ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1702

Target Organ Associations in Polyarteritis Nodosa (PAN): Results of a Worldwide Collaboration Study

Omer Karadag1, Shunsuke Furuta 2, Alojzija Hocevar 3, Ummugulsum Gazel 4, Andrea Hinojosa-Azaola 5, Serdal Ugurlu 6, Seerapani Gopaluni 7, Ertugrul Cagri Bolek 8, Berkan Armagan 9, Fatma Alibaz-Oner 10, Matija Tomšič 11, Gizem Ayan 12, Ayten Yazici 13, Eduardo Martin-Nares 14, Susy Marcela Sánchez-Cubías 5, Mehmet Nedim Tas 15, Ayşe Cefle 16, Kenan Aksu 17, Hajime Kono 18, Tamihiro Kawakami 19, Yoshiyuki Abe 20, Timuçin Kaşifoğlu 21, Mehmet Engin Tezcan 22, Onay Gercik 23, Sadettin Kilickap 24, Haner Direskeneli 25, Seza Ozen 26, Ali İhsan Ertenli 8 and David Jayne 7, 1Hacettepe University Vasculitis Centre, Ankara, Turkey / Vasculitis and Lupus Clinic, Addenbrooke’s Hospital, University of Cambridge, UK, Ankara, Turkey, 2Department of Alergy and Immunology, Chiba University Hospital, Chiba, Japan, Chiba, Japan, 3Department of Rheumatology, University Medical Center Ljubljana, Slovenia, LJUBLJANA, Slovenia, 4Marmara University, Faculty of Medicine, Department of Rheumatology, Istanbul, Turkey, Istanbul, Turkey, 5Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Mexico City, Mexico, 6Istanbul University - Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, istanbul, Turkey, 7Vasculitis and Lupus Clinic, Addenbrooke’s Hospital, University of Cambridge, UK, Cambridge, United Kingdom, 8Hacettepe University Vasculitis Centre, Ankara, Turkey, ankara, Turkey, 9Department of Internal Medicine, Hacettepe University, Faculty of Medicine, Ankara, Turkey., Ankara, Turkey, 10Marmara University Faculty of Medicine,Department of Rheumatology,Istanbul,Turkey, Istanbul, Turkey, 11Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, Slovenia, LJUBLJANA, Slovenia, 12University of Ottawa Faculty of Medicine, Rheumatology, Ottawa, ON, Canada, 13Kocaeli University, Faculty of Medicine, Division of Rheumatology, Kocaeli, Kocaeli, Turkey, 14Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, 15Ege University, Faculty of Medicine, Department of Rheumatology, Izmir, Turkey, Izmir, Turkey, 16Division of Rheumatology, Kocaeli University School of Medicine, Kocaeli, Turkey, Kocaeli, Turkey, 17Ege University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İzmir, Turkey, 18Teikyo University School of Medicine, Tokyo, Japan, 19Department of Dermatology, Tohoku Medical and Pharmaceutical University, Japan, Tohoku, Japan, 20Department of Rheumatology, Juntendo University, Tokyo, Japan, Tokyo, Japan, 21Eskişehir Osmangazi University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Eskişehir, Turkey, 22Department of Rheumatology, Kartal Dr. Lutfi Kirdar Training and Research Hospital , Istanbul, Turkey, Istanbul, Turkey, 23Izmir Katip Celebi University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İzmir, Turkey, 24Hacettepe University, Faculty of Medicine, Department of Preventive Oncology, Ankara, Turkey, Ankara, Turkey, 25Marmara University, School of Medicine, Division of Rheumatology, Istanbul, Turkey, 26Hacettepe University Hospital, Ankara, Turkey

Meeting: 2019 ACR/ARP Annual Meeting

Keywords:

Session Information

Date: Monday, November 11, 2019

Title: Vasculitis – Non-ANCA-Associated & Related Disorders Poster III: Behçet’s Disease & Other Vasculitides

Session Type: Poster Session (Monday)

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

Background/Purpose: Polyarteritis nodosa (PAN) is a rare type of the systemic vasculitides with heterogeneous presentations.There is a paucity of information on the current phenotypes, ethnic and geographic differences of PAN. A global PAN study group has been working for clinical subphenotypes and GWAS studies.

Methods: A retrospective survey of databases from centres was done for PAN patients fulfilling the EMEA Vasculitis Classification algorithm. Patients with typical angiographic and/or histopathologic findings consistent with PAN were enrolled. In addition to baseline clinical characteristics, treatment and outcome data was recorded.

