Comparison of EULAR/PRINTO/PReS Ankara 2008 and 2022 ACR/EULAR classification criteria for granulomatosis with polyangiitis in children.

OBJECTIVE: Granulomatosis with polyangiitis (GPA) is an ANCA-associated vasculitis. The 2022 ACR/EULAR-endorsed classification criteria for GPA was derived using data only from adult patients. We aimed to assess the performance of the ACR/EULAR classification criteria for GPA in paediatric patients and compare it with the EULAR/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS)-endorsed Ankara 2008 criteria for GPA. METHODS: Retrospective data of paediatric patients with GPA in 20 centres from 9 countries were evaluated. The diagnosis of GPA was made according to the expert opinion. The sensitivity, specificity, positive predictive value, and negative predictive value of the criteria sets were evaluated. RESULTS: The study included 77 patients with GPA and 108 controls [IgA vasculitis (n = 44), Takayasu's arteritis (n = 20), microscopic polyangiitis (n = 16), polyarteritis nodosa (n = 14), Behçet's disease (n = 12), eosinophilic granulomatosis with polyangiitis (n = 1) and Cogan's syndrome (n = 1)] with a median age of 17.8 and 15.2 years, respectively. Among patients with GPA, constitutional symptoms (85.7%) and ENT involvement (79.2%) were the most common presentations. In the GPA group, 73 patients fulfilled the Ankara 2008 criteria and 69 the ACR/EULAR classification criteria. Sensitivities of the Ankara 2008 criteria and the ACR/EULAR classification criteria were 94.8% and 89.6%, while specificities were 95.3% and 96.3%, respectively. No significant difference was found between sensitivities and specificities of both classification criteria (P = 0.229 and P = 0.733, respectively). CONCLUSION: In children, both the ACR/EULAR and EULAR/PRINTO/PReS Ankara 2008 classification criteria for GPA perform well and similarly.

Oral ulcer activity assessment with the composite index according to different treatment modalities in Behçet's syndrome: a multicentre study.

OBJECTIVES: The aim of this multicentre study was to understand patients' needs and to evaluate the oral ulcer activity with the Composite Index (CI), according to different treatment modalities in Behçet's syndrome (BS). METHODS: BS patients (n=834) from 12 centres participated in this cross-sectional study. Oral ulcer activity (active vs. inactive) and the CI (0: inactive vs. 1-10 points: active) were evaluated during the previous month. The effects of treatment protocols [non-immunosuppressive: non-IS vs. immunosuppressive: (ISs)], severity (mild vs. severe), disease duration (<5 years vs. ≥5 years) and smoking pattern (non-smoker vs. current smoker) were analysed for oral ulcer activity. RESULTS: Oral ulcer activity was observed in 65.1% of the group (n=543). In both genders, the activity was higher in mild disease course with non-IS treatment group compared to severe course with ISs (p<0.05). As a resistant group, patients with mild disease course whose mucocutaneous symptoms were unresponsive to non-IS medications were treated with ISs in a limited period and achieved the highest CI scores in females. Oral ulcer activity and poor CI score were associated with disease duration less than 5 years compared to others in male patients (p<0.05). CONCLUSIONS: Oral ulcer activity pattern is affected by both the combination of disease course, treatment protocols and disease duration. CI scores reflected the oral clinical activity and CI might be a candidate scale to evaluate the efficacy of treatments during the follow-up of oral ulcer activity in BS.

What is the role of diffusion tensor imaging (DTI) in detecting subclinical pyramidal tract dysfunction in Behçet's and neuro-Behçet's cases?

