Lupus nephritis (LN), one of the most severe complications of systemic lupus erythematosus (SLE), has a complex pathogenesis involving various endogenous factors including autoimmune complex deposition, inflammatory cell infiltration, and cellular damage. Recent research has increasingly highlighted the prominent role of inflammasomes, particularly the nucleotide-binding oligomerization domain-like receptor protein 3 (NLRP3) inflammasome, in LN pathogenesis. Substantial evidence has confirmed its significant role in both the onset and progression of LN. Given that the NLRP3 inflammasome is a critical factor in triggering and exacerbating LN, its mechanism of action warrants in-depth exploration. Furthermore, research on intervention strategies targeting the NLRP3 inflammasome to ameliorate LN is of great significance. This article reviews the latest advances in the role of the NLRP3 inflammasome in LN pathogenesis and related intervention studies, which may offer new insights for the clinical diagnosis and treatment of LN.
Humans ; Lupus Nephritis/etiology* ; NLR Family, Pyrin Domain-Containing 3 Protein/metabolism* ; Inflammasomes/immunology* ; Animals

Objective To explore the characteristics of myocardial injury in patients with acute myocardial infarction(AMI)complicated by pleural effusion and its effect on long-term prognosis.Methods It was a prospective single-center study.Patients with AMI who were admitted to hospital within 15 days from symptom onset and performed echocardiography and cardiac magnetic resonance imaging(CMR)during hospitalization were consecutively enrolled and assigned to the with-pleural effusion group and the without-pleural effusion group according to the echocardiography result.Baseline data,cardiac magnetic resonance myocardial injury index and echocardiography characteristics were compared between the two groups.The occurrence of major adverse cardiovascular and cerebrovascular events(MACCE)was recorded through outpatient follow-up and telephone follow-up,including all-cause death,re-infarction,revascularization,rehospitalization for congestive heart failure and stroke.Cox regression analysis was performed to analyze influencing factors of all-cause death.Results Among 211 patients,31(14.7%)patients had pleural effusion and 180(85.3%)had no pleural effusion.Compared with the group without pleural effusion,the left ventricular end-diastolic diameter was larger,and left ventricular ejection fraction assessed by echocardiography was lower in the group with pleural effusion(P＜0.05).There were no significant differences in infarct size,left ventricular end-diastolic volume,left ventricular end-systolic volume,left ventricular ejection fraction and the presence of microvascular obstruction and intramyocardial hemorrhage between the two groups in CMR(all P＞0.05).At a median follow-up of 31 months,MACCE occurred in 43(20.4%)patients,and there was no significant difference between the two groups(χ2=3.160,P=0.075).Six cases(2.8%)had all-cause death.The incidence of all-cause death was higher in the group with pleural effusion than that in the group without pleural effusion(9.7%vs.1.7%,P<0.05).There was no significant difference in the incidence of other adverse events between the two groups(P＞0.05).Multivariate Cox regression analysis showed that advanced age and presence of pleural effusion were independent risk factors of all-cause death during follow-up.Conclusion Patients with AMI combined with pleural effusion have more severe myocardial injury and higher all-cause mortality.

Antibodies, Antiphospholipid ; Antiphospholipid Syndrome ; Female ; Humans ; Killer Cells, Natural ; Pregnancy ; Pregnancy Complications

Objective:To document the clinical features of children with cerebral palsy (CP) using magnetic resonance imaging (MRI).Methods:The gross motor functioning of 325 children diagnosed as having CP was graded using the gross motor function classification system (GMFCS). The GMFCS grades were correlated with MRI results in univariate and multivariate logistic regression analyses. The significance of any relationship between the MRI results and co-morbidities was tested using chi-squared tests.Results:Cerebral dysplasia, cerebroventricular enlargement, periventricular leukomalacia (PVL), abnormal signals in the thalami, and morphological changes after hypoxic ischemic encephalopathy were all found to be significantly correlated with GMFCS grading. Moreover, the chi-squared tests indicated that PVL children, children with thinning of the corpus callosum and/or abnormal signals in the thalami were significantly more likely to have visual, auditory or speech impairment complications and/or mental retardation.Conclusions:The findings from MRI correlate well with types of CP, GMFCS grades and co-morbidities among CP children. MRI can be an effective tool for early diagnosis and prognosis of CP in children, indicating needs for clinical rehabilitation.

