Jianming Lai

Background: Vascular Behçet’s disease (VBD) rare and severe subtype of Behçet disease characterized by vascular inflammation. Its prevalence in the pediatric population is extremely low. This case series aims to delineate the clinical features and long-term outcomes of pediatric VBD to enhance clinical recognition and optimize management strategies. Case presentation: We retrospectively reviewed five pediatric patients with VBD, all of whom were female. Arterial involvement was universal, with a subset of patients exhibiting concurrent venous disease. All patients showed a favorable response to glucocorticoids combined with immunosuppressants or biologic agents. Clinical manifestations were dominated by mucocutaneous and systemic features, including erythema nodosum, oral ulcers, genital ulcers, persistent fever, and abdominal pain. Laboratory evaluation revealed elevated inflammatory markers (C-reactive protein and/or erythrocyte sedimentation rate) in all cases. Vascular imaging confirmed the presence of vascular wall thickening and luminal stenosis. Colonoscopy at diagnosis identified multiple intestinal ulcers with surrounding mucosal hyperemia and edema in all five patients. “Clinical improvement“ was defined as resolution of symptoms and normalized inflammatory markers; “resolution” meant complete disappearance of vascular lesions; “amelioration” meant ≥50% reduction in wall thickening or stenosis. During follow-up, all patients exhibited significant clinical improvement, with vascular lesions demonstrating resolution or significant amelioration on repeat imaging; intestinal ulcers healed in the four patients who underwent follow-up colonoscopy. Conclusions: This pediatric case series highlights that VBD can affect vessels of various sizes and typically presents with a constellation of characteristic mucocutaneous and systemic symptoms. Early diagnosis and proactive vascular screening followed by combination immunosuppressive therapy were associated with favorable clinical and radiological outcomes. Causal conclusions cannot be drawn due to the lack of a control group. Increased awareness of this rare vasculopathy is critical for prompt intervention and improved patient prognosis.

Objective: Vasculo-Behçet’s syndrome (VBS) is a rare subtype of Behçet's syndrome (BS) characterized by vascular involvement, and its clinical profile in the pediatric population remains poorly characterized due to limited available data. This study aimed to describe the clinical characteristics, treatment strategies, and prognosis of pediatric VBS and to provide evidence for early identification and clinical management. Methods: A retrospective analysis was performed on the clinical data of 12 pediatric patients (≤18 years old) with VBS admitted to three centers from January 2013 to December 2023. Demographic data, clinical manifestations, laboratory results, treatment regimens, and follow-up outcomes were collected and analyzed. Results: Among the 12 patients, there were 5 boys and 7 girls, with a median age at onset of 9.5 years (range: 3–13 years). Arterial involvement was observed in 10/12 cases, mainly characterized by vascular wall thickening (6/12 cases) and luminal stenosis (5/12 cases), involving the pulmonary artery, aorta, and multiple other sites. Venous involvement was found in 5/12 cases, predominantly wall thickening with thrombosis (3/12 cases). Multisystem involvement was common, including the skin (10/12 cases), the gastrointestinal tract (9/12 cases), and the urinary system (6/12 cases). Inflammatory markers (CRP/ESR) were elevated in 11/12 cases. All patients received glucocorticoid therapy; 11/12 cases received it combined with immunosuppressants, 9/12 cases with biological agents, and 4/12 cases underwent surgical treatment. With a median follow-up of 2 years (range: 4 months–5 years), 8/12 cases achieved stable remission, 2/12 cases (both complicated by aneurysms) had multiple relapses, 1/12 case died of sudden cardiac death, and 1/12 case showed no improvement. Conclusion: Pediatric VBS is a rare and heterogeneous condition with frequent arterial involvement in this cohort. Vascular wall thickening may aid in the early recognition of this condition, while aneurysms may be associated with poorer outcomes.

Childhood-onset Takayasu arteritis (c-TAK) in infants and young children often presents with systemic inflammation rather than ischemic symptoms, and its c

Paediatric Rheumatology; Authors: M. Wen, M. Li, L. Wang, Y. Xu, D. Zhang, J. Zhu, F. Wu, J. Lai