The aim of this study is to investigate the pyramidal tract integrity with DTI in Behçet's and neuro-Behçet's cases. We performed this technique in two subgroups of neuro-Behçet's patients (parenchymal and vascular), and Behçet's cases without neurological involvement and control group. Totally, 28 patients were investigated. The control group was composed of 14 healthy people. Cranial MR and DTI were performed in three patient groups and the control group. At DTI, circular regions of interest (ROI) were symmetrically drawn on axial slices on the left and right sides along the pyramidal tract pathway at two levels: middle one third of the cerebral peduncle and posterior limb of the internal capsule. Fractional anisotropy (FA) values for each ROI were obtained by averaging all voxels within the ROI. Calculated FA values on both sides (left and right) of the posterior limb of the internal capsule and cerebral peduncle are significantly lower in all three patient groups when compared to the control group. But there is no any difference of FA values in the selected brain regions of three patient groups. FA values on the posterior limb of the internal capsule and cerebral peduncle do not show a statistically significant difference in parenchymal neuro-Behçet's cases. Our study demonstrates that DTI can detect subclinical pyramidal tract dysfunction in neuro-Behçet's and Behçet's patients. Detection of subclinical nervous system involvement is crucial for morbidity in Behçet's disease. For this reason, studies based on DTI, which include a large number of patients and explore different brain regions, are needed to guide clinicians.

Diagnostic/classification criteria in pediatric Behçet's disease.

Behçet's disease (BD) is a variable vessel vasculitis characterized by recurrent oral and genital aphthosis accompanied by skin, ocular, gastrointestinal, neurologic, and articular involvement. BD is not common in childhood and the disease characteristics considerably differ between adults and children. 18 diagnostic/classification criteria have been published for BD to date. The pediatric BD (PEDBD) criteria, published in 2015, focused on pediatric BD, while the others mainly based on adult studies and are not validated for children. The aim of this review is to summarize the data about diagnostic/classification criteria for BD and to discuss the use and performance of the current criteria in pediatric BD. The covered topics are the characteristics of the diagnostic/classification criteria sets for BD, the factors restricting the universal use/acceptance of these criteria, and pediatric studies testing the performance of BD criteria sets. Having valid and universally accepted criteria with high performance is very important in pediatric BD as they help us determine patients for our studies and guide us through our clinical practice. There are less than 10 pediatric studies testing the performances of BD diagnostic/classification criteria. Their results suggest that revised ICBD (The International Criteria for BD) has the highest sensitivity, while ISG (The International Study Group) criteria remain as the most specific criteria set. Larger multinational pediatric BD cohorts with adequate control groups are required to compare the performance of the different criteria sets in children and to improve the performance of the existing PEDBD criteria.

Behcet's disease in Iran: Analysis of 7641 cases.

Objective: To analyze Behcet's Disease (BD) in Iran, from 1975 to 2018, and compare to 35 large/small reports from other countries.Methods: Patients from all over Iran, when suspected, were sent to the BD Unit. The diagnosis was done by expert opinion. All data were recorded in the BD registry (updated in each follow-up). The data are given in percentage with 95% confidence Intervals.Results: The mean age at onset was 25.6 years. Standard deviation (SD) was 9.8. The mean disease duration was 11.7 years (SD: 8.9). Males were 55.8% (54.7-56.9), Females 44.2% (43.1-45.3), Oral Aphthosis (OA) 97.5% (97.1-97.9), genital aphthosis (GA) 64.4% (63.3-65.5), skin lesions 62.2% (61.1-63.3), ocular lesions 55.6% (54.5-56.7), Joint Manifestations 38.1% (37.0-39.2), Gastrointestinal 6.8% (6.2-7.4), Vascular 8.9% (8.3-9.5), neurological (central-peripheral) 3.9% (3.5-4.3), epididymitis 4.6% (4.1-5.1). Lab tests were positive pathergy test 50.4% (49.3-51.5), elevated ESR 51.1% (50.0-52.2), abnormal urinalysis 13.4% (12.6-14.2). The International Study Group (ISG, 1990) criteria and the International Criteria for Behcet's Disease (ICBD, 2014) had respectively a sensitivity of 76.2% (75.2-77.2) and 96.6% (96.2-97.0). The specificity was 99.3% (99.1-99.5) and 97.3% (96.9-97.7). The accuracy was 86.4% (85.8-87.0) and 96.9% (96.6-97.2).Conclusion: The most frequent manifestations were OA, GA, skin manifestations, and ocular manifestations.

The performance of different classification criteria in paediatric Behçet's disease.

