a Systematic Literature Review on the Treatment of Skin, Mucosa and Joint Involvement of BehçEt’s Syndrome Informing the Eular Recommendations for the Management of BehçEt’s Syndrome

Pietro Leccese¹, Yesim Ozguler², Robin Christensen³, Sinem Nihal Esatoglu⁴, Dongsik Bang⁵, Bahram Bodaghi⁶, Aykut Ferhat Celik⁷, Farida Fortune⁸, Julien Gaudric⁹, Ahmet Gul¹⁰, Ina Kotter¹¹, Alfred Mahr¹², Robert J Moots¹³, Ignazio Olivieri¹⁴, Jutta Richter¹⁵, David Saadoun¹⁶, Carlo Salvarani¹⁷, Frances Scuderi¹⁸, PETROS P SFIKAKIS¹⁹, Aksel Siva²⁰, Miles Stanford²¹, Ilknur Tugal-tutkun²², Richard West²³, Sebahattin Yurdakul², Hasan Yazici² and Gulen Hatemi², ¹Rheumatology, Rheumatology Department of Lucania, San Carlo Hospital of Potenza and Madonna delle Grazie Hospital of Matera, Matera, Italy, Matera, Italy, ²Istanbul University, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, ³The Parker instutute, RC, Copenhagen, Denmark, ⁴Rheumatology, Istanbul University, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, ⁵Department of Dermatology, Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea, Seoul, Korea, The Democratic People's Republic of, ⁶Ophthalmology, Pierre and Marie Curie University - Paris 6 Paris, France, Paris, France, ⁷Istanbul University, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Gastroenterology, Istanbul, Turkey, ⁸Dental Institute, Barts and The London NHS Trust, London, United Kingdom, ⁹Department of Vascular surgery GHPS, Paris, France, ¹⁰Department of Internal Medicine, Division of Rheumatology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey, ¹¹Internal Medicine, Robert-Bosch-Hospital, Stuttgart, Germany, ¹²Hospital Saint-Louis, Paris, France, ¹³Academic Rheumatology Unit, University of Liverpool, Liverpool, United Kingdom, ¹⁴U.O. Reumatologia, A.O. Ospedale San Carlo, Potenza, Italy, ¹⁵Tubingen University, Tubingen, Germany, ¹⁶Department of Internal Medicine and Clinical Immunology, AP-HP Pitié-Salpêtrière Hospital, Paris, France, ¹⁷Rheumatology Unit, Arcispedale S Maria Nuova, IRCCS, Reggio Emilia, Italy, ¹⁸Patient partner, Catania, Italy, ¹⁹First Department of Propedeutic Internal Medicine, Laikon Hospital, Athens University Medical School, Athens, Greece, ²⁰Department of Neurology, Istanbul University, Cerrahpasa Medical Faculty, Department of Neurology, Istanbul, Turkey, ²¹Ophthalmology, Guy's and St. Thomas' Hospital, London, United Kingdom, ²²Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey, ²³Patient partner, London, United Kingdom

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: anti-TNF therapy and arthritis, Behcet's syndrome

Session Information

Date: Tuesday, November 15, 2016

Title: Vasculitis - Poster III: Rarer Vasculitides

Session Type: ACR Poster Session C

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

A Systematic Literature Review on the Treatment of Skin, Mucosa and Joint Involvement of Behçet’s Syndrome Informing the Eular Recommendations for the Management of Behçet’s Syndrome

Background/Purpose: The aim of this systematic literature review was to inform the task force for updating the European League Against Rheumatism recommendations for the management of Behçet’s Syndrome (BS), about the evidence for treatment of skin, mucosa and joint involvement of BS.

Methods: A systematic literature search, data extraction and statistical analyses according to pre-specified eligibility criteria were performed using the GRADE approach. The protocol for the systematic literature review was registered at PROSPERO (CRD42015027033). The Cochrane Library, including the Cochrane Central Register of Controlled Trials (CENTRAL), Cochrane Database of Systematic Reviews (CDSR), Database of Abstracts of Reviews of Effects (DARE), Health Technology Assessments (HTA), MEDLINE (from 1950), EMBASE (from 1980) and International Pharmaceutical Abstracts Database (IPAD) were systematically searched. Randomised controlled trials (RCT), non-randomised controlled clinical trials and open label trials (OLT) on BS comparing an active intervention (alone or in combination) with any control or placebo were eligible. If controlled trials were not available for answering a specific research question, uncontrolled evidence from cohort studies or case series involving ≥ 5 patients were considered. The quality of evidence was assessed by using the GRADE approach. Risk ratios were calculated for the binary outcomes whereas for the continuous outcomes we calculated the standardized mean difference (SMD).

Results: Among the 3927 references that we have screened, 22 studies satisfied the inclusion criteria for mucocutaneous involvement and 15 studies for joint involvement. Seventeen of these studies were randomized controlled trials assessing mucocutaneous and/or joint involvement. RCTs with colchicine, azathioprine, interferon-alpha, thalidomide, etanercept and apremilast showed different levels of beneficial results on different types of skin and mucosa lesions and arthritis. Differences in the outcome measures that were used across the included studies made it difficult to compare the results. These agents were generally well tolerated with few adverse events causing withdrawal from the study in BS patients.

Conclusion: It was gratifying to see that randomized controlled trials formed the majority (17/22, 77%) of the sources forming the basis for the recommendations related to skin mucosa and joint involvement in the updated EULAR Recommendations for the management of BS.

Disclosure: P. Leccese, None; Y. Ozguler, None; R. Christensen, None; S. N. Esatoglu, None; D. Bang, None; B. Bodaghi, None; A. F. Celik, None; F. Fortune, None; J. Gaudric, None; A. Gul, None; I. Kotter, None; A. Mahr, None; R. J. Moots, UCB Pharma, Novartis, Pfizer, 2,Chugai, Cellgene, Novartis, Pfizer, Roche, 5; I. Olivieri, None; J. Richter, None; D. Saadoun, None; C. Salvarani, None; F. Scuderi, None; P. P. SFIKAKIS, None; A. Siva, None; M. Stanford, None; I. Tugal-tutkun, None; R. West, None; S. Yurdakul, None; H. Yazici, None; G. Hatemi, None.

To cite this abstract in AMA style:

Leccese P, Ozguler Y, Christensen R, Esatoglu SN, Bang D, Bodaghi B, Celik AF, Fortune F, Gaudric J, Gul A, Kotter I, Mahr A, Moots RJ, Olivieri I, Richter J, Saadoun D, Salvarani C, Scuderi F, SFIKAKIS PP, Siva A, Stanford M, Tugal-tutkun I, West R, Yurdakul S, Yazici H, Hatemi G. a Systematic Literature Review on the Treatment of Skin, Mucosa and Joint Involvement of BehçEt’s Syndrome Informing the Eular Recommendations for the Management of BehçEt’s Syndrome [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/a-systematic-literature-review-on-the-treatment-of-skin-mucosa-and-joint-involvement-of-behcets-syndrome-informing-the-eular-recommendations-for-the-management-of-behcets-syndrome/. Accessed .

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