Systemic lupus erythematosus (SLE) is a highly heterogeneous systemic autoimmune disease characterized by multi-organ involvement, recurrent flares, and chronic progression. With advances in diagnostics and therapeutics, SLE management is shifting from disease control toward long-term remission and organ protection. Incorporating recent global evidence and characteristics of the Chinese population, the National Clinical Research Center for Dermatologic and Immunologic Diseases and the Chinese SLE Treatment and Research Group (CSTAR) have developed the Chinese guidelines for the diagnosis and treatment of systemic lupus erythematosus (2025 edition). Centered on a treat-to-target strategy, the guidelines prioritize clinical remission and low disease activity as primary goals, optimizing the use of glucocorticoids, immunosuppressants, biologics, and novel therapies. Key updates include individualized management recommendations for lupus nephritis, multi-organ involvement, and antiphospholipid syndrome, alongside first-ever evidence-based recommendations on vaccination in SLE patients. By integrating large-scale domestic cohort studies and real-world data, this edition provides more precise and actionable guidance, offering significant value for standardizing and improving SLE diagnosis, treatment, and long-term management in China.

COVID-19 associated Guillain-Barré syndrome (GBS) caused by peripheral nerve damage after SARS-CoV-2 infection is one of the most common COVID-19 related nervous system inflammatory diseases, with high incidence of respiratory failure and mortality. Positive SARS-CoV-2 RNA in cerebrospinal fluid of COVID-19 associated GBS patients has been rarely reported. This paper reports 4 patients with COVID-19 associated GBS in China who developed neurological symptoms 4-15 days after fever and were confirmed SARS-CoV-2 infection. All patients presented with progressive weakness of both lower limbs, 3 patients with autonomic dysfunction such as defecation and urination disorders, and 1 patient with polycranial neuritis and Miller-Fisher syndrome such as bilateral facial palsy, dysphagia, diplopia and ataxia. Nerve conduction velocity and F wave were abnormal in 3 patients, and motor conduction pathway was abnormal in 1 patient. Anti-ganglioside antibodies were tested in 3 patients, and GD1a-IgG was positive in 1 patient. All 4 patients underwent metagenomic next-generation sequencing examination in blood and cerebrospinal fluid. SARS-CoV-2 RNA was positive in blood and cerebrospinal fluid of 3 patients, and SARS-CoV-2 RNA was positive in cerebrospinal fluid of 1 patient.

Spondyloarthritis (SpA) is a group of chronic inflammatory diseases which predominantly involve spine and/or peripheral joints. SpA can be disabling and seriously affect the quality of life and function of patients. With the increasing clinical use of targeted drug therapy, precise and standardized use becomes the focus. China's first Consensus on Targeted Drug Therapy for Spondyloarthritis was developed by National Clinical Research Center for Dermatologic and Immunologic Diseases using international norms for consensus development. The consensus addresses 13 important clinical questions, ranging from principles, patient eligibility, pre-treatment screening, treatment initiation, drug selection and switch, co-medication, to adverse event monitoring of targeted drug therapy in SpA, and recommends treatment for specific patients, playing a key role in guiding clinical practices.

Objective:To evaluate the efficacy and safety of hypomethylating agents (HMA) in patients with myelodysplastic syndromes (MDS) .Methods:A total of 409 MDS patients from 45 hospitals in Zhejiang province who received at least four consecutive cycles of HMA monotherapy as initial therapy were enrolled to evaluate the efficacy and safety of HMA. Mann-Whitney U or Chi-square tests were used to compare the differences in the clinical data. Logistic regression and Cox regression were used to analyze the factors affecting efficacy and survival. Kaplan-Meier was used for survival analysis. Results:Patients received HMA treatment for a median of 6 cycles (range, 4-25 cycles) . The complete remission (CR) rate was 33.98% and the overall response rate (ORR) was 77.02%. Multivariate analysis revealed that complex karyotype ( P=0.02, OR=0.39, 95% CI 0.18-0.84) was an independent favorable factor for CR rate. TP53 mutation ( P=0.02, OR=0.22, 95% CI 0.06-0.77) was a predictive factor for a higher ORR. The median OS for the HMA-treated patients was 25.67 (95% CI 21.14-30.19) months. HMA response ( P=0.036, HR=0.47, 95% CI 0.23-0.95) was an independent favorable prognostic factor, whereas complex karyotype ( P=0.024, HR=2.14, 95% CI 1.10-4.15) , leukemia transformation ( P<0.001, HR=2.839, 95% CI 1.64-4.92) , and TP53 mutation ( P=0.012, HR=2.19, 95% CI 1.19-4.07) were independent adverse prognostic factors. There was no significant difference in efficacy and survival between the reduced and standard doses of HMA. The CR rate and ORR of MDS patients treated with decitabine and azacitidine were not significantly different. The median OS of patients treated with decitabine was longer compared with that of patients treated with azacitidine (29.53 months vs 20.17 months, P=0.007) . The incidence of bone marrow suppression and pneumonia in the decitabine group was higher compared with that in the azacitidine group. Conclusion:Continuous and regular use of appropriate doses of hypomethylating agents may benefit MDS patients to the greatest extent if it is tolerated.