OBJECTIVES: Behçet's disease (BD) is a variable vessel vasculitis. The most widely used classification criteria for adults is the International Behçet's Study Group (ISG) criteria. Recently, the paediatric BD (PEDBD) classification criteria has been developed for children. For disease activity, there are mainly two severity scores; the Iranian BD dynamic activity measure (IBDDAM) and BD current activity form (BDCAF). We tested the performances of PEDBD and ISG criteria and the correlation between severity scores and physician global assessment (PGA) in children with BD. METHODS: Thirty BD patients from Hacettepe University, Ankara, Turkey; 24 from Erciyes University, Kayseri, Turkey; and 14 BD patients from Rambam Medical Centre, Haifa, Israel were included. As controls, children with systemic lupus erythematosus, polyarteritis nodosa, and Crohn disease from Turkey and Israel were included. The sensitivity and specificity of the PEDBD and ISG criteria were evaluated based on the features of the patients before or at 16 years of age. The gold standard for the diagnosis of BD was based on expert opinion at each centre. Expert PGA (visual analogue scale between 0-10; where 0 indicates no disease activity), IBDDAM, and BDCAF were evaluated at the time of diagnosis and at last follow-up in all patients. RESULTS: Sixty-eight BD (disease onset≤16 years; 44.1% male) and 90 control patients were included. The sensitivity and specificity of PEDBD/ISG criteria were 73.5%/52.9% and 97.7%/100%, respectively. Thirty-two (47%) patients with BD failed to fulfill ISG criteria while almost all met PEDBD criteria. The median (interquartile range; IQR) IBDDAM and BDCAF scores at diagnosis were 6(4)/4(2); significantly decreased to 1(2)/1(2), respectively at latest follow-up (p<0.001 for both). The median (IQR) PGA score at diagnosis was 5(2); significantly decreased to 1(2) at latest follow-up (p<0.001). IBDDAM positively correlated with BDCAF (r=0.637; p<0.001). PGA positively correlated with BDCAF and IBDDAM (r=0.502; p<0.001 and r=0.624;p<0.001, respectively). CONCLUSIONS: In our study, the PEDBD criteria showed better sensitivity than ISG criteria which is a big advantage for paediatric patients for early diagnosis. We also demonstrated that the severity scores were positively correlated with each other and PGA; thus may be used in clinical practice for paediatric BD patients.

Birmingham Behçet's service: classification of disease and application of the 2014 International Criteria for Behçet's Disease (ICBD) to a UK cohort.

BACKGROUND: This study reports on the analysis of the application and diagnostic predictability of the revised 2014 ICBD criteria in an unselected cohort of UK patients, and the ensuing organ associations and patterns of disease. METHODS: A retrospective cohort study was conducted using a database of electronic medical records. Three categories were recognised: clinically defined BD, incomplete BD and rejected diagnoses of BD. We applied the ISG 1990 and ICBD 2014 classification criteria to these subgroups to validate diagnostic accuracy against the multidisciplinary assessment. RESULTS: Between 2012 and 2015, 281 patients underwent initial assessment at an urban tertiary care centre: 190 patients with a confirmed diagnosis of BD, 7 with an incomplete diagnosis, and 84 with a rejected diagnosis. ICBD 2014 demonstrated an estimated sensitivity of 97.89% (95% CI: 94.70 to 99.42) and positive likelihood ratio of 1.21 (1.10 to 1.28). The strongest independent predictors were: Central nervous lesions (OR = 10.57, 95% CI: 1.34 to 83.30); Genital ulceration (OR = 9.05, 95% CI: 3.35 to 24.47); Erythema nodosum (OR = 6.59, 95% CI: 2.35 to 18.51); Retinal vasculitis (OR = 6.25, 95% CI: 1.47 to 26.60); Anterior uveitis (OR = 6.16, 95% CI: 2.37 to 16.02); Posterior uveitis (OR = 4.82, 95% CI: 1.25 to 18.59). CONCLUSIONS: The ICBD 2014 criteria were more sensitive at picking up cases than ISG 1990 using the multidisciplinary assessment as the gold standard. ICBD may over-diagnose BD in a UK population. Patients who have an incomplete form of BD represent a distinct group that should not be given an early diagnostic label. Behçet's disease is a complex disease that is best diagnosed by multidisciplinary clinical assessment. Patients in the UK differ in their clinical presentation and genetic susceptibility from the original descriptions. This study also highlights an incomplete group of Behçet's patients that are less well defined by their clinical presentation.

Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD.

BACKGROUND: We aimed to describe the main features of Behçet's disease (BD) in children in the largest prospective cohort to date and to propose a classification. METHODS: An international expert consensus group was formed to define a data set of minimal symptoms for the inclusion of patients. Patients were entered prospectively during 66 months. Experts classified patients on a consensus basis. The concordance of two international classifications was analysed in confirmed patients with BD. Comparisons of subgroups of patients helped define consensus criteria. BD-associated clinical manifestations were also investigated in three control diseases extracted from an independent data set (Eurofever). FINDINGS: In total, 42 centres from 12 countries included 230 patients; data for 219 (M/F ratio=1) could be analysed. The experts classified 156 patients (71.2%) as having confirmed BD. Males more often than females showed cutaneous, ocular and vascular symptoms and females more often genital aphthosis. Age at disease onset and skin and vascular involvement were lower for European than non-European children. Oral aphthosis was the presenting sign for 81% (179/219) of patients. The mean delay to the second symptom was 2.9±2.2 years. International classifications were not concordant with the expert classification. Our paediatric classification contains six categories, a minimum of three signs (each in a distinct category) defining paediatric BD. Three clinical signs discriminated our cohort from the Eurofever cohorts. INTERPRETATION: We present a comprehensive description of a large cohort of patients from both European and non-European countries and propose the first classification of paediatric BD for future therapeutic trials.

Criteria for Behçet's disease with reflections on all disease criteria.

With no specific histologic, laboratory or imaging features the diagnosis/classification of Behçet's Disease (BD) remains clinical. As such, disease criteria are needed. The International Study Group Criteria set is the most widely used. It has some limitations, especially in telling BD from Crohn's disease. On the other hand the main issue, as it also applies to many of the other criteria sets in rheumatology, is our lack of appreciation of a list of misconceptions--some examples of which are unluckily also found in the 2010 ACR/EULAR RA Criteria set--about diagnostic/classification criteria making and their implementation. 1. The view that classification and diagnostic criteria should be different is ill advised in that the cerebral/arithmetic basis of both are the same. 2. The default promise of diagnostic criteria to come once we formulate a classification criteria set is an extension of the previous misconception. 3.Taking pains to avoid circularity in criteria making is unwarranted since the essence of criteria making is circular. In addition we fail to exploit the utility of the disease criteria in ruling out, rather than ruling in, the diseases we seek. Finally we also fail to appreciate the paramount importance of the Bayesian prior (the pretest) probability in formulating and implementing these disease criteria. To formulate criteria tailored to subspecialties, as well as giving the often forgotten family history more importance in our criteria sets are some ways to improve the prior probability on which our diagnostic/classification decisions will be based. We first have to reconcile with ourselves that probabilities are very important in our practice and research. Moreover that reconciliation must also be shared with the public, which includes our patients.

Impact of the positive pathergy test on the performance of classification/diagnosis criteria for Behcet's disease.

BACKGROUND: The only diagnostic test that currently exists for Behcet's disease (BD) is the pathergy test. A positive pathergy test (PPT) is an important component of many of the 16 sets of classification/diagnosis criteria used to diagnose BD. The aim of this study was to determine the importance of a PTT in the performance of the diagnosis/classification criteria for BD. PATIENTS AND METHODS: All patients listed in the BD registry of the Rheumatology Research Center, Tehran (6,727) and 4,648 BD controls were enrolled in the study. The diagnosis was clinical when no other diagnosis could explain the patient's manifestations. The criteria were tested with and without PPT results. Sensitivity, specificity, and accuracy were calculated. RESULTS: Without PPT, all sets of criteria lost sensitivity, gained specificity, and lost accuracy, with the exception of the Cheng-Zhang criteria. The largest loss in sensitivity was for the Hubault-Hamza (35 %) and Dilsen (17.3 %) criteria; the least was for the Curth (1.9 %) and ICBD (6.5 %) criteria. The largest gain on specificity was for the Dilsen (4.7 %) and Curth (3.1 %) criteria; the least was for the Japan (0.1 %) and Japan revised (0.1 %) criteria. The greatest loss in accuracy was for the Hubault-Hamza (20.4 %) and Dilsen revised (9.3 %) criteria; the least was for the ICBD (3.6 %), while Curth gained 0.3 %. CONCLUSION: Without PPT as a criterion for the diagnosis of BD, the sensitivity and accuracy of the sets of classification/diagnosis criteria decrease, while the specificity improves.

