Publications by authors named "Hasan Yazici"

145 Publications

Behçet syndrome.

Nat Rev Dis Primers 2021 09 16;7(1):67. Epub 2021 Sep 16.

(Rheumatology) Academic Hospital Istanbul, Istanbul, Turkey.

Behçet syndrome is a systemic vasculitis with an unknown aetiology affecting the small and large vessels of the venous and arterial systems. The presence of symptom clusters, regional differences in disease expression and similarities with, for example, Crohn's disease suggest that multiple pathological pathways are involved in Behçet syndrome. These disease features also make formulating disease criteria difficult. Genetic studies have identified HLA-B*51 as a genetic risk factor. However, the low prevalence of HLA-B*51 in many patients with bona fide disease, especially in non-endemic regions, suggests that other factors must also be operative in Behçet syndrome. Despite lacking a clear aetiological mechanism and definition, management of manifestations that include major vascular disease, eye disease and central nervous system involvement has improved with the help of new technology. Furthermore, even with our incomplete understanding of disease mechanisms, the prognoses of patients with Behçet syndrome, including those with eye disease, continue to improve. New treatment options and a better understanding of the underlying pathogenesis for various manifestations of this condition are required to further improve the management of the disease, which will improve patient quality of life.
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http://dx.doi.org/10.1038/s41572-021-00301-1DOI Listing
September 2021

Effect size, the misnomer.

Clin Exp Rheumatol 2021 Sep 7. Epub 2021 Sep 7.

New York University School of Medicine, New York, NY, USA.

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September 2021

The winter of my discontent.

Authors:
Hasan Yazıcı

Turk Arch Pediatr 2021 1;56(3):177-178. Epub 2021 May 1.

Former Chair, The Ethics Committee, Istanbul University.

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http://dx.doi.org/10.5152/TurkArchPediatr.2021.190421DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8152639PMC
May 2021

Concerns regarding the analysis of a Takayasu arteritis cohort: comment on the article by Goel et al.

Arthritis Rheumatol 2021 May 13. Epub 2021 May 13.

NYU Grossman School of Medicine, New York, NY.

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http://dx.doi.org/10.1002/art.41784DOI Listing
May 2021

The Editor's note. First taking the log out of our own eyes.

Authors:
Hasan Yazici

Clin Exp Rheumatol 2021 Jan-Feb;39 Suppl 128(1). Epub 2021 Feb 19.

Academic Hospital, Istanbul, Turkey.

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May 2021

Immunogenicity of Infliximab Among Patients With Behçet Syndrome: A Controlled Study.

Front Immunol 2020 22;11:618973. Epub 2020 Dec 22.

Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.

Background: Immunogenicity of tumor necrosis factor alpha inhibitors (TNFis) has been recognized as an important problem that may cause loss of efficacy and adverse events such as infusion reactions. TNFis are being increasingly used among patients with Behçet syndrome (BS) and scarce data exist on this topic.

Objective: We aimed to investigate the prevalence of anti-infliximab (IFX) antibodies in patients with Behçet syndrome together with suitable controls.

Methods: We collected serum samples from 66 consecutive Behçet syndrome patients (51 M, 15 F, mean age 37 ± 9 years) who were treated with IFX. Additionally, similarly treated 27 rheumatoid arthritis, 53 ankylosing spondylitis, 25 Crohn's disease patients, and 31 healthy subjects were included as controls. Samples were collected just before an infusion, stored at -80°C until analysis, and serum IFX trough levels and anti-IFX antibodies were measured by ELISA. We used a cut-off value of 1 μg/ml for serum IFX trough level, extrapolating from rheumatoid arthritis studies.

Results: Anti-IFX antibodies were detected in four (6%) Behçet syndrome, five (18.5%) rheumatoid arthritis, three (12%) Crohn's disease, and one (2%) ankylosing spondylitis patient. The median serum IFX trough level was significantly lower in patients with anti-IFX antibodies compared to those without antibodies [2.32 (IQR: 0.6-3.6) vs. 3.35 (IQR: 1.63-5.6); p = 0.019]. The serum IFX trough level was lower than the cut-off value in 6/13 (46%) patients with anti-IFX antibodies and in 25/158 (16%) patients without anti-IFX antibodies (p = 0.015). Among the four Behçet syndrome patients with anti-IFX antibodies, two experienced relapses and two had infusion reactions.