AIM:To investigate the impact of platycodin D(PD)on the viability,migration,invasion,apop-tosis and cell cycle of osteosarcoma cells in vitro,along with its underlying mechanisms.METHODS:Human osteosarco-ma cells MG63 and U2OS were divided into control group(0 μmol/L)and PD treatment group(6.25,12.5,25,50 and 100 μmol/L,respectively).Human osteosarcoma cells MG63 and U2OS were categorized into control groups(0 μmol/L PD)and PD treatment groups(6.25,12.5,25,50 and 100 μmol/L).The CCK-8 assay determined cell viability and identified effective treatment concentrations.MG63(15 μmol/L PD)and U2OS(25 μmol/L PD)were specifically ana-lyzed.Cell scratch and Transwell assays assessed migration and invasion.Hoechst 33342 staining examined nuclear mor-phological changes.Flow cytometry analyzed cell cycle distribution and apoptosis rate.Western blot measured protein ex-pression levels:cleaved caspase-3,cleaved PARP,c-Jun N-terminal kinase(JNK),p-JNK,B-cell lymphoma-2(Bcl-2),Bcl-2-ssociated X protein(BAX),matrix metalloproteinase 2(MMP-2),MMP-9,cyclin-dependent kinase 4(CDK4),cyclin D1,CDK1,cyclin B1,extracellular signal-regulated kinase(ERK)and p-ERK.Proteome sequencing of MG63 cells was performed.RESULTS:PD treatment significantly decreased cell survival,scratch healing rate,and invasive cell numbers,while increasing apoptosis rates(P<0.05).Morphological changes such as nuclear hyperchroma-tism and fragmentation were observed in PD-treated cells.PD induced G2/M phase arrest in MG63 and G0/G1 phase arrest in U2OS cells.PD treatment upregulated BAX,cleaved caspase-3,cleaved PARP,and p-JNK/JNK,while downregulat-ing Bcl-2,MMP-2,MMP-9,CDK4,cyclin D1,CDK1,cyclin B1,and p-ERK/ERK(P<0.05).Proteome sequencing re-vealed PD's involvement in cell division,cell cycle regulation,focal adhesion,apoptosis,and the MAPK signaling path-way.CONCLUSION:PD inhibits cell viability,migration,and invasion of osteosarcoma cells in vitro,while promoting apoptosis and inducing cell cycle arrest.These effects are likely mediated through modulation of the MAPK signaling path-way.

Objective:To retrospectively analyze efficacy and safety of granulocyte colony-stimulating factor(G-CSF)in relapsed/refractory B cell acute lymphoblastic leukemia(R/R B-ALL)patients with neutropenia(NE)after receiving CAR-T cell therapy.Methods:From March 2017 to December 2022,99 patients with R/R B-ALL developed NE after receiving CAR-T cell therapy in Tianjin First Central Hospital were collected and divided into two groups according to using time of G-CSF.One was early G-CSF group(received G-CSF within 7 days,n=56),the other was control group(received G-CSF after 7 d,n=43),whose recovery of NE and occurrence of adverse reactions after G-CSF were compared.Results:Duration of NE in early G-CSF group was shorter than control group[4(2,5.7)vs 11(9,14),P<0.05],but there were no significant differences in the lowest absolute neutrophil count(ANC),degree of NE inhibition and incidence of infection(P=0.261,P=0.09,P=0.111).There was no significant difference between incidence and severity of cytokine release syndrome(CRS)between two groups,and CRS was controllable in all patients.Conclusion:Early application of G-CSF in R/R B-ALL patients after CAR-T cell therapy can shorten duration of NE,and has no significant effect on adverse reactions after CAR-T cells.

