Management of major organ involvement of Behçet's syndrome

a systematic review for update of the EULAR recommendations

Yesim Ozguler, Pietro Leccese, Robin Christensen, Sinem Nihal Esatoglu, Dongsik Bang, Bahram Bodaghi, Aykut Ferhat Çelik, Farida Fortune, Julien Gaudric, Ahmet Gul, Ina Kötter, Alfred Mahr, Robert J Moots, Jutta Richter, David Saadoun, Carlo Salvarani, Francesco Scuderi, Petros P Sfikakis, Aksel Siva, Miles Stanford & 6 others Ilknur Tugal-Tutkun, Richard West, Sebahattin Yurdakul, Ignazio Olivieri, Hasan Yazici, Gulen Hatemi

Research output: Contribution to journalReviewResearchpeer-review

Abstract

Objective: To assess the efficacy and safety of treatment modalities for major organ involvement of Behçet's syndrome (BS), in order to inform the update of the EULAR recommendations for the management of BS.

Methods: A systematic literature review of all randomized controlled trials, controlled clinical trials, or open label trials assessing eye, vascular, nervous system or gastrointestinal system involvement of BS was performed. If controlled trials were not available for answering a specific research question, uncontrolled studies or case series were also included.

Results: We reviewed the titles and abstracts of 3927 references and 161 studies met our inclusion criteria. There were only nine randomized controlled trials. Observational studies with IFN-α and monoclonal anti-TNF antibodies showed beneficial results for refractory uveitis. Meta-analysis of case-control studies showed that immunosuppressives decreased the recurrence rate of deep vein thrombosis significantly whereas anticoagulants did not. CYC and high dose glucocorticoids decreased mortality in pulmonary arterial aneurysms and postoperative complications in peripheral artery aneurysms. Beneficial results for gastrointestinal involvement were obtained with 5-ASA derivatives and AZA as first line treatment and with thalidomide and/or monoclonal anti-TNF antibodies in refractory cases. Observational studies for nervous system involvement showed improved outcome with immunosuppressives and glucocorticoids. Meta-analysis of case-control studies showed an increased risk of developing nervous system involvement with ciclosporin-A.

Conclusion: The majority of studies related to major organ involvement that informed the updated EULAR recommendations for the management of BS were observational studies.

Original languageEnglish
JournalRheumatology (Oxford, England)
Volume57
Issue number12
Pages (from-to)2200–2212
ISSN1462-0324
DOIs
Publication statusPublished - 13. Aug 2018

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Nervous System
Immunosuppressive Agents
Glucocorticoids
Meta-Analysis
Case-Control Studies
Randomized Controlled Trials
Uveitis
Controlled Clinical Trials
Cyclosporine
Safety
Lung
Research

Keywords

  • Behçet’s syndrome
  • Eye involvement
  • Gastrointestinal involvement
  • Nervous system involvement
  • Treatment
  • Uveitis
  • Vascular involvement