Urban legends: recurrent aphthous stomatitis.

Recurrent aphthous stomatitis (RAS) is the most common idiopathic intraoral ulcerative disease in the USA. Aphthae typically occur in apparently healthy individuals, although an association with certain systemic diseases has been reported. Despite the unclear etiopathogenesis, new drug trials are continuously conducted in an attempt to reduce pain and dysfunction. We investigated four controversial topics: (1) Is complex aphthosis a mild form of Behçet's disease (BD)? (2) Is periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome a distinct medical entity? (3) Is RAS associated with other systemic diseases [e.g., celiac disease (CD) and B12 deficiency]? (4) Are there any new RAS treatments? Results from extensive literature searches, including a systematic review of RAS trials, suggested the following: (1) Complex aphthosis is not a mild form of BD in North America or Western Europe; (2) Diagnostic criteria for PFAPA have low specificity and the characteristics of the oral ulcers warrant further studies; (3) Oral ulcers may be associated with CD; however, these ulcers may not be RAS; RAS is rarely associated with B12 deficiency; nevertheless, B12 treatment may be beneficial, via mechanisms that warrant further study; (4) Thirty-three controlled trials published in the past 6 years reported some effectiveness, although potential for bias was high.

Classification Criteria for Behçet Disease Uveitis.

PURPOSE: To determine classification criteria for Behçet disease uveitis. DESIGN: Machine learning of cases with Behçet disease and 5 other panuveitides. METHODS: Cases of panuveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated on the validation set. RESULTS: One thousand twelve cases of panuveitides, including 194 cases of Behçet disease with uveitis, were evaluated by machine learning. The overall accuracy for panuveitides was 96.3% in the training set and 94.0% in the validation set (95% confidence interval 89.0, 96.8). Key criteria for Behçet disease uveitis were a diagnosis of Behçet disease using the International Study Group for Behçet Disease criteria and a compatible uveitis, including (1) anterior uveitis; (2) anterior chamber and vitreous inflammation; (3) posterior uveitis with retinal vasculitis and/or focal infiltrates; or (4) panuveitis with retinal vasculitis and/or focal infiltrates. The misclassification rates for Behçet disease uveitis were 0.6% in the training set and 0% in the validation set, respectively. CONCLUSIONS: The criteria for Behçet disease uveitis had a low misclassification rate and seemed to perform sufficiently well for use in clinical and translational research.

Chronological changes in the systemic manifestations of intestinal Behcet's disease and their significance in diagnosis.

PURPOSE: Intestinal Behçet's disease (BD) is challenging to diagnose, especially if the patient presents with typical colonoscopic findings of intestinal BD without systemic manifestations of BD. We performed this study to evaluate the systemic manifestations of BD in patients with typical colonoscopic findings of intestinal BD at the time of initial presentation and to identity the chronologic changes of these features during an extended follow-up period. METHODS: One hundred twenty-six consecutive patients who showed typical colonoscopic findings of intestinal BD at a single institution in Korea were enrolled. Clinical and endoscopic data were collected from a medical database and using a written questionnaire. Parameters including demographic characteristics and the subset type of BD at the initial and endpoints of the follow-up were analyzed. RESULTS: The mean follow-up period was 63.9 ± 50.9 months. The number of cases that satisfied the International Study Group for Behçet's Disease criteria at initial diagnosis, 19.0%, increased to 53.2% by the end of follow-up. When the Japanese criteria were used for classification, the proportion of complete and incomplete type BD increased (2.4% and 26.2% to 18.3% and 49.2%, respectively), while that of suspected and not-satisfied subtype BD decreased (22.2% and 49.2% to 19.0% and 13.5%, respectively) during the follow-up period. CONCLUSIONS: Our data suggest that patients who lack the systemic manifestations of BD could be included in the category of intestinal BD when typical intestinal lesion is identified, indicating that close examination and early treatment should be considered in such patients.

