington, GlaxoSmithKline, 2,Eli Lilly and Co., 2,Sanofi, 5; S. R. Ytterberg, None; P. A. Merkel, None; D. Danda, None; P. C. Grayson, None.

    To cite this abstract in AMA style:

    Goel R, Gribbons KB, Maksimowicz-McKinnon K, Hoffman GS, Kumar S, Joseph G, Ravindran R, Nair A, Cuthbertson D, Carette S, Khalidi NA, Koening CL, Langford C, McAlear CA, Monach PA, Moreland LW, Pagnoux C, Seo P, Sreih AG, Warrington KJ, Ytterberg SR, Merkel PA, Danda D, Grayson PC. Discovery and Validation of Novel Disease Subsets in 806 Patients with Takayasu’s Arteritis across Four International Cohorts [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/discovery-and-validation-of-novel-disease-subsets-in-806-patients-with-takayasus-arteritis-across-four-international-cohorts/. Accessed .

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