Objective:To investigate the clinical characteristics of patients with rheumatic diseases and abnormal liver function, as well as determine the proportion and severity of liver function abnormalities.Methods:Cross-sectional study. Data were collected from patients registered in the Chinese Rheumatism Date Center from 2011 to 2021. The rheumatic diseases analyzed in this study were rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren syndrome (SS), ankylosing spondylitis (AS), and gout. Patient data, including demographic characteristics [ such as age, sex, body mass index,(BMI), and smoking history], liver function test results [including alanine aminotransferase (ALT), aspartate aminotransferase, alkaline phosphatase(ALP), and total bilirubin], and use of anti-rheumatic immune drugs and liver-protective drugs, were collected and compared between groups with normal and abnormal liver functions. In addition, the proportions of abnormal liver function were compared between sex and age groups.Results:A total of 116 308 patients were included in this study, including 49 659 with RA, 17 597 with SLE, 9 039 with SS, 11 321 with AS, and 28 692 with gout. The lowest proportion of liver function abnormalities was observed in patients with RA[11.02% (5 470/49 659)], followed by those with SS[17.97% (1 624/9 039)] and AS [18.22% (2 063/11 321) ], whereas patients with SLE [21.14% (3 720/17 597) ] and gout [28.73% (8 242/28 692)] exhibited the highest proportion of these abnormalities. Elevated ALT, mostly classified as grade 1, was the most commonly noted liver function abnormality, whereas elevated ALP was the least common. Some patients who took liver-protective drugs had normal liver function, with the lowest percentage observed in patients with gout [7.45% (36/483) ] and ranging from 21.7% to 30.34% in patients with RA, SLE, SS, and AS. The proportion of liver function abnormalities was higher in males than in females for all disease types [RA: 13.8%(1 368/9 906) vs. 10.3%(4 102/39 753); SLE: 33.6% (479/1 424) vs. 20.0% (3 241/16 173); SS: 25.4%(111/437) vs. 17.6%(1 513/8 602); AS: 20.1%(1 629/8 119) vs. 13.6% (434/3 202); and gout: 29.3% (8 033/27 394) vs. 16.1% (209/1 298)]. In RA, SLE, and AS, the proportions of liver function abnormalities were similar across all age groups. In SS, the proportion of liver function abnormalities increased with age [<40 years: 14.9%(294/1 979); 40-59 years: 18.1%(858/4 741); ≥60 years: 20.4%(472/2 319)], whereas a reversal of this trend was observed in gout [<40 years: 34.9%(4 294/12 320); 40-59 years: 25.5%(2 905/11 398);≥60 years: 21.0%(1 042/4 971)].Conclusions:The proportions of combined liver function abnormalities in patients with rheumatologic diseases were high, and the utilization rates of liver-protective drugs were low. It is necessary to pay more attention to monitoring patients′ liver function, timely administer liver-protective drugs, and optimize liver-protective regimens during the treatment of rheumatic diseases.

Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a group of systemic small vasculitis characterized by ANCA positive in serum. Three diseases are included in this group of diseases: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). In China, standardized diagnosis and treatment of AAV is still lacking. Based on the evidence and guidelines from China and abroad, the Chinese Rheumatology Association formulated the standardization of diagnosis and treatment of ANCA associated vasculitis. The purpose is to standardize the diagnosis of AAV and disease activity assessment, and recommend the treatment strategies.