Cite this

Ozguler, Yesim ; Leccese, Pietro ; Christensen, Robin ; Esatoglu, Sinem Nihal ; Bang, Dongsik ; Bodaghi, Bahram ; Çelik, Aykut Ferhat ; Fortune, Farida ; Gaudric, Julien ; Gul, Ahmet ; Kötter, Ina ; Mahr, Alfred ; Moots, Robert J ; Richter, Jutta ; Saadoun, David ; Salvarani, Carlo ; Scuderi, Francesco ; Sfikakis, Petros P ; Siva, Aksel ; Stanford, Miles ; Tugal-Tutkun, Ilknur ; West, Richard ; Yurdakul, Sebahattin ; Olivieri, Ignazio ; Yazici, Hasan ; Hatemi, Gulen. / Management of major organ involvement of Behçet's syndrome : a systematic review for update of the EULAR recommendations. In: Rheumatology (Oxford, England). 2018 ; Vol. 57, No. 12. pp. 2200–2212.
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title = "Management of major organ involvement of Beh{\cc}et's syndrome: a systematic review for update of the EULAR recommendations",
abstract = "Objective: To assess the efficacy and safety of treatment modalities for major organ involvement of Beh{\cc}et's syndrome (BS), in order to inform the update of the EULAR recommendations for the management of BS.Methods: A systematic literature review of all randomized controlled trials, controlled clinical trials, or open label trials assessing eye, vascular, nervous system or gastrointestinal system involvement of BS was performed. If controlled trials were not available for answering a specific research question, uncontrolled studies or case series were also included.Results: We reviewed the titles and abstracts of 3927 references and 161 studies met our inclusion criteria. There were only nine randomized controlled trials. Observational studies with IFN-α and monoclonal anti-TNF antibodies showed beneficial results for refractory uveitis. Meta-analysis of case-control studies showed that immunosuppressives decreased the recurrence rate of deep vein thrombosis significantly whereas anticoagulants did not. CYC and high dose glucocorticoids decreased mortality in pulmonary arterial aneurysms and postoperative complications in peripheral artery aneurysms. Beneficial results for gastrointestinal involvement were obtained with 5-ASA derivatives and AZA as first line treatment and with thalidomide and/or monoclonal anti-TNF antibodies in refractory cases. Observational studies for nervous system involvement showed improved outcome with immunosuppressives and glucocorticoids. Meta-analysis of case-control studies showed an increased risk of developing nervous system involvement with ciclosporin-A.Conclusion: The majority of studies related to major organ involvement that informed the updated EULAR recommendations for the management of BS were observational studies.",
keywords = "Beh{\cc}et’s syndrome, Eye involvement, Gastrointestinal involvement, Nervous system involvement, Treatment, Uveitis, Vascular involvement",
author = "Yesim Ozguler and Pietro Leccese and Robin Christensen and Esatoglu, {Sinem Nihal} and Dongsik Bang and Bahram Bodaghi and {\cC}elik, {Aykut Ferhat} and Farida Fortune and Julien Gaudric and Ahmet Gul and Ina K{\"o}tter and Alfred Mahr and Moots, {Robert J} and Jutta Richter and David Saadoun and Carlo Salvarani and Francesco Scuderi and Sfikakis, {Petros P} and Aksel Siva and Miles Stanford and Ilknur Tugal-Tutkun and Richard West and Sebahattin Yurdakul and Ignazio Olivieri and Hasan Yazici and Gulen Hatemi",
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Ozguler, Y, Leccese, P, Christensen, R, Esatoglu, SN, Bang, D, Bodaghi, B, Çelik, AF, Fortune, F, Gaudric, J, Gul, A, Kötter, I, Mahr, A, Moots, RJ, Richter, J, Saadoun, D, Salvarani, C, Scuderi, F, Sfikakis, PP, Siva, A, Stanford, M, Tugal-Tutkun, I, West, R, Yurdakul, S, Olivieri, I, Yazici, H & Hatemi, G 2018, 'Management of major organ involvement of Behçet's syndrome: a systematic review for update of the EULAR recommendations', Rheumatology (Oxford, England), vol. 57, no. 12, pp. 2200–2212. https://doi.org/10.1093/rheumatology/key242

Management of major organ involvement of Behçet's syndrome : a systematic review for update of the EULAR recommendations. / Ozguler, Yesim; Leccese, Pietro; Christensen, Robin; Esatoglu, Sinem Nihal; Bang, Dongsik; Bodaghi, Bahram; Çelik, Aykut Ferhat; Fortune, Farida; Gaudric, Julien; Gul, Ahmet; Kötter, Ina; Mahr, Alfred; Moots, Robert J; Richter, Jutta; Saadoun, David; Salvarani, Carlo; Scuderi, Francesco; Sfikakis, Petros P; Siva, Aksel; Stanford, Miles; Tugal-Tutkun, Ilknur; West, Richard; Yurdakul, Sebahattin; Olivieri, Ignazio; Yazici, Hasan; Hatemi, Gulen.

In: Rheumatology (Oxford, England), Vol. 57, No. 12, 13.08.2018, p. 2200–2212.