Evaluation of different classification criteria in children with Behcet disease: results from a single referral center.

OBJECTIVES: Behcet Disease (BD) is a systemic vasculitis, first described with a triad of oral aphthous ulcers, genital ulcers, and uveitis. The authors aimed to share the clinical properties and utilities of three distinct classification criteria for BD in this study. METHODS: This case-control study was conducted in pediatric BD patients, diagnosed between January 2012 and July 2019. The control group included 53 children with other rheumatic disorders. Sensitivity and specificity for International Study Group (ISG), International Criteria for BD (ICBD), and pediatric criteria for BD (PEDBD) criteria were tested. RESULTS: The mean age at symptom onset and diagnosis of the 16 BD patients (6 females, 10 males) were 11.2 ± 3.6 and 13 ± 3.1 years, respectively. The sensitivity and specificity of ISG criteria were 87.5% and 100%. Furthermore, ICBD criteria had a sensitivity and specificity of 93.7% and 98.1%, whereas the authors found sensitivity and specificity as 93.7% and 96.2% for PEDBD. CONCLUSION: ICBD and PEDBD reached higher sensitivity for pediatric BD diagnosis and ICBD had the highest specificity. The authors speculate that the utilization of ICBD may provide early diagnosis of BD in childhood, prevent related morbidities and misdiagnosis.

Behçet's syndrome in Italy: a detailed retrospective analysis of 396 cases seen in 3 tertiary referral clinics.

Behçet's syndrome (BS) is a multisystemic disorder displaying a marked variability across different geographic areas. The main aim of this study was to analyze demographic and clinical features of a cohort of BS patients diagnosed in three tertiary referral centers in Italy and detect potential associations between the different manifestations. Medical records of 396 patients (218 females, 178 males) were retrospectively analyzed. Mean age at onset was 30.00 ± 18.75 years with a female-to-male ratio of 1.22:1. Mucocutaneous features were the most frequent starting manifestations of BS, followed by eye inflammation. Erythema nodosum (p = 0.007), arthritis/arthralgias (p = 0.0115), and central nervous system (CNS) signs (p = 0.014) were significantly over-represented in female patients, whereas male gender was associated with lower mean age at onset (p = 0.031), higher frequency of pseudofollicular lesions, and uveitis (p = 0.00134 and p < 0.0001 respectively), particularly for posterior segment involvement and panuveitis (p < 0.0001). Regarding the association between disease features, genital ulcers were negatively associated with uveitis (p < 0.0001) and vascular involvement (p < 0.0001). Other negative associations were detected between uveitis and gastrointestinal involvement (p = 0.008), pseudofolliculitis and CNS signs (p = 0.031), vascular involvement (p = 0.002) and erythema nodosum (p = 0.013). Logistic regression identified male gender and genital ulcers, respectively, with a higher (OR 2.199 [1.397-3.461], p < 0.001) and lower risk (OR 0.157 [0.090-0.273], p < 0.0001) of developing major organ involvement. Our evaluations found that the disease had started mostly in the second and third decade with most severe features in the male gender, and that patients presenting with mucocutaneous manifestations were less prone to develop major organ involvement.

Relationship between periodontal parameters and Behçet's disease and evaluation of different treatments for oral recurrent aphthous stomatitis.

BACKGROUND AND OBJECTIVE: The highest prevalence of Behçet's disease (BD) has been reported in Turkey, and therefore research of relations between BD and other disorders have become important in the Turkish population. Since oral aphthous ulcers impair tooth brushing, reducing complaints about aphthous ulcers will motivate the patient to maintain better oral hygiene performance and will thus reduce plaque accumulation and periodontal scores. The purpose of this controlled case study was to evaluate the relationship between the severity of periodontal scores and Behçet's disease, and to compare the treatment modalities with neodymium-doped yttrium aluminium garnet (Nd:YAG) laser and medication on the recurrent aphthous ulcers in BD patients by considering the degree of pre- and post-treatment pain, discomfort and functional complications. MATERIAL AND METHODS: The periodontal status of 28 BD patients was evaluated according to periodontal indices. The BD patients were also assessed for clinical severity score as described previously. Levels of pre- and post-treatment pain and functional complications were assessed at patient visits on days 1, 4 and 7. RESULTS: Periodontal indices, the number of oral ulcers and the daily frequency of tooth brushing were related to the severity scores of BD (p < 0.001). The results indicated that BD patients treated with the Nd:YAG laser had less post-treatment pain and fewer functional complications and reported immediate relief of pain and faster healing (p < 0.001). CONCLUSION: Our results suggest that periodontal status is worse in BD patients and is associated with disease severity; also, the Nd:YAG laser has better patient acceptance, shorter treatment time and lower rates of pain and post-treatment adverse events among BD patients with oral recurrent aphthous stomatitis.

