Behçet’s disease in Egypt: a multicenter nationwide study on 1526 adult patients and review of the literature

Gheita, Tamer A.; Latif, Eiman Abd El; El‐Gazzar, Iman I.; Samy, Nermeen; Hammam, Nevin; Noor, Rasha Abdel; El-Shebeiny, Emad; El-Najjar, Amany R.; Eesa, Nahla N.; Salem, Mohamed; Ibrahim, Saad M.; Elessawi, Dina F.; Elsaman, Ahmed M.; Fathi, Hanan M.; Sallam, Rehab A.; El-Shereef, Rawhya R.; Elazeem, Mervat I. Abd; Said, Emtethal A.; Khalil, Noha M.; Shahin, Dina; El‐Saadany, Hanan M.; Elkhalifa, Marwa; Nasef, Samah Ismail; Abdalla, Ahmed M; Noshy, Nermeen; Fawzy, Rasha M.; Saad, Ehab; Moshrif, A.; El-Shanawany, Amira T.; Abdelfattah, Y.; Khalil, Heba Allah Essam E.

doi:10.1007/s10067-019-04570-w

Cited by 54 publications

(26 citation statements)

References 45 publications

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“…However, when applying this cluster pattern, we should consider phenotypic differences among racial and ethnic groups. As compared with cohorts from Middle East countries [54,55], our cohort had a higher frequency of GI involvement and lower frequency of vascular and ocular lesions.…”

Section: Discussionmentioning

confidence: 56%

Cluster analysis of phenotypes of patients with Behçet’s syndrome: a large cohort study from a referral center in China

et al. 2021

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Introduction Behcet’s syndrome (BS) is a complex, heterogeneous disorder. However, classification of its subgroups is still debated. The purpose of this study was to investigate the clinical features and aggregation of patients with BS in China, based on manifestations and organ involvements. Methods This was a cross-sectional study of BS patients in Huadong Hospital of Fudan University between September 2012 and January 2020. We calculated relative risks (RRs) of clinical variables according to sex. Moreover, we conducted a hierarchical cluster analysis applied according to eighteen variables to determine subgroups of patients. Results A total of 860 BS patients were included. Male sex was associated with ocular involvement (RR 2.32, 95% CI 1.67, 3.22, P < 0.0001), vascular involvement (RR 2.00, 95% CI 1.23, 3.23, P = 0.004), cardiac lesion (RR 5.46, 95% CI 2.33, 12.77, P < 0.0001), and central nervous system involvement (RR 2.95, 95% CI 1.07, 6.78, P = 0.007) and was negatively associated with genital ulcers (RR 0.84, 95% CI 0.79, 0.91, P < 0.0001). Five clusters (C1–C5) were observed. C1 (n = 307) showed the skin and mucosa type. In C2 (n = 124), all had articular involvement, barely having major organ involvement except for 18 cases with intestinal lesions. In C3 (n = 156), the gastrointestinal type, 144 patients presented with intestinal involvement, and 36 patients with esophageal ulcers. In C4 (n = 142), all subjects presented with uveitis. C5 (n = 131) consisted of 44 patients with cardiac lesions, 58 with vascular involvement, and 26 cases having central nervous system involvement. Conclusion Our analysis confirmed sex differences in phenotypes of BS. Cluster analysis identified gastrointestinal, uveitis, and cardiovascular involvement cluster separately in different subsets, which represents the most commonly involved organs. Further research is required to replicate and clarify the patterns of phenotype in BS.

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Section: Discussionmentioning

confidence: 56%

Cluster analysis of phenotypes of patients with Behçet’s syndrome: a large cohort study from a referral center in China

et al. 2021

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“…The spectrum of juvenile-onset BS resembled that of the adult counterpart. In support of this finding, a recent Egyptian study of 1562 adults with BS, also including 91 patients (17.2%) with juvenile-onset, did not show any differences in disease manifestations between young and adult patients [19]. This may suggest that differences in pediatric and adult cohorts may be related to disease duration rather than age of onset.…”

Section: Discussionmentioning

confidence: 56%

“…BS usually outbreaks during the third decade [13]. However, a small percentage of patients may experience their first symptom before the age of 16 years [14][15][16][17][18][19]. Due to the relapsing-remitting nature of this syndrome, many years can pass between the first clinical manifestation and the full-blown disease.…”