Factor analysis was used to analyze target organ associations of these 229 patients. Four factors were identified by factor analysis of variables sex, ethnicity, disease onset age, pediatric-onset, monogenic disease relationship, HBV relationship, any cutaneous features, any musculoskeletal symptoms, any constitutional symptoms, any other involved areas (abdominal, renal, testicular, neurologic, ear nose and throat (ENT), cardiac, pulmonary), positive angiographic findings and relapses.

Results: PAN Cohorts from 5 different countries were investigated in this study. (Japan: n=39, Mexico: n=29, Slovenia: n=14, TUR: n=100, UK: n=47). Totally 229 (M/F: 130/99 and Caucasian 70.3%, Asian 17.0%, and Hispanic 12.7%) patients were included in the study. Four were HBV-related, and 21 of TR patients had a monogenic form of disease (Familial Mediterranean Fever association in 14, deficiency of adenosine deaminase 2-DADA2- in 7).

20.5% of patients were cutaneous-only PAN patients. 45.4% of patients had radiologic, and 69.4% had biopsy-proven PAN.  Median age at disease onset was 38.5 (IQR 24.0-57.0) years. During a median 60 (16-130) months follow-up, 23 patients died.

Factor analysis revealed 4 factors that explained 60% of the original information on the matrix as follows: Factor 1,represented the association between any of gastrointestinal findings, renal involvement, and testicular involvement which are negatively associated with any of cutaneous involvement; Factor 2,the association between any of musculoskeletal findings, and any positive constitutional symptoms; Factor 3,any neurologic involvement was associated with ENT involvement, Factor 4,any monogenic relationship FMF or ADA itself. The eigenvalues of the 4 factors were 1.853, 1.297, 1.164, and 1.085, in decreasing order, i.e., the highest contribution to the overall variance in the matrix came from the togetherness of the 3 clinical manifestations that made up Factor 1. Factors, their relative contributions to the total variance and the communalities for each of the variables are shown in Table.

Conclusion: The target organ associations found in this study could support the hypothesis of subphenotypes in PAN. Factor 1-more organ involvement with less cutaneous features seems to be the most severe form. Patients with any monogenic genetic background (FMF or DADA2) have distinct target organ associations. Even though ENT involvement was rare, its association with neurologic involvement might define a different subphenotype- looks like a ANCA negative medium vessel vasculitis.

Table: Target organ associations in Polyarteritis Nodosa -PAN-

Disclosure: O. Karadag, None; S. Furuta, None; A. Hocevar, None; U. Gazel, None; A. Hinojosa-Azaola, None; S. Ugurlu, None; S. Gopaluni, None; E. Bolek, None; B. Armagan, None; F. Alibaz-Oner, None; M. Tomšič, None; G. Ayan, None; A. Yazici, None; E. Martin-Nares, None; S. Sánchez-Cubías, None; M. Nedim Tas, None; A. Cefle, None; K. Aksu, None; H. Kono, Celgene Corporation, 2; T. Kawakami, None; Y. Abe, None; T. Kaşifoğlu, None; M. Tezcan, None; O. Gercik, None; S. Kilickap, None; H. Direskeneli, None; S. Ozen, Enzyvant, 8; A. Ertenli, None; D. Jayne, Astra Zeneca, 5, Boehringer-Ingelheim, 5, Celgene, 5, ChemoCentryx, 2, 5, GSK, 2, 5, Infla-Rx, 5, InflaRx GmbH, 5, Insmed, 5, Roche Genetech, 2, Sanofi Genzyme, 2, Takeda, 5.

To cite this abstract in AMA style:

Karadag O, Furuta S, Hocevar A, Gazel U, Hinojosa-Azaola A, Ugurlu S, Gopaluni S, Bolek E, Armagan B, Alibaz-Oner F, Tomšič M, Ayan G, Yazici A, Martin-Nares E, Sánchez-Cubías S, Nedim Tas M, Cefle A, Aksu K, Kono H, Kawakami T, Abe Y, Kaşifoğlu T, Tezcan M, Gercik O, Kilickap S, Direskeneli H, Ozen S, Ertenli A, Jayne D. Target Organ Associations in Polyarteritis Nodosa (PAN): Results of a Worldwide Collaboration Study [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/target-organ-associations-in-polyarteritis-nodosa-pan-results-of-a-worldwide-collaboration-study/. Accessed .

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