Background:Ziwan and Taoren(ZT)is a classic medicine pair in the formula of Mai Dong Di Shao Decoction,has been used to treat primary Sjogren’s syndrome(pSS)for more than 20 years.But its action mechanism is still un...Background:Ziwan and Taoren(ZT)is a classic medicine pair in the formula of Mai Dong Di Shao Decoction,has been used to treat primary Sjogren’s syndrome(pSS)for more than 20 years.But its action mechanism is still unknown.This study is aimed to reveal the potential mechanism of ZT treated pSS and discover its active compounds of ZT and therapeutic target for pSS.Methods:Firstly,the potential pathways of ZT for pSS treatment were predicted through network pharmacology and GO and KEGG enrichment analysis.Secondly,the inter-structural relationships between active compounds of ZT and target proteins were visualized using molecular docking techniques.Finally,efficacy and mechanism were conducted through in vivo experiments,such as water intake,spleen index,hematoxylin-eosin staining pathological changes,ELISA,Western Blot analysis,and immunofluorescence staining.Results:Nine active compounds were extracted from network pharmacology,including quercitrin,luteolin,kaempferol,β-sitosterol,isorhamnetin,galangin,hederagenin,diosmetin and gibberellin 7.Seven disease targets were identified:RELA,TP53,AKT1,interleukin(IL)6,MAPK1,ESR1,IL10;with RELA being the most core target.KEGG and GO enrichment analysis indicated that ZT may act through the TLR/NF-κB/RELA inflammatory mechanism process.preliminary results of molecular docking showed that ZT’s active compounds bind well to the RELA(p65)receptor.In vivo results demonstrated that a high dose of ZT significantly improved water intake and reduced lymphocytes infiltration in submandibular gland pathology in NOD mice.The expression content of AQP5 and vasoactive intestinal peptide in the submaxillary gland was significantly increased,while levels of inflammatory factors such as tumor necrosis factor-α,IL-6,and IL-1βalong with protein expressions including toll-like receptor4,p-p65 and p-IKKα/βin NF-κB pathway were reduced.Conclusions:The ZT treatment exhibits a promising efficacy in mitigating dryness symptoms of pSS,potentially attributed to its capacity for suppressing the TLR/NF-κB inflammatory signaling pathway.展开更多
Objective: To analyze the epidemiological characteristics of growth, as well as factors associated with growth retardation in children with primary nephrotic syndrome (PNS), and to investigate the effect of glucocorti...Objective: To analyze the epidemiological characteristics of growth, as well as factors associated with growth retardation in children with primary nephrotic syndrome (PNS), and to investigate the effect of glucocorticoid (GC) use duration on growth retardation in these children. Methods: Clinical and laboratory data of 353 PNS children treated at our hospital from July 2014 to June 2015 were collected through the medical record management system. Height, weight, and GC usage were recorded. Follow-up assessments were conducted in August 2022 for the original group, recording height, weight, and GC usage. Height and weight were evaluated using standard deviation scores (SDS). Categorical data were analyzed using chi-square test while continuous measurement data were analyzed using t-test or rank-sum test. Linear regression was used to assess the association between two single independent variables, and logistic regression analysis was used to screen for risk factors related to growth retardation in children with PNS. Results: Among the 353 PNS children enrolled in this study, male-to-female ratio of 2.64:1 (256 males vs 97 females). A total of 119 children exhibited growth retardation, incidence rate of 33.71%. The duration of GC usage among those with growth retardation was significantly longer compared to those without it (762.81 ± 934.50 days vs 263.77 ± 420.49 days;p Conclusion: PNS children treated with GC have a high incidence of growth retardation, and a high proportion of short stature in adulthood, especially in children with growth retardation in childhood, most of them have short stature after grown up. Time of GC usage is a risk factor for growth retardation in children with PNS.展开更多
Objective Primary Sjogren’s syndrome(pSS)is a systemic autoimmune disease that mainly affects the exocrine gland,especially in women.Currently,the results of studies on the menstruation or fertility of pSS patients r...Objective Primary Sjogren’s syndrome(pSS)is a systemic autoimmune disease that mainly affects the exocrine gland,especially in women.Currently,the results of studies on the menstruation or fertility of pSS patients remain controversial.This study aimed to examine the menstrual and reproductive characteristics of pSS patients.Methods Clinical data of 449 pSS patients who were admitted to Tongji Hospital in Hubei,China,from January 2015 to November 2021 were obtained and their menstrual and reproductive information analyzed.In addition,the clinical features of pSS patients with premenopausal or postmenopausal onset were compared.Results The spontaneous abortion rate of pSS patients was not higher than the reported rate of the general population and that the age of