Conclusions: Immunogenicity does not seem to be a frequent problem in Behçet syndrome patients treated with IFX, but may be associated with relapses and infusion reactions, when present.
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http://dx.doi.org/10.3389/fimmu.2020.618973DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7782473PMC
June 2021

The OMERACT Core Set of Domains for Outcome Measures in Behçet Syndrome.

Arthritis Care Res (Hoboken) 2020 Nov 17. Epub 2020 Nov 17.

Division of Rheumatology and Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

Objective: There is an unmet need for reliable, validated, and widely-accepted outcome measures for randomized clinical trials in Behçet syndrome (BS). The Outcome Measures in Rheumatology Clinical Trials (OMERACT) BS Working Group, a large, multi-disciplinary group of experts in BS and patients with BS, worked to develop a Core Set of data-driven outcome measures for use in all clinical trials of BS.

Methods: The Core Domain Set was developed through a comprehensive, iterative, multi-stage project which included a systematic review, a focus group meeting and qualitative patient interviews, a survey among experts in BS, a Delphi exercise involving both patients and physician-experts in BS, and utilization of the data, insight, and feedback generated by these processes to develop a final Core Domain Set.

Results: All steps were completed and domains were delineated across the organ systems involved in this disease. Since trials in BS often focus on specific manifestations and not the disease in its entirety, the final proposed Core Set includes 5 domains mandatory for study in all trials in BS (disease activity, new organ involvement, quality of life, adverse events, and death) with additional sub-domains mandatory for study of specific organ-systems. The final Core Set was endorsed at the 2018 OMERACT meeting.

Conclusion: The Core Set of Domains in BS provides the foundation through which the international research community, including clinical investigators, patients, biopharmaceutical industry, and government regulatory bodies can harmonize the study of this complex disease, compare findings across studies, and advance development of effective therapies.
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http://dx.doi.org/10.1002/acr.24511DOI Listing
November 2020

Correspondence on 'classification criteria: time for a rethink 'by D Porter .

Ann Rheum Dis 2020 Sep 16. Epub 2020 Sep 16.

Division of Rheumatology, Department of Internal Medicine, New York University School of Medicine, New York, New York, USA

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http://dx.doi.org/10.1136/annrheumdis-2020-218775DOI Listing
September 2020

The P-value crisis and the issue of causality.

Authors:
Hasan Yazici

Rheumatology (Oxford) 2020 Jul;59(7):1467-1468

Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.

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http://dx.doi.org/10.1093/rheumatology/keaa152DOI Listing
July 2020

Behçet Syndrome as a Construct

Authors:
Hasan Yazici

Turk J Med Sci 2020 11 5;50(SI-2):1585-1586. Epub 2020 Nov 5.

Department of Behçet Disease Resarch Center, Cerrahpaşa And Academic Hospital, İstanbul University, İstanbul, Turkey

We still do not know the cause(s) of Behçet syndrome. Most probably several, separate disease mechanisms are involved. I, like some others, propose we call it not a disease but a syndrome, a construct with a list of strong and weak elements. I like to think that this frank admission of our ignorance of its cause(s) will be an important semantic stimulus for more meaningful research.
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http://dx.doi.org/10.3906/sag-2002-145DOI Listing
November 2020

Comment on: Clinical course of acute deep vein thrombosis of the legs in Behçet's syndrome: reply.

Rheumatology (Oxford) 2020 04;59(4):914-915

Division of Rheumatology, Department of Internal Medicine, Istanbul University-Cerrahpasa, Istanbul, Turkey.

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http://dx.doi.org/10.1093/rheumatology/kez637DOI Listing
April 2020

Takayasu's arteritis: associated inflammatory diseases.

Clin Exp Rheumatol 2020 Mar-Apr;38 Suppl 124(2):61-68. Epub 2020 Jan 14.

Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.

Objectives: Case reports and series suggest that Takayasu's arteritis (TAK) can co-exist with other inflammatory disorders. We conducted a formal study to look specifically at the frequency of such inflammatory disorders in a large cohort of TAK followed by a single tertiary centre.