Research output: Contribution to journalReviewResearchpeer-review

TY - JOUR

T1 - Management of major organ involvement of Behçet's syndrome

T2 - a systematic review for update of the EULAR recommendations

AU - Ozguler, Yesim

AU - Leccese, Pietro

AU - Christensen, Robin

AU - Esatoglu, Sinem Nihal

AU - Bang, Dongsik

AU - Bodaghi, Bahram

AU - Çelik, Aykut Ferhat

AU - Fortune, Farida

AU - Gaudric, Julien

AU - Gul, Ahmet

AU - Kötter, Ina

AU - Mahr, Alfred

AU - Moots, Robert J

AU - Richter, Jutta

AU - Saadoun, David

AU - Salvarani, Carlo

AU - Scuderi, Francesco

AU - Sfikakis, Petros P

AU - Siva, Aksel

AU - Stanford, Miles

AU - Tugal-Tutkun, Ilknur

AU - West, Richard

AU - Yurdakul, Sebahattin

AU - Olivieri, Ignazio

AU - Yazici, Hasan

AU - Hatemi, Gulen

PY - 2018/8/13

Y1 - 2018/8/13

N2 - Objective: To assess the efficacy and safety of treatment modalities for major organ involvement of Behçet's syndrome (BS), in order to inform the update of the EULAR recommendations for the management of BS.Methods: A systematic literature review of all randomized controlled trials, controlled clinical trials, or open label trials assessing eye, vascular, nervous system or gastrointestinal system involvement of BS was performed. If controlled trials were not available for answering a specific research question, uncontrolled studies or case series were also included.Results: We reviewed the titles and abstracts of 3927 references and 161 studies met our inclusion criteria. There were only nine randomized controlled trials. Observational studies with IFN-α and monoclonal anti-TNF antibodies showed beneficial results for refractory uveitis. Meta-analysis of case-control studies showed that immunosuppressives decreased the recurrence rate of deep vein thrombosis significantly whereas anticoagulants did not. CYC and high dose glucocorticoids decreased mortality in pulmonary arterial aneurysms and postoperative complications in peripheral artery aneurysms. Beneficial results for gastrointestinal involvement were obtained with 5-ASA derivatives and AZA as first line treatment and with thalidomide and/or monoclonal anti-TNF antibodies in refractory cases. Observational studies for nervous system involvement showed improved outcome with immunosuppressives and glucocorticoids. Meta-analysis of case-control studies showed an increased risk of developing nervous system involvement with ciclosporin-A.Conclusion: The majority of studies related to major organ involvement that informed the updated EULAR recommendations for the management of BS were observational studies.

AB - Objective: To assess the efficacy and safety of treatment modalities for major organ involvement of Behçet's syndrome (BS), in order to inform the update of the EULAR recommendations for the management of BS.Methods: A systematic literature review of all randomized controlled trials, controlled clinical trials, or open label trials assessing eye, vascular, nervous system or gastrointestinal system involvement of BS was performed. If controlled trials were not available for answering a specific research question, uncontrolled studies or case series were also included.Results: We reviewed the titles and abstracts of 3927 references and 161 studies met our inclusion criteria. There were only nine randomized controlled trials. Observational studies with IFN-α and monoclonal anti-TNF antibodies showed beneficial results for refractory uveitis. Meta-analysis of case-control studies showed that immunosuppressives decreased the recurrence rate of deep vein thrombosis significantly whereas anticoagulants did not. CYC and high dose glucocorticoids decreased mortality in pulmonary arterial aneurysms and postoperative complications in peripheral artery aneurysms. Beneficial results for gastrointestinal involvement were obtained with 5-ASA derivatives and AZA as first line treatment and with thalidomide and/or monoclonal anti-TNF antibodies in refractory cases. Observational studies for nervous system involvement showed improved outcome with immunosuppressives and glucocorticoids. Meta-analysis of case-control studies showed an increased risk of developing nervous system involvement with ciclosporin-A.Conclusion: The majority of studies related to major organ involvement that informed the updated EULAR recommendations for the management of BS were observational studies.

KW - Behçet’s syndrome

KW - Eye involvement

KW - Gastrointestinal involvement

KW - Nervous system involvement

KW - Treatment

KW - Uveitis

KW - Vascular involvement

U2 - 10.1093/rheumatology/key242

DO - 10.1093/rheumatology/key242

M3 - Review

VL - 57

SP - 2200

EP - 2212

JO - Rheumatology

JF - Rheumatology

SN - 1462-0324

IS - 12

ER -