ベーチェット病

OBJECTIVES: Studies on drug survival provide real-world data on efficacy as well as safety. We aimed to evaluate the long-term drug survival of adalimumab (ADA) in a large cohort of patients with Behçet's syndrome (BS). METHODS: We conducted a retrospective chart review of 335 BS patients who started ADA between 2008 and 2023. All treatment discontinuations were considered as events, regardless of the underlying reason, to reflect overall treatment persistence in a real-world setting. RESULTS: ADA retention rates at 6, 12, and 24 months were 88.9%, 78.3%, and 67.1%, respectively. Over a mean follow-up of 26.6 (23) months, 137 patients (41%) discontinued ADA. The most common reason for discontinuation was inefficacy, followed by adverse events, non-compliance, remission, and other reasons. No significant associations were observed between treatment discontinuation and age, sex, disease duration, prior tumor necrosis factor inhibitor exposure, concomitant disease-modifying antirheumatic drug use, or major organ involvement. Dose interval extension was performed in 36 patients, with relapse or flare occurring in 5 (14%), all of whom achieved remission after dose re-adjustment. Dose intensification was applied in 30 patients, resulting in remission in 17 (56.6%). CONCLUSION: ADA showed a good retention rate at month 6, followed by a gradual decline in drug retention over time. Secondary inefficacy was the most common reason for treatment discontinuation, followed by adverse events and non-compliance, whereas primary inefficacy was uncommon. Dose intensification to achieve active disease control and dose de-escalation in patients with sustained remission may be feasible management strategies.

Behçet's disease is a systemic vasculitis that can cause life-threatening complications. We report the case of a 28-year-old man with Behçet's disease who developed severe hypertension and generalized seizures. Imaging revealed concurrent complete occlusions of the left renal and common carotid arteries, with segmental infarction of the right kidney. Renal vasculature involvement caused marked hyperreninemia and renovascular hypertension, subsequently triggering posterior reversible encephalopathy syndrome. Treatment with high-dose glucocorticoids, cyclophosphamide, and strict blood pressure control resulted in clinical and neuroimaging resolution. This case illustrates that Behçet's disease can manifest as occult, multivessel arterial occlusions. Early systemic vascular screening and prompt immunosuppression are pivotal for preventing irreversible complications.

UNLABELLED: We examined associations between clinical features at first paediatric rheumatology assessment and eventual benign/self-limited (Group 1) versus systemic/chronic (Group 2) diagnostic categories in children referred for recurrent oral aphthosis. We retrospectively studied 404 children evaluated from 2010 to 2024. Baseline clinical variables were documented at the first rheumatology visit before final diagnosis; follow-up assessments were performed every 3-6 months. Eight prespecified clinical features entered multivariable logistic regression. Firth and diagnosis-exclusion sensitivity analyses addressed sparse data and diagnostic-composition bias. CRP/ESR and HLA-B51 were analysed separately. The cohort included 198 males (49.0%) and 206 females (51.0%); age at symptom onset ranged from 2 months to 15 years. Group 1 included 267 patients, and Group 2 included 137. Recurrent fever (aOR, 0.25; 95% CI, 0.12-0.49) and tonsillitis/pharyngitis (aOR, 0.17; 95% CI, 0.09-0.32) were associated with lower odds of Group 2, whereas genital aphthosis was associated with higher odds (aOR, 13.08; 95% CI, 3.04-56.32). After simultaneous exclusion of PFAPA and Behçet disease spectrum diagnoses, the fever/pharyngitis associations were attenuated, and the genital aphthosis estimate became uninformative because of sparse data, while arthralgia/arthritis remained positively associated. Elevated CRP/ESR was not independently associated with Group 2; HLA-B51 positivity was more frequent in Group 2 among selectively tested patients. CONCLUSION: In children referred for recurrent oral aphthosis, recurrent fever and pharyngitis were mainly associated with benign/self-limited diagnoses, whereas genital aphthosis was mainly associated with systemic/chronic diagnoses. These associations were largely driven by PFAPA and Behçet spectrum disease, respectively. Articular involvement showed the most consistent positive association across sensitivity analyses. These findings are descriptive, not a validated diagnostic prediction tool. WHAT IS KNOWN: • Oral aphthae are common and non-specific in childhood. • Associated symptoms guide diagnostic work-up and follow-up. WHAT IS NEW: • PFAPA-characteristic features clustered with benign/self-limited diagnoses; genital aphthosis with systemic/chronic diagnoses. • After PFAPA/Behçet spectrum exclusion, these signals attenuated; articular involvement was the most consistent positive association.

BACKGROUND/OBJECTIVES: Erythema nodosum (EN) is a septal panniculitis clinically characterized by bilateral erythematous tender subcutaneous nodules typically found on the shins. While recognized as the most common form of panniculitis, it is less frequently documented in children and infants. Currently, there are no established guidelines to diagnose and manage EN in children. This review aims to consolidate current data on triggers, clinical manifestations, and treatment options, and propose a management algorithm for pediatric erythema nodosum (PEN). METHODS: A systematic literature review of published PEN cases up to April 22, 2024, was conducted using PubMed, Embase, Cochrane, and MEDLINE databases. RESULTS: After full-text article review, 239 articles were included, representing 959 patients. The mean age was 10.2 years, ranging from 0.58 to 17.5 years. Infections were the main triggers: bacterial infections in 302 patients (31.5%), predominantly streptococcal; tuberculosis in 193 patients (20.1%); followed by idiopathic cases in 80 patients (8.3%); inflammatory bowel diseases (IBD) in 66 patients (6.9%); Behçet's disease in 61 patients (6.4%); leprosy in 38 patients (4.0%); drug related in 28 patients (2.9%); and to a lesser extent various other infectious agents, genetic diseases, vaccines, autoimmune and inflammatory conditions, and malignancies. Diagnosis relied mainly on clinical assessment, with only 111 patients (11.6%) undergoing skin biopsy. Most patients received supportive care, including NSAIDs, along with treatment of the underlying cause. CONCLUSIONS: Erythema nodosum affects children. It is mainly triggered by infections. The diagnosis is primarily clinical. Supportive care is the mainstay of treatment along with addressing the underlying cause.

During the review process, it was identified that the ISSN number in the header linked to the manuscript titled "Fatal Aortic Regurgitation in Behçet's Disease: A Case Report Highlighting Pitfalls and Lessons in Preoperative Diagnosis" required correction. The necessary update has been made to ensure accuracy in the publication record. [1]. We apologize for any inconvenience caused and appreciate the opportunity to rectify this matter. The original article can be found online at https://www.eurekaselect.com/article/151962. Original: 1573-4056/26 Corrected: 1875-6603/26.