Methods: There were 238 patients registered with a diagnosis of TAK. Of these, 19 died, 18 were lost to follow-up and 3 did not wish to respond to our questionnaire. The remaining 198 (175 F/23 M) patients were called back at the outpatient clinic. A standardised form sought whether the patient was also diagnosed with inflammatory bowel disease (IBD), ankylosing spondylitis (AS), Behçet's syndrome (BS), autoimmune or any other inflammatory disorder. The presence of skin-mucosa lesions, inflammatory eye disease and inflammatory back pain were also specifically sought for.

Results: We identified 37 (19%) patients with inflammatory bowel disease (n=12, 6%), ankylosing spondylitis (n=15, 8%) or Behçet's syndrome (n=10, 5%). Thirteen (6.5%) patients had systemic or localised autoimmune disease and 9 (4.5%) miscellaneous inflammatory diseases. Among the 139 patients without any concomitant disease, inflammatory back pain (n=49, 35%) was the most common feature, followed by recurrent oral ulcer (n=20, 14%) erythema nodosum (n=17, 12%), arthritis (n=12, 9%) papulopustular lesions (n=8, 6%) and uveitis/scleritis (n=6, 4%). Only 64 patients (32%) did not have any concomitant disease/condition or specific clinical feature.

Conclusions: TAK does co-occur with IBD, AS and less frequently with BS in about 1/5 of the patients, at least in a hospital setting. There is no clear temporal pattern. The high prevalence of inflammatory back pain in the dorsal spine in TAK needs further scrutiny.
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September 2020

Increased frequency of obstructive sleep apnea syndrome in Behçet's syndrome patients with superior vena cava syndrome.

Clin Exp Rheumatol 2019 Nov-Dec;37 Suppl 121(6):132-136. Epub 2019 Dec 9.

Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.

Objectives: Superior vena cava syndrome (SVCS) is a medical emergency which can also be seen in Behçet's syndrome (BS). Having noted that BS patients with SVCS frequently complained of sleep disturbances, snoring and sleep apnea, suggesting obstructive sleep apnea (OSA), we formally surveyed the risk for OSA among BS patients.

Methods: We studied 28 patients, all male, with SVCS (Group 1), 129 with vascular involvement without a SVCS (Group 2) and 151 with no vascular involvement (Group 3). In addition, 100 apparently healthy individuals (Group 4) were studied. The Berlin questionnaire (BQ), a validated screening tool with a high sensitivity and modest specificity that identifies individuals with high-risk for OSA, was administered to all study participants.

Results: The study groups were similar with regard to age (Group 1, mean age: 44.3±9.7; Group 2, mean age: 41.5±8.7, Group 3, mean age: 40.4±9.4 and Group 4, mean age: 42.1±9.4) mean body mass index and the frequency of hypertension and other comorbidities. The frequency of those patients at high-risk for OSA according to the BQ was 57%, 17%, 17% and 11% in Groups 1, 2, 3 and 4, respectively (p<0.05). Age-adjusted ORs of OSA compared to healthy controls (Group 4) was 11.00 (95%CI: 4.01-30.07) for Group 1, 1.78 (95%CI: 0.81-3.94) for Group 2, 1.92 (95%CI: 0.90-4.14) for Group 3.

Conclusions: BS patients with SVCS are at high risk for OSA. This is probably due to the external pressure of the significant presence of venous collaterals that surround the upper airways. Our results should be further confirmed by polysomnography, and future research should be carried out to clarify the causes of this association.
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January 2020

Long term follow-up of Behçet's syndrome patients treated with cyclophosphamide.

Rheumatology (Oxford) 2020 09;59(9):2264-2271

Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.

Objectives: CYC remains an important treatment option for Behçet's syndrome (BS) patients with life-threatening manifestations. However, adverse events may occur with CYC and this has led to increased use of biologic agents in other vasculitides. We investigated short and long term adverse events associated with CYC use in BS patients.

Methods: We conducted a retrospective chart review of all BS patients treated with CYC between 1972 and 2006. Patients were called in and a standard form was used for collecting demographic characteristics, indication for CYC, its cumulative dose and short term adverse events, defined as those causing discontinuation of CYC, hospitalization and/or death, long term adverse events, including infertility and malignancy, and outcome.

Results: Of 5790 BS patients, 198 (3.4%) had used at least one dose of CYC. Main indications were vascular or neurological involvement. After a median follow-up of 17 years, 52 (26%) patients had died, 113 (57%) could be contacted, and 33 (17%) were lost to follow-up. Vascular involvement was the leading cause of death (n = 27). Seventeen (9%) patients experienced short term adverse events with haemorrhagic cystitis being the most common. After a median follow-up of 25 years (interquartile range: 15-26 years), 17 malignancies occurred in 15 (8%) patients. Infertility was experienced by 26 (30%) patients.

