Kasabach–Merritt syndrome (KMS) is a rare complication of hemangioma. KMS mostly occurs in the pediatric population with typical clinical manifestations, including thrombocytopenia, consumptive coagulation, and purpura. However, the pathogenesis of KMS is still unclear and the KMS therapy is controversial. We report here a case of KMS and multiple, giant, hepatic hemangiomas in a 34-year-old female patient who was successfully treated in our hospital. Glucocorticoid along with supportive treatments was administrated immediately to reverse fatal disseminated intravascular coagulation and acute hemolysis. After the acute phase, glucocorticoid was tapered slowly and sirolimus was added to treat the hemangiomas. In conclusion, the risk factors of gestation, interventional treatment, and autoimmune disturbance might contribute to the pathogenesis of KMS. Additionally, treatment with glucocorticoid and sirolimus is effective in KMS and multiple giant hepatic hemangiomas.

Objectives: Studies have proven that improving patients' acceptance of chronic pain could be an effective therapy for alleviating pain and other symptoms. Our objectives were to investigate the correlation between chronic pain acceptance and clinical variables in ankylosing spondylitis (AS), and the prediction role of chronic pain acceptance for biologics treatment. Methods: First, 167 AS patients were recruited to complete a series of questionnaires, including the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Functional Index (BASFI), Chronic Pain Acceptance Questionnaire (CPAQ), Hospital Anxiety and Depression Scale (HADS), and Tampa Scale for Kinesiophobia (TSK). Bivariate correlation analysis was utilized to investigate the correlation between pain acceptance and clinical variables. Based on the level of chronic pain acceptance and serum C-reactive protein (CRP), patients were separated into four subgroups. Then, another 68 patients initiating anti-tumor necrosis factor (TNF) treatment were recruited to complete the questionnaires at baseline (T0) and 3 months after treatment (T3). The changes in clinical variables and treatment response were compared between multiple subgroups. Results: Chronic pain acceptance had strong correlations with anxiety, depression and fear of movement, and moderate correlations with BASFI and pain intensity. Both activity engagement (AE) and pain willingness (PW) had significant correlations with pain intensity, BASFI and psychological status. In addition, AE had a significant correlation with disease duration, while PW had a significant correlation with ASDAS-CRP. Subgroup analysis showed that patients with low chronic pain acceptance and high levels of serum CRP had the highest BASDAI. Among patients initiating anti-TNF treatment, those with high pain acceptance and high levels of serum CRP achieved the most obvious reduction in BASDAI after 3 months treatment. Conclusion: Pain acceptance is a new tool to assess pain in AS which may also reflect physical and psychological status. Clinicians should identify high-risk patients with low chronic pain acceptance and high levels of serum CRP, and give psychological and pharmacological intervention promptly. Moreover, the combination of baseline chronic pain acceptance and serum CRP level could be used to predict the treatment response in AS patients initiating biologics treatment.

Purpose . The purpose of this study was to cross-culturally adapt and validate the Simplified Chinese version of the Lower Extremity Functional Scale (SC-LEFS). Methods . The original English version of the Lower Extremity Functional Scale was translated and cross-culturally adapted into Simplified Chinese according to international guidelines. The SC-LEFS and 36-Item Short-Form Health Survey were administered to 213 patients with lower extremity musculoskeletal disorders. Psychometric properties including internal consistency, test-retest reliability, content validity, and construct validity were tested. Results . There were no floor or ceiling effects for the SC-LEFS. The scale had high values for internal consistency (Cronbach α=0.97) and test-retest reliability (intraclass correlation coefficient=0.97). Corrected item-total correlations for every item ranged from 0.67 to 0.89. And the item-level content validity index (I-CVI) for each item ranged from 0.78 to 1.00. Principal component analysis revealed a one-factor structure. Nine of ten prior hypotheses were confirmed, which further supports good construct validity within the SC-LEFS. Conclusion . The SC-LEFS has high internal consistency, good test-retest reliability and content validity, convergent construct validity, and a one-factor structure. Thus, it could be regarded as a reliable and valid tool to assess activity limitations in Chinese patients with lower extremity musculoskeletal disorders.

Calcium oxalate (CaOx), the major constituent of most kidney stones, induces inflammatory infiltration and injures renal tubular cells. However, the role of γδT cells in CaOx-mediated kidney injury remains unclear. Therefore, this study investigated the distribution of intrarenal γδT cells and T cell receptor δ (TCRδ) immune repertoires in response to interactions with CaOx crystals. CaOx crystal mouse model was established by glyoxylate injection. Flow cytometer was used to analyze the expression of CD69 and IL-17 from intrarenal γδT cells. Furthermore, TCR immune repertoire sequencing (IR-Seq) was used to monitor the profile of the TCRδ immune repertoire. Our results indicated that CaOx crystals lead to obvious increases in the expression and activation of intrarenal γδT cells. In TCRδ immune repertoire, the majority of V/J gene and V–J/V–D–J combination segments, barring individual exceptions, were similar between kidneys with CaOx formation and control kidneys. Impressively, high complementarity determining region 3 (CDR3) diversity was observed in response to CaOx crystal formation along with distinct CDR3 distribution and abundance. Our work suggests the presence of aberrant γδT cell activation and reconstitution of the TCRδ immune repertoire in response to CaOx crystal deposition.