Section: Introductionmentioning

confidence: 99%

Clinical profile and evolution of patients with juvenile-onset Behçet’s syndrome over a 25-year period: insights from the AIDA network

et al. 2021

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Behçet’s syndrome (BS) represents an understudied topic in pediatrics: the main aims of our study were to characterize demographic and clinical features of a cohort of BS patients with juvenile-onset managed in three tertiary referral centers in Italy, evaluate their evolution in the long-term, and detect any potential differences with BS patients having an adult-onset. Medical records of 64 juvenile-onset and 332 adult-onset BS followed-up over a 2-year period were retrospectively analyzed and compared. Mean age ± SD of first symptom-appearance was 10.92 ± 4.34 years with a female-to-male ratio of 1.06:1. Mucocutaneous signs were the most frequent initial manifestations, followed by uveitis. Throughout the disease course, genital aphthae (76.56%) and pseudofolliculitis (40.63%) prevailed among the mucocutaneous signs, while major organ involvement was represented by gastrointestinal and ocular involvement (43.75 and 34.38%, respectively). No significant differences emerged for both mucocutaneous signs and specific major organ involvement between juvenile-onset and adult BS patients. After excluding nonspecific abdominal pain, juvenile-onset BS patients were less frequently characterized by the development of major organ involvement (p = 0.027). Logistic regression detected the juvenile-onset as a variable associated with reduced risk of long-term major organ involvement (OR 0.495 [0.263–0.932], p = 0.029). In our cohort, juvenile-onset BS resembled the clinical spectrum of adult-onset patients. Pediatric patients with a full-blown disease at onset showed a more frequent mucocutaneous involvement. In addition, patients with juvenile-onset seemed to develop less frequently major organ involvement and had an overall less severe disease course.

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“…Study ethics approval was obtained from the University Health Ethics Research Board. More details on ECR‐BDSG recruitment and data collection can be found elsewhere 12 …”

Section: Methodsmentioning

confidence: 99%

Development and validation of a Behçet's Disease Damage Index for adults with BD: An Explicit, Composite and Rated (ECR) tool

et al. 2021

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Background Behçet's disease (BD) is a chronic multisystem variable vessel vasculitis. Disease damage is irreversible and permanent. Validated tools evaluating damage are limited. Enhancements in the clinical treatment of vasculitis will take place from the development of refined and exclusive indices for individual vasculitic syndromes including BD and attempting their international validation. Objectives This aim was to develop and validate a simple BD Damage Index (BDI). Methods This was a nationwide study including 1252 BD patients. The work consisted of 3 stages. Stage 1: items generation for score content. Stage 2: items selection for the draft score was performed by an expert rheumatologist. Stage 3: the content validity of the draft score was assessed and BDI, Vasculitis Damage Index (VDI), Antineutrophil cytoplasmic antibody‐associated Vasculitis Index of Damage (AVID) and Combined Damage Assessment Index (CDAI) were calculated and compared. Results The mean age of the BD patients was 36.1 ± 9.9 years. Stages 1 and 2 resulted in a BDI instrument containing 73 items with a maximum score of 100. Stage 3, the VDI, CDAI, AVID, and BDI were 2.9 ± 2.2, 3.1 ± 2.3, 3.1 ± 2.3 and 5.1 ± 2.9, respectively. High correlations (r = .9) between comparable damage scores assured acceptable concurrent validity. Conclusion The proposed BDI represents a new robust and potentially useful tool when dealing with BD chronic status.

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Behçet’s disease in Egypt: a multicenter nationwide study on 1526 adult patients and review of the literature

Cited by 54 publications

References 45 publications

Cluster analysis of phenotypes of patients with Behçet’s syndrome: a large cohort study from a referral center in China

Cluster analysis of phenotypes of patients with Behçet’s syndrome: a large cohort study from a referral center in China

Clinical profile and evolution of patients with juvenile-onset Behçet’s syndrome over a 25-year period: insights from the AIDA network

Development and validation of a Behçet's Disease Damage Index for adults with BD: An Explicit, Composite and Rated (ECR) tool

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