Conclusion: Long term adverse events such as malignancy and infertility were major problems in our BS patients treated with CYC. These results underline the need for safer treatment modalities that are at least as effective as CYC.
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http://dx.doi.org/10.1093/rheumatology/kez598DOI Listing
September 2020

Editorial: Introduction, vasculitis 2020.

Curr Opin Rheumatol 2020 01;32(1):1-2

Department of Medicine (Rheumatology), New York University School of Medicine, New York, USA.

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http://dx.doi.org/10.1097/BOR.0000000000000675DOI Listing
January 2020

Familial Mediterranean fever: different faces around the world.

Clin Exp Rheumatol 2019 Nov-Dec;37 Suppl 121(6):18-22. Epub 2019 Oct 17.

Academic Hospital, Istanbul, Turkey.

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January 2020

Clinical course of acute deep vein thrombosis of the legs in Behçet's syndrome.

Rheumatology (Oxford) 2020 04;59(4):799-806

Division of Rheumatology, Behcet's Disease Research Center, Department of Internal Medicine, Istanbul University-Cerrahpasa.

Objectives: Lower extremity deep vein thrombosis (LEDVT) is a serious complication of Behçet's syndrome. Management constitutes mainly of administration of immunosuppressives, but the predictors of relapse and the optimal choice of immunosuppressives remain unclear. In this prospective study, we aimed to detect the risk and predictors of relapse and treatment response to different modalities.

Methods: All Behçet's syndrome patients who presented with a first episode of acute LEDVT between 2010 and 2014 were prospectively followed with a standard protocol. Acute LEDVT was confirmed by Doppler ultrasonography. Serial planned Doppler ultrasonography assessments were performed during follow-up and additionally repeated in case of clinical suspicion. Recanalization rate was assessed at each visit. Our first-line treatment strategy consisted of AZA and CSs. IFN-alpha was used in patients who were refractory to or could not tolerate AZA or had concomitant eye involvement requiring further treatment.

Results: Thirty-three patients with LEDVT (26 M/7 F) were prospectively followed for 40.7 ± 13.4 months. Among the 33 patients, 23 relapses were observed in 15 patients. Relapse rates were 29%, 37% and 45% at 6, 12 and 24 months, respectively. Among the possible predictors of relapse, poor recanalization was the only significant factor [hazard ratio 4.34 (95% CI 1.96, 10.0)]. Overall 29 patients were treated with AZA and 17 with IFN-alpha. The relapse rate was lower and recanalization rate was higher with IFN-alpha compared with AZA (12% vs 45% and 86% vs 45%).

Conclusion: The relapse rate for LEDVT in Behçet's syndrome is high despite AZA treatment. IFN-alpha seems to be a promising agent for preventing LEDVT relapses and achieving good recanalization.
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http://dx.doi.org/10.1093/rheumatology/kez352DOI Listing
April 2020

Acknowledged statistical help and a better use of p values: a proposal.

Clin Exp Rheumatol 2019 Sep-Oct;37(5):855-857. Epub 2019 Jul 19.

Internal Medicine, Rheumatology, Istanbul Academic Hospital, Istanbul, Turkey.

Objectives: The p-value is commonly misused. We hypothesised that a close cooperation with a statistician would go along with a more proper use of p-values. We considered a close cooperation present, when a statistician was a coauthor, or a formal statistical help was acknowledged in a study report.

Methods: Randomised controlled trials published in 2015-16 in 4 widely read rheumatology journals were searched for a close cooperation with a statistician, the inclusion of effect sizes, confidence intervals, exact rather than relative p-values and the omission of p-values in tables depicting trial entry data.

Results: There were only 28/133 (21%) articles in which a formal statistical help was acknowledged (Group I). The rest (Group II) gave no acknowledgement of a close cooperation. Reporting of effect sizes (96% vs. 71%) and exact p-values (88% vs. 69%) were more in Group I (p=0.01, and p=0.08, respectively).