Background Limited studies focused on characteristics of childhood-onset Takayasu arteritis (cTAK) throughout growth and development. Methods 111 cTAK patients were recruited from six tertiary hospitals across China from January 2009 to December 2021. Patients were classified into infant group (P < 0.0125). Adolescent patients were more likely to present with headache (33.3%), dizziness (24.2%) and fatigue (51.5%; all P-trend < 0.001). Infantile patients were more likely to have coronary artery involvement (75.0%, P trend < 0.001). Adolescent patients were more likely to have superior mesenteric artery (36.4%, P trend = 0.005), carotid artery (30.3%, P trend = 0.003), and celiac trunk artery stenosis (27.3%, P trend = 0.005).Younger patients, especially infants, had a lower proportion of glucocorticoids use (P trend = 0.001). 73.0% (81/111) of patients were in remission, with a median follow-up time of 2.00 [2.00, 5.00] years. Conclusion The age-specific patterns identified in this study offered valuable insights for a comprehensive understanding of cTAK. Impact Infantile Takayasu arteritis patients tended to present with fever, elevated inflammatory biomarkers and coronary artery involvement. Adolescent patients were more likely to present with headache, dizziness and fatigue, with superior mesenteric artery, carotid artery involvement and celiac trunk artery stenosis. Clinical manifestations and vascular involvement of childhood-onset Takayasu arteritis differ across age groups. The clinical manifestations of childhood-onset Takayasu arteritis lack specificity. The age-specific patterns identified in this study may provide clues for early diagnosis.

This multicenter study aimed to address the heterogeneity of chronic recurrent multifocal osteomyelitis (CRMO) by identifying clinical subtypes through cluster analysis, exploring clinical features, treatment approaches, and short-term prognosis to improve management of pediatric CRMO. Data from 42 pediatric CRMO patients (47.6% male; mean age 7.87 ± 3.45 years) diagnosed between June 2018 and June 2024 were analyzed. Using cluster analysis with 17 variables, patients were categorized into phenotypic subgroups. Statistical tests assessed differences in clinical features, treatment, and outcomes. Kaplan-Meier survival analysis and log-rank tests evaluated recurrence risk and final Physician Global Assessment(PGA) scores. Patients were classified into two groups: chronic bone pain and acute systemic inflammation. Significant differences were found in fever occurrence (P = 0.002), C-reactive protein (CRP), interleukin-6(IL-6), cytokines including tumor necrosis factor-α(TNF-α) elevation (P = 0.013, 0.003, 0.029), and Hemoglobin(HB), alkaline phosphatase (ALP) reduction (P = 0.007, < 0.001). PGA scores also differed significantly (P < 0.001). Although baseline differences existed, post-treatment recurrence risk and final PGA scores showed no significant differences (P = 0.247, P = 0.211). Treatment differed only in glucocorticoid use; non-steroidal anti-inflammatory drugs (NSAIDs), disease-modifying antirheumatic drugs(DMARDs), TNF inhibitors, and diphosphonates showed no statistical differences. Both groups reached remission approximately 12 months post-diagnosis. Two distinct clinical phenotypes of pediatric CRMO were identified, each achieving favorable outcomes with tailored treatments. Recognizing these phenotypes may guide clinical strategies and improve prognosis for CRMO patients.

This study aims to summarize and explore the clinical characteristics, treatment strategies, and follow-up outcomes of infantile Takayasu arteritis (TA) in the Chinese Han population. In this retrospective study, we collected clinical data, Kerr scores (the NIH score), and arterial involvement scores (AIS) of infants diagnosed with TA at the time of initial diagnosis and at 1, 3, 6, and 12 months following initial treatment. Prognostic differences between various treatment groups were also analyzed. A total of 18 Chinese Han infants with TA were included. The average age of onset was 85.06 ± 54.64 days. The main clinical manifestations included fever (77.78%) and hypertension (55.56%). Both the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were elevated above normal. The average Kerr score was 3.28 ± 0.67, and the AIS was 10.67 ± 4.30. The most commonly affected arteries were the coronary arteries (72.22%) and the abdominal aorta (72.22%), with arterial wall thickening present in all patients (100%). All patients were initially diagnosed with bacterial infections, and 16 were specifically diagnosed with Kawasaki disease empirically. Ten patients received infliximab treatment, and eight were treated with tocilizumab. At 6 months post-treatment, both the AIS and Kerr scores showed significant improvement compared to initial values (t = 2.469, p = 0.019; t = 12.369, p = 0.000). No deaths occurred during the 12-month follow-up. No significant differences were observed between the tocilizumab and infliximab treatment groups. ITA is primarily characterized by fever and hypertension, along with elevated inflammatory biomarkers. The condition is easily diagnosed empirically with bacterial infections and Kawasaki disease, particularly in female infants around 3 months of age. Arterial involvement is often extensive and severe. Aggressive treatment within the first six months after diagnosis can significantly improve clinical outcomes. Both infliximab and tocilizumab demonstrated effective control of arterial inflammation.