A composite index for determining the impact of oral ulcer activity in Behcet's disease and recurrent aphthous stomatitis.

BACKGROUND: Although number, frequency and healing time of oral ulcers and pain are generally used for clinical practice and studies in Behcet's disease (BD) and recurrent aphthous stomatitis (RAS), no standardized activity index is currently present to monitor clinical manifestations associated with oral ulcers. The aim of this study was to develop a standardized composite index (CI) to assess oral ulcer activity in BD and RAS. METHODS: In this cross-sectional study, 121 patients with BD and 45 patients with RAS were included. Sixty-five percentage of BD and 68.9% of RAS patients were in active stage during the previous 3 months. The developed CI included the presence of oral ulcers, ulcer-related pain and functional status and was evaluated in patients with both active and inactive disease for content validity. RESULTS: Composite index score was observed to be higher in active patients with RAS (6.94 + or - 2.19) compared with active BD patients (6.01 + or - 2.04) (P = 0.04). The number of oral ulcers and healing time of oral ulcers were significantly higher in RAS compared with BD (P = 0.018, P = 0.001 respectively). CI score correlated with the number of oral ulcers in both BD and RAS (P = 0.000, P = 0.002 respectively). CI score was '0' for inactive patients without oral ulcer in BD and RAS. CONCLUSIONS: The presented CI as an oral ulcer activity index seems to be a reliable and suitable tool for evaluating the clinical impact and disease-specific problems in BD and RAS.

Classifying patients with Behçet's disease for disease severity, using a discriminating analysis method.

BACKGROUND: Interleukin (IL)-8 has been shown to correlate with the activity of Behçet's disease (BD). The aim of this study was to develop tools as reliable as IL-8 levels in defining BD severity. METHODS: In total, 32 patients with BD and 16 healthy controls were included in the study. Medical history, physical examination, routine laboratory investigations and measurement of serum IL-8 levels were performed. The patients were classified as groups I and II based on the serum IL-8 levels. The patients were then reassigned to groups based on the predictions through discriminating analysis. RESULTS: In addition to the differences between patient and control groups, the differences between groups I and II, group I and controls, and group II and controls were statistically significant. The discriminating analysis results showed that the patients had been assigned to groups I and II with 100% accuracy. CONCLUSION: Discriminating analysis using clinical and routine laboratory findings indicated that 100% of the patients were accurately assigned to the same groups as their IL-8 levels indicated. This suggests that the classification made according to discriminating analysis using these routine variables is a reliable method in determination of the disease severity.

[Diagnosis of intestinal Behçet's disease].

Due to similar manifestations of intestinal Behçet's disease (BD) to those of other colitis such as Crohn's disease or intestinal tuberculosis, it is still challenging for gastroenterologist to accurately diagnose intestinal BD in patients with ileo-colonic ulcers. Moreover, no reliable diagnostic criteria for intestinal BD have been developed yet. Therefore, IBD Study Group of KASID was formulated to establish the guideline for the diagnosis of intestinal BD using a modified Delphi process. The novel diagnostic criteria for intestinal BD were developed based on two aspects; colonoscopic findings and extra-intestinal systemic manifestations, in which patients were categorized into 4 groups including definite, probable, suspected, and non-diagnostic for intestinal BD. Furthermore, Disease Activity Index for intestinal BD was developed through a Korean multicenter study. These diagnostic and disease activity guidelines will contribute to understand intestinal BD.