Conclusions: While a formal acknowledgement of a close cooperation was notably infrequent at 21%, this went along with improvement in some aspects of p-value reporting. If substantiated by further studies, we propose that a formally acknowledged statistical help should improve p-value reporting. Like all professionals, statisticians would like their name/office to be formally associated with their good work.
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October 2019

Increased vein wall thickness in Behçet disease.

J Vasc Surg Venous Lymphat Disord 2019 09 15;7(5):677-684.e2. Epub 2019 Feb 15.

Academic Hospital, Istanbul, Turkey.

Objective: Lower extremity (LE) deep venous thrombosis (DVT) is the main feature of vascular involvement in Behçet disease (BD). We thought that vein wall thickness (VWT) could be a surrogate marker for venous inflammation and hence predict future vascular involvement. We assessed VWT in proximal LE veins in BD patients without DVT, BD patients with DVT, and healthy controls in a formal, masked protocol.

Methods: We studied 50 (43 male and 7 female) BD patients with LE DVT (group 1), 50 (43 male and 7 female) BD patients without any vascular involvement (group 2), and 50 (43 male and 7 female) age- and sex-matched apparently healthy controls (group 3). Two radiologists blinded to the diagnosis of BD used ultrasound to measure VWT of common femoral vein, femoral vein, and great saphenous vein in both legs. Interobserver reliability was assessed using the intraclass correlation coefficient and Bland-Altman plots.

Results: There was good agreement between the two observers. The mean VWT was significantly increased in both BD patients with LE DVT and those without apparent vascular involvement compared with the healthy controls, whereas those with LE DVT had the highest VWT.

Conclusions: VWT of proximal deep and superficial LE veins is increased among the BD patients without any clinical and radiologic vascular involvement. This information, after prospective work, might be useful in management and elucidating disease mechanisms in vascular BD.
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http://dx.doi.org/10.1016/j.jvsv.2018.11.006DOI Listing
September 2019

Assessment of deep venous thrombosis in the lower extremity in Behçet's syndrome: MR venography versus Doppler ultrasonography.

Intern Emerg Med 2019 08 2;14(5):705-711. Epub 2019 Feb 2.

Division of Rheumatology, Department of Internal Medicine, Cerrahpaşa Medical Faculty, University of Istanbul, 81310, Istanbul, Turkey.

Lower extremity venous thrombosis (DVT) is the most common vascular manifestation of Behçet's syndrome (BS). Currently, Doppler ultrasonography (USG) is the most commonly preferred imaging modality in the diagnosis and follow-up of patients with acute and chronic DVT. Magnetic resonance (MR) venography, a quick and a non-invasive imaging modality, is successfully used to detect DVT in various settings. We had been unaware of studies with MR venography in BS. The aim of this study is to compare the diagnostic value of true fast imaging with steady-state precession magnetic resonance (True-FISP MR) venography and Doppler USG in the assessment of chronic DVT among patients with BS. 28 BS patients with chronic lower extremity DVT were studied. Common femoral (CFV) and femoral vein (FV) on both right and left sides were examined for the presence of thrombosis, recanalisation, collaterals and reflux. There are findings of chronic DVT in all Doppler USG images of 28 patients (45 of 56 FV and 35 of 56 CFV), while MR venography detects chronic thrombotic changes in 26/28 (93%) patients (43 of 52 FV and 28 of 52 CFV). Collateral veins are detected in 19 patients (19/28) with MR venography, whereas they are present in only 7 (7/28) with USG (P = 0.003). Furthermore, patients with severe post-thrombotic syndrome are more likely to have collateral formation on the MR compared to those without (12/14 vs 7/14; P = 0.043). Among patients with BS, MR venography might be an alternative or additional method to detect chronic thrombosis in the lower extremities.
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http://dx.doi.org/10.1007/s11739-019-02040-4DOI Listing
August 2019

Faecal but not serum calprotectin levels look promising in predicting active disease in Behçet's syndrome patients with gastrointestinal involvement.

Clin Exp Rheumatol 2018 Nov-Dec;36(6 Suppl 115):90-96. Epub 2018 Dec 13.

Division of Rheumatology, Department of Internal Medicine, Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Istanbul, Turkey.

Objectives: The faecal calprotectin (FC) test is widely used as a non-invasive method for identifying intestinal inflammation. A recent study suggested FC may help to diagnose gastrointestinal involvement of Behçet's syndrome (GIBS). We aimed to determine whether FC helps to distinguish active from inactive intestinal involvement in GIBS.