Objective:To investigate the clinical features and treatment status of immune-related myositis (IRM) caused by immune checkpoint inhibitors (ICIs) in order to improve the diagnosis and treatment rate of the disease.Methods:Two cases of IRM combined with the diagnosis and treatment were described and the literature about IRM in the past 10 years was reviewed, and the clinical data of 59 patients were analyzed.Results:IRM was more common in males, with a total of 47 (79.7%). IRM usually occurred after 45 days of medication or after two doses. The clinical manifestations were mainly myalgia and muscle weakness, which were more common in the limbs. The initial symptoms were ptosis and diplopia. Fifty patients (84.7%) had serum creatine kinase (CK) levels higher than twice the upper limit of normal (UNL). In immunological examinations, 18 patients were found to be positive for anti-rhabdoid muscle antibody (AsM-Ab), while most of the myositisspecific antibodies (MSAs) and myositis-associated antibodies (MAAs) were negative. Thirty-four patients (75.6%) had abnormal EMG, and most patients showed myogenic injury. Muscle magnetic resonance imaging (MRI) showed muscle edema and inflammation in 8 patients. Muscle biopsies from 18 (45.0%) patients showed varying degrees of necrotic myofibers. Fifty-seven patients (96.6%) discontinued ICIs after developing IRM, 54(91.5%) received cortico-steroids, and 20(33.9%) received other treatments including intravenous immuno-globulin (IVIG), plasma exchange.Conclusion:IRM can occur in the early stage of ICIs treatment. Electro-myography, muscle MRI and muscle biopsy in suspicious cases can improve the diagnosis rate of the disease. Early use of corticoteroid, IVIG and other immunotherapy can effectively alleviate the disease.

Objective:To compare the differences in sleep structure between healthy children and children with cerebral palsy (CP) using polysomnography (PSG).Methods:Fifty-six children aged 1-15 hospitalized for cerebral palsy formed the experimental group, while 30 healthy children served as controls. Both groups were given 24-hour PSG, and their sleep structures were compared and analyzed.Results:The incidence of sleep disorders in the children with cerebral palsy (55.4%) was significantly higher than among the healthy children (20.0%). The average sleep latency was significantly higher than among the healthy children, while the duration and the percentage of the rapid eye movement (REM) stage were significantly lower than among the healthy children. Total sleep time [(458.47±95.62)min], sleep efficiency [(74.26±13.63)%], duration of REM [(68.90±42.70)min] and REM percentage [(13.87±7.12)%] were all significantly lower for the children with severe cerebral palsy than for those with mild or moderate disorder. Their time to wake up after falling asleep was significantly longer. Moreover, the duration of REM and the REM percentage of children with dyskinetic cerebral palsy were significantly lower than for those with spastic cerebral palsy.Conclusions:The incidence of sleep disorders among children with cerebral palsy is higher than among healthy children. They have more difficulty in falling asleep and have a shorter REM stage. Children with severe cerebral palsy and involuntary cerebral palsy have more sleep problems.

Objective:To explore the clinical application and long-term safety of hydroxychloroquine sulfate (HCQ) in the treatment of rheumatic diseases.Methods:A multi-center cross-sectional study was conducted between August 2017 and August 2018 in a random sample of eleven medical institutions of rheumatology and immunology in China. Patients who took HCQ for more than 3 months were enrolled into this study. The cumulative dose and long-term side effects of HCQ were recorded. The changes of laboratory indexes before and after treatment with HCQ were analyzed. Categorical variables were presented with counts and proportions, and evaluated by Chi-square test. Continuous parametric data were presented as Mean±standard deviation, and evaluated by Student's t test or Mann-Whitney U test. P-values less than 0.05 were considered statistically significant. Results:A total of 886 patients with rheumatic diseases were enrolled into this study, including 505 cases with systemic lupus erythematosus (57.0%), 210 cases with rheumatoid arthritis (23.7%), 80 cases with Sj?gren's syndrome (9.0%), 57 cases with undifferentiated connective tissue disease (6.4%), 12 cases of systemic vasculitis (1.4%), 10 cases of mixed connective tissue disease (1.1%), 7 cases of myositis (0.8%) and 5 cases with systemic sclerosis (0.6%). The most common long-term side effects of HCQ was skin or mucous lesions (12.4%) and vision problems (8.0%). Other adverse reactions included problems of digestive system (3.0%), nervous system (2.1%), musculoskeletal system (1.1%) and cardiovascular system (0.9%). 140 cases (15.8%) had stopped taking HCQ during the treatment. More than half of them decided to stop taking medicine by themselves. Fifty-four patients (6.1%) stopped using HCQ due to side effects while 24 of them took it again, and another 12 patients (1.4%) stopped the drug due to remission of illness. Patients were divided into three groups according to the cumulative dose of HCQ: less than 500 g, 500-1 000 g and more than 1 000 g respectively. There was significant difference in the incidence of long-term side effects among the three groups ( χ2=6.382, P=0.041). The last group (more than 1 000 g) suffered the highest incidence of long-term adverse reactions (37.1%). No severe adverse drug reactions were observed in this study. Conclusion:Hydroxychloroquine is widely used in the treatment of rheumatic diseases. The incidence of long-term side effects is 20.4%, is 6.1% lead to drug withdrawal, which are especially related to the cumulative doses. It should be adjusted properly according to the clinical application.