Immunization with regulatory T cell ( Treg ) epitope peptides to activate and induce Tregs, by which to suppress pathological autoimmune responses and reconstitute a new homeostasis, is a promising therapeutic regimen for autoimmune rheumatic diseases. However, it is usually hard to induce po-tent peptide-specific immune responses in vivo with small molecular peptides. Bacterial flagellin is one of the agonists triggering innate immune responses. When used as carrier, it shows strong adjuvant activity to its conjugated antigens. In some particular situations, bacterial flagellin can also activate and induce Tregs. Thus if Treg epitope peptides are covalently conjugated to a bacterial flagellin, the conjugates should be able to effectively enhance the Treg-based immune responses via flagellin itself and the adjuvanticity of flagellin to Treg epitope peptides, and thereby enhance the immunotherapeutic effects on autoimmune rheumatic diseases.

目的检测系统性红斑狼疮（SLE）患者外周血中CCR7 lo PD-1 hi 滤泡辅助性T细胞（Tfh）的比例,并探讨其临床意义。方法收集31例SLE患者、29例类风湿关节炎（RA）患者、12例干燥综合征（SS）患者和37名健康对照的外周血样本。采用流式细胞术检测Tfh表面C-X-C趋化因子受体3（CXCR3）、诱导型共刺激分子（ICOS）和信号淋巴细胞激活分子家族成员5（SLAMF5）的表达水平,以及外周血中CCR7 lo PD-1 hi Tfh和CCR7hiPD-1lo Tfh的比例。分析SLE患者外周血中CCR7 lo PD-1 hi Tfh的比例与临床指标、成浆细胞比例的相关性。结果相比CCR7hiPD-1lo Tfh,CCR7 lo PD-1 hi 表面CXCR3、ICOS和SLAMF5的表达水平较高（t=3.73、5.06、8.27,P均＜0.01）。SLE和RA患者外周血中CCR7 lo PD-1 hi Tfh的比例分别为5.79%（2.97%,8.95%）和4.57%（2.51%,7.60%）,均高于健康对照组[2.93%（1.63%,5.21%）,U=314.5、332.5,P均＜0.01],而SS患者外周血中CCR7 lo PD-1 hi Tfh的比例与健康对照相比差异无统计学意义（U=183.0,P＞0.05）。SLE患者外周血中CCR7 lo PD-1 hi Tfh的比例与系统性红斑狼疮疾病活动指数（SLEDAI）、抗双链DNA抗体滴度、成浆细胞比例均呈正相关（r=0.447 1、0.517 4、0.466 9,P均＜0.05）。结论 SLE患者外周血中CCR7 lo PD-1 hi Tfh比例增加与SLEDAI升高和成浆细胞比例的增加相关,检测患者外周血Tfh亚群可间接反映生发中心及B淋巴细胞的功能状态,对SLE的诊断、监测和预后有重要意义。

Acceptance of chronic pain has increasingly become a significant issue in the field of pain management. Many researchers have suggested that patients with better acceptance of pain are more likely to have better functioning both in physical and psychological status. In many countries, the Chronic Pain Acceptance Questionnaire (CPAQ) and CPAQ-8 have been validated and utilized frequently to measure the pain acceptance of patients with chronic pain. However, the CPAQ and CPAQ-8 yet have not been introduced and validated in Mainland China.In this study, we aimed to translate the English version of the CPAQ into simplified Chinese, make proper cross-cultural adaptations, and validate the psychometric properties of the Chinese version of the CPAQ and the CPAQ-8.The English version of the CPAQ was first linguistically translated and cross-culturally adapted to formulate a Chinese version. Then, we recruited 224 patients from a pain clinic and every participant was asked to finish a series of questionnaires. Finally, statistical analysis was performed to test the psychometric properties of the CPAQ and the CPAQ-8.Both confirmatory factor analysis (CFA) and principal component analysis (PCA) confirmed a 2-factor structure for the CPAQ and the CPAQ-8. Nine out of 10 of the hypotheses were validated for construct validity. The overall intraclass correlation coefficient (ICC) value for the CPAQ and CPAQ-8 were 0.92 and 0.89, respectively. In addition, the Cronbach α values for both the CPAQ and the CPAQ-8 showed excellent test-retest reliability.In conclusion, the original CPAQ was successfully developed into the Chinese version of the CPAQ and CPAQ-8 with excellent validity and reliability. The scores of the CPAQ or CPAQ-8 might be a strong predictor for the physical and psychological function of chronic pain patients. In addition, to improve the satisfaction of surgery patients, we recommend measuring patients' pain acceptance using the CPAQ or CPAQ-8 before and after the surgery. For patients with lower acceptance, psychological interventions may be more effective than treatment that simply reduces symptoms. Finally, we suggest that the Chinese version of the CPAQ and CPAQ-8 are appropriate for use in clinical settings or fundamental research in Mainland China.

肿瘤坏死因子α（tumor necrosis factor-alpha, TNF-α）是一种具有多重效应的细胞因子，其在炎症反应、免疫应答等过程中均发挥着重要作用。TNF-α拮抗剂的问世为风湿病患者带来了福音。目前，其已被美国食品药品监督管理局批准应用于类风湿关节炎、强直性脊柱炎、银屑病关节炎等疾病的治疗。除了这些已获批的适应证外，国内外文献报道亦显示，TNF-α拮抗剂在多种适应证外的风湿免疫性疾病中均可使用，但其在不同疾病中使用的有效性及安全性不同，而这种差异可能与疾病的发病机制不同有关。本文将就TNF-α拮抗剂在风湿免疫性疾病中超适应证使用的情况及机制作一综述，为其今后的临床应用及研究提供方向。