Methods: We tried to contact 70 GIBS patients registered in our tertiary multidisciplinary clinic. We prospectively collected faecal specimens and serum from 39 GIBS patients who gave informed consent assessing calprotectin and CRP levels followed by a colonoscopy. We included 47 Crohn's disease (CD) patients as controls. Active disease was defined as having ulcer/s on colonoscopy. We filled the Disease Activity Index for Intestinal Behçet's Disease (DAIBD) and Crohn's Disease Activity Index (CDAI). The cut-off for positive FC was defined as ≥150 μg/g.

Results: Ulcers were detected in 12/39 GIBS patients. Sensitivity and specificity of the FC test for active disease was 91.7 (95%CI:61.5-99.8) and 74.1% (95%CI:53.7-88.9). Median FC and CRP levels and DAIBD scores were higher among patients with ulcers, whereas serum calprotectin and CDAI scores were not. A negative FC test was the only significant predictor of remission (OR:37.04, 95%CI:2.4-561.6; p=0.009) on multivariate analysis. Among CD patients, 16/25 active patients and 3/22 patients in endoscopic remission had a positive FC test (OR:11, 95%CI:11-49).

Conclusions: FC, but not serum calprotectin seems to be a useful non-invasive tool for assessing disease activity in GIBS. Whether the presence of oral ulcers can cause false positive results remains to be studied.
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March 2019

Editorial: A review of vasculitis 2019: an introduction.

Curr Opin Rheumatol 2019 01;31(1):1-2

Department of Medicine (Rheumatology), New York University School of Medicine.

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http://dx.doi.org/10.1097/BOR.0000000000000565DOI Listing
January 2019

Management of major organ involvement of Behçet's syndrome: a systematic review for update of the EULAR recommendations.

Rheumatology (Oxford) 2018 12;57(12):2200-2212

Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.

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.
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http://dx.doi.org/10.1093/rheumatology/key242DOI Listing
December 2018

Management of skin, mucosa and joint involvement of Behçet's syndrome: A systematic review for update of the EULAR recommendations for the management of Behçet's syndrome.

Semin Arthritis Rheum 2019 02 19;48(4):752-762. Epub 2018 May 19.

Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Istanbul 34098, Turkey. Electronic address:

Objectives: The aim of this systematic review was to inform the update of European League Against Rheumatism (EULAR) Recommendations for the management of Behçet's syndrome (BS), on the evidence for the treatment of skin, mucosa and joint involvement of BS.

Methods: A systematic literature search, data extraction, statistical analyses and assessment of the quality of evidence were performed according to a pre-specified protocol using the PRISMA guidelines. Studies that assessed the efficacy of an intervention in comparison to an active comparator or placebo for oral ulcers, genital ulcers, papulopustular lesions, nodular lesions or arthritis were included. Where possible, risk ratios were calculated for binary outcomes and mean difference for continuous outcomes.

Results: Among the 3927 references that were screened, 37 were included in the analyses. Twenty-seven of these assessed mucocutaneous and 17 assessed joint involvement. Twenty-one of these studies were randomised controlled trials (RCTs). RCTs with colchicine, azathioprine, interferon-alpha, thalidomide, etanercept and apremilast showed beneficial results with some differences according to lesion type and gender. These agents were generally well tolerated with few adverse events causing withdrawal from the study.

Conclusions: RCTs comprised more than a half (21/37, 57%) of the sources included in the evidence synthesis related to skin, mucosa and joint involvement applicable for the EULAR Recommendations for the management of BS. Differences in the outcome measures that were used across the included studies often made it difficult to combine and compare the results.
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http://dx.doi.org/10.1016/j.semarthrit.2018.05.008DOI Listing
February 2019

Assessing the possible association of anti-TNF use with new malignancies: A neglected methodological consideration.

Pharmacoepidemiol Drug Saf 2018 08 19;27(8):894-901. Epub 2018 Jun 19.

Academic Hospital, Istanbul, Turkey.

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http://dx.doi.org/10.1002/pds.4579DOI Listing
August 2018

2018 update of the EULAR recommendations for the management of Behçet's syndrome.

Ann Rheum Dis 2018 06 6;77(6):808-818. Epub 2018 Apr 6.

Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.