Objective:To investigate the expression levels and clinical significance of glioma-associated oncogene homolog 1 (GLI1) and sonic hedgehog signaling molecule (Shh) in the malignant transformation of ovarian endometriosis (EM).Methods:The expressions of GLI1 and Shh were detected by real-time reverse transcription (RT)-polymerase chain reaction (PCR) and EnVision method in 50 cases of ovarian EM tissues, 35 cases of atypical endometriosis (aEM) and 50 cases of endometriosis-associated ovarian cancer (EAOC). The expression differences of two molecular markers in the malignant transformation of ovarian EM were compared, and the relationships between two molecular markers and the clinicopathological features and prognosis of EAOC were analyzed.Results:(1) RT-PCR showed that the expression levels of GLI1 mRNA in EM, aEM and EAOC group were 1.77±0.40, 3.54±0.44, and 7.80±0.24, respectively. The expression levels of Shh mRNA were 0.95±0.21, 3.14±0.35, and 5.41±0.31, respectively. GLI1 and Shh mRNA in EAOC group were significantly higher than those in EM and aEM group (all P<0.01), and there were statistically significant differences between EM and aEM group (all P<0.01). The percentages of GLI1 in ovarian EM, aEM and EAOC were 32% (16/50), 57% (20/35), and 66% (33/50), respectively, meanwhile, the positive expression rates of Shh were 20% (10/50), 49% (17/35), and 54% (27/50), respectively (all P<0.01). GLI1 mRNA expression was positively correlated with Shh mRNA expression in EAOC tissues ( r=0.721, P<0.01). The expressions of GLI1 protein were proportionated to Shh protein in EAOC tissues ( r=0.608, P=0.001). (2) The expression of GLI1 was significantly related to the International Federation of Gynecology and Obstetrics (FIGO) stage, cancer antigen 125 (CA 125) levels, lymph node metastasis, and Platinum resistance in EAOC patients (all P<0.05). The expression of Shh were related to FIGO stage and lymph node metastasis in EAOC patients (all P<0.05). Logistic regression analysis showed that GLI1 expression was an independent risk factor for poor prognosis in EAOC patients ( P<0.05). Kaplan-meier survival analysis showed that the overall survival rate of EAOC patients with high GLI1 expression and low GLI1 expression was 12.1% and 35.3%, respectively, with statistical significance ( χ2=10.73, P<0.01). The overall survival rate of EAOC patients with high and low expression of Shh protein was 11.1% and 30.4%, in which there was statistically significant difference ( χ2=3.96, P=0.047). Conclusion:GLI1 and Shh are highly associated with the malignant transformation of ovarian EM, which may play a role in promoting malignant degeneration of ovarian EM, and the high expression of GLI1 and Shh indicates a poor prognosis in EAOC patients.

Objective:To explore the efficacy and safety of adalimumab (ADA) in Chinese Takayasu arteritis (TAK) patients.Methods:This study prospectively included ten refractory active TAK patients. The clinical, laboratory and imaging data of these patients before and after ADA treatment were collected and compared. Statistical analysis was carried out with the statistical product and service solutions (SPSS) program V.24.0. We performed univariate analysis by Wilcoxon-Mann-Whitney test for numerical data according to normality. The P value <0.05 was interpreted as statistically significant. Results:Ten TAK patients recruited in this study were all female, with a median age of 27.5 [25.0, 36.0] years. Fever or fatigue (6/10, 60%), neck pain (6/10, 60%), and limb claudication (4/10, 40%) were the most common clinical manifestations. The most common pattern of vascular involvement was Numano type V (7/10, 70%). The serum erythrocyte sedimentation rate (ESR) [22.0(4.5, 37.5) mm/1 h vs 7.0(4.5, 31.0) mm/1 h, Z=-2.04, P=0.042] and IL-6 level [12.3(3.6, 57.7) ng/L vs 2.8(2.0, 24.0) ng/L, Z=-2.19, P=0.028] reduced significantly after ADA treatment. The thickness of the common carotid artery intima decreased from 2.7(2.2, 4.0) mm to 2.3(1.6, 3.4) mm after ADA treatment ( Z=-1.99, P=0.046). The lumen diameter of the common carotid artery increased from 0.31(0.20, 0.42) cm to 0.37(0.29, 0.43) cm ( Z=-2.02, P=0.043). The glucocorticoid dosage was reduced from [11.2(6.9, 15.0) mg to 10.0(5.0, 13.1) mg, Z=-1.89, P=0.059]. In the 10 patients, 6 patients (60%) reached complete response, 2 patients (20%) reached the partial response, and the total effective rate was 80%(8/10). Pneumonia in one TAK patient was recorded. Conclusion:The results of this study suggest, that ADA can be used for refractory active TAK patients, and is safe.