Objective To investigate the role and mechanism of resveratrol in a rat model of complex region-al pain syndrome type Ⅰ(CRPS1). Methods 50 male SD rats were divided into control group(group A),sham operation group(group B),model group 1(group C),model group 2(group D),and model group 3(group E). Groups A,B and C received 5%DMSO;group D received ISO-1 of 1 mg/(kg·d);and group E received resveratrol of 20 mg/(kg · d)for 14 days. Pain behaviors were assessed on days 0,7,and 14. Serum levels of MIF and TNF-αwere detected by ELISA. MIF ,total ERK1/2 and p-ERK1/2 in sciatic nerve were detected by Western blot. Re-sults On days 7 and 14 after treatment,resveratrol injection,similar to ISO-1,significantly improved the pain threshold;serum levels of MIF and TNF-α were significantly decreased;expressions of MIF ,total ERK1/2 and p-ERK1/2 in sciatic nerve were also decreased significantly in group E,which were significantly lower than group C (P < 0.05). Conclusions Resveratrol can significantly improve pain threshold ,decrease expressions of MIF and p-ERK1/2 in a rat model of CRPS1,which might be involved in the inhibition of ERK signaling pathway.

Objective To evaluate the value and the expression of Axl receptor tyrosine kinase (AxlTK) in renal pathology of lupus nephritis (LN) patients. Methods The expression of AxlTK were detected in 29 LN and 10 primary nephritic syndrome (NS) patients using immunohistochemistry in renal tissue. The correlations between the levels of AxlTK on glomeruli and clinical manifestations, laboratory parameters, disease activity, and renal pathology in LN patients were investigated. T test and single factor analysis of variance were used tp compare between groups, and Pearson or Spearman test was used for correlation analysis. Results AxlTK was extensively expressed on surface of mesangial cells, endothelial cells in glomeruli. There was more expression of AxlTK on mesangial cells in LN patients [(7.0 ±2.2)×10-2] than that in primary NS patients [(3.1 ±1.2) ×10-2] (t=5.382, P<0.01). The expression of AxlTK in the glomeruli of LN patients with oral ulcers is higher than that in patients without of ulcers (t=2.259, P=0.032), and it was positively correlated with the anti-nucleosome antibody (AnuA) level in patients (r=0.488, P=0.017). The expression of AxlTK on glomeruli were higher in class Ⅳ and classⅢ of LN patients than that of class Ⅴ, but there was no statistically significant difference between them (P>0.05). Conclusion The expression of Axl TK in glomerular is significantly increased in glomeruli in LN patients. The expression level of AxlTK on glomeruli is correlated with the occurrence of oral ulcers and AnuA. The level of AxlTK expression is related to pathological categories of kidney in LN patients. The results of this study suggest that AxlTK might participate in the pathogenesis of LN.

Objective To explore the status of glucocorticoid application in patients with systemic lupus erythematosus (SLE) in China.Methods Epidemiological survey was used.The SLE patients who met the 1997 classification criteria of American College of Rheumatology were enrolled.The usage of glucocorticoid and related adverse reactions were recorded and analyzed.Results A total of 400 SLE patients were enrolled,including 35 men and 365 women.The average age was (37.4 ± 14.0) years old,and the average duration of disease was (6.7 ± 5.8) years.There were 310 patients using glucocorticoid as maintenance.Sixty-one percent (n =244) patients started using medium dose (prednisone 30-＜ 60 mg/d) as the initial treatment of glucocorticoid,which lasted for (37 ± 11) days.The time of drug duration in patients with low dose prednisone (7.5-＜30 mg/d)and high dose (60-100 mg/d) was(92 ± 20)and (17 ± 3) days respectively (P ＜ 0.05 between 3 groups).However,patients receiving different initial dosage were of no discrepancy in the maintenance therapy.During maintenance,even though 51.0％ (n =158) patients were on prednisone 2.5-5 mg/d,the duration of drug use in ＞ 5-10 mg/d groupwas longer [(29.9 ± 3.3) months].Patients with involvement of internal organs had a higher tendency to use 60-100 mg/d prednisone or pulse-dose therapy in the initial treatment,nevertheless these two groups had no difference of maintenance dosage.Among all 400 patients,62 patients withdrew glucocorticoid,including 17 patients with disease remission (4.3％),44 by self-withdrawal and one with adverse reaction.Conclusion In China,the medium dosage of glucocorticoid is the most common initial treatment in patients with SLE.Prednisone 2.5-5 mg/d was the most common choicefor maintenance therapy.Currently,the proportion of glucocorticoid withdrawal remains low in SLE patients achieving remission.