Several new treatment modalities with different mechanisms of action have been studied in patients with Behçet's syndrome (BS). The aim of the current effort was to update the recommendations in the light of these new data under the auspices of the European League Against Rheumatism (EULAR) Standing Committee for Clinical Affairs. A task force was formed that included BS experts from different specialties including internal medicine, rheumatology, ophthalmology, dermatology, neurology, gastroenterology, oral health medicine and vascular surgery, along with a methodologist, a health professional, two patients and two fellows in charge of the systematic literature search. Research questions were determined using a Delphi approach. EULAR standardised operating procedures was used as the framework. Results of the systematic literature review were presented to the task force during a meeting. The former recommendations were modified or new recommendations were formed after thorough discussions followed by voting. The recommendations on the medical management of mucocutaneous, joint, eye, vascular, neurological and gastrointestinal involvement of BS were modified; five overarching principles and a new recommendation about the surgical management of vascular involvement were added. These updated, evidence-based recommendations are intended to help physicians caring for patients with BS. They also attempt to highlight the shortcomings of the available clinical research with the aim of proposing an agenda for further research priorities.
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http://dx.doi.org/10.1136/annrheumdis-2018-213225DOI Listing
June 2018

Behçet syndrome: a contemporary view.

Nat Rev Rheumatol 2018 Jan;14(2):119

This corrects the article DOI: 10.1038/nrrheum.2017.208.
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http://dx.doi.org/10.1038/nrrheum.2018.3DOI Listing
January 2018

Behçet syndrome: a contemporary view.

Nat Rev Rheumatol 2018 02 3;14(2):107-119. Epub 2018 Jan 3.

New York University School of Medicine, NYU Hospital for Joint Diseases, Department of Medicine (Rheumatology), 333 East 38th Street, New York, NY, 10016, USA.

The presence of symptom clusters, regional differences in disease expression and similarities with, for example, Crohn's disease suggest multiple pathological pathways are involved in Behçet syndrome. These features also make formulating disease criteria difficult. Genetic studies have identified HLA-B*51 to be the important genetic risk factor. However, the low prevalence of HLA-B*51 in many patients with bone fide disease, especially in non-endemic regions, suggests other factors must also be operative in Behçet syndrome. This consideration is also true for the newly proposed 'MHC-I-opathy' concept. Despite lacking a clear aetiological mechanism and definition, management of manifestations that include major vascular disease (such as Budd-Chiari syndrome and pulmonary artery involvement), eye disease and central nervous system involvement has improved with the help of new technology. Furthermore, even with our incomplete understanding of disease mechanisms, the prognoses of patients with Behçet syndrome, including those with eye disease, continue to improve.
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February 2018

The prevalence of Behçet's disease in the north of Jordan: a hospital-based epidemiological survey.

Clin Exp Rheumatol 2017 Nov-Dec;35 Suppl 108(6):51-54. Epub 2017 Nov 27.

Cerrahpaşa Hospital, University of Istanbul, Turkey.

Objectives: To estimate the prevalence of Behçet's disease (BD) in Jordan, with the additional aim of comparing this prevalence among hospital workers in other geographical areas.

Methods: In the first stage of our survey, 2,569 employees from 6 hospitals in north Jordan were interviewed using a screening questionnaire to identify individuals with recurrent oral ulcers (ROU), a previous diagnosis of BD (PDBD) and/or any major symptom related to BD. In the second stage, all individuals with ROU or PDBD identified at stage 1, were examined by 2 rheumatologists for the presence/confirmation of BD according to the International Study Group (ISG) criteria. Pathergy test was performed according to recommendations.

Results: ROU were present in 210 (8.2%) individuals. BD was confirmed in 10 employees with PDBD. Seven more BD patients were found. Mean age of 17 BD patients was 38.6±10.7 (range 26-65 y). M: F was 2.4:1. Pathergy test was positive in 8/17. A family history of ROU or BD was noted in 9 (52%) and 3 (25.0%), respectively, compared to 227 (8.9%) and 62 (2.6%) in the whole group, excluding the BD patients (p<0.001 and 0.008, respectively). The prevalence rate of BD in the north of Jordan was estimated as 66:10.000 (95% CI 34.8 to 97.5:10000).

Conclusions: The results of this first ever survey indicated that the prevalence of BD in the north of Jordan is among the highest in the world. This prevalence can now be compared to hospital workers in other geographical areas.
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February 2018
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