menarche,menstrual cycle,and menstrual period of pSS patients did not significantly differ from those reported in the general population;however,early menopause seemed to be more common in pSS patients.Skin involvement(27.96%vs.15.00%,P=0.005)and hyperglobulinemia(10.64%vs.4.16%,P=0.033)were more common in patients with premenopausal pSS onset,but patients with postmenopausal onset had a significantly greater incidence of interstitial lung disease(32.50%vs.17.02%,P=0.0004).Also,erythropenia(47.00%vs.31.25%,P=0.002),hypoalbuminemia(19.49%vs.8.22%,P=0.0009),and prevalence of high hypersensitive C-reactive protein levels(21.67%vs.10.94%,P=0.005)were more common in pSS patients with postmenopausal onset.Notably,the rate of abnormal pregnancy was significantly greater in patients with premenopausal onset(9.72%vs.2.50%,P=0.011).Conclusion Patients with pSS onset before or after menopause may have different risks in pulmonary involvement and laboratory manifestations.展开更多
BACKGROUND Primary membranous nephrotic syndrome with chylothorax as the first manifestation is an unusual condition.To date,only a few cases have been reported in clinical practice.CASE SUMMARY The clinical data of a...BACKGROUND Primary membranous nephrotic syndrome with chylothorax as the first manifestation is an unusual condition.To date,only a few cases have been reported in clinical practice.CASE SUMMARY The clinical data of a 48-year-old man with primary nephrotic syndrome combined with chylothorax admitted to the Department of Respiratory and Critical Care Medicine of Shaanxi Provincial People's Hospital were retrospec-tively analysed.The patient was admitted to the hospital for 12 d due to shortness of breath.Imaging showed pleural effusion,laboratory tests confirmed true chylothorax,and renal biopsy revealed membranous nephropathy.After primary disease treatment and early active symptom treatment,the prognosis of the patient was good.This case suggests that chylothorax is a rare complication of primary membranous nephrotic syndrome in adults,and early lymphan-giography and renal biopsy can assist in the diagnosis when there are no contrain-dications.CONCLUSION Primary membranous nephrotic syndrome combined with chylothorax is rare in clinical practice.We report a relevant case to provide case information for clinicians and to improve diagnosis and treatment.展开更多
Objective: To explore better therapy and reduce the rate of re-relapse of primary nephritic syndrome in children who had been treated with corticosteroids but relapsed. Methods: Eighty relapsers were enrolled from Jan...Objective: To explore better therapy and reduce the rate of re-relapse of primary nephritic syndrome in children who had been treated with corticosteroids but relapsed. Methods: Eighty relapsers were enrolled from Jan. 1994 to Apr. 2000, who were randomly divided into two groups. The treatment group (n=39) had been treated with tripterysium glucosides for three months,with the control group (n=41) members were treated with cyclophosphmide (CTX) by intermission intravenous pulse, with total dose of CTX not being more than 150 mg/kg. Prednisone, meanwhile, was given to both groups. The total treatment period of prednisone was prolonged by 12-18 months. Results: After following up for 3-7 years, the re-relapse rates of both groups were observed. The re-relapse rate of the treatment group was 28.2% to 29.3% in the CTX-controlled group. The re-relapse rates between two groups were almost similar, and with no observed significant difference (P>0.05). The side effect of tripterysium glucosides was less than that of CTX. Conclusion: For the treatment of relapsing nephritic syndrome in children, the combination of tripterysium glucosides and prolonged corticosteroid therapy is as effective as the regimen of CTX plus prolonged use of prednisone.展开更多
Objective To evaluate the sensitivity and specificity of international classification criteria (2002) for primary Sjogren's syndrome (pSS) and the role of lower lip biopsy in diagnosis of pSS in Chinese patients....Objective To evaluate the sensitivity and specificity of international classification criteria (2002) for primary Sjogren's syndrome (pSS) and the role of lower lip biopsy in diagnosis of pSS in Chinese patients. Mothoda Patients who were diagnosed by the experts/rheumatologists as pSS during 1990-2002 from the Department of Rheumatology, Peking Union Medical College Hospital were retrospectively collected as experimental group. Patients who were diagnosed as non-pSS connective tissue diseases or non-connective tissue diseases served as control group. Those with a history of head-neck radiation, hepatitis C virus infection, AIDS, lymphoma, sarcoidosis, graft versus host disease (GVHD), and anti-acetylcholine drug use were exempted. Both groups were required to complete questionnaires about symptoms such as dry eyes and dry mouth, and complete the objective tests of keratoconjunctivitis and xerostomia including Schirmer test, corneal staining, unstimulated salivary flow, sialography, lower lip biopsy, and antinuclear antibodies (including anti-SSA/SSB antibodies) test. Results A total of 330 pSS patients were included in experimental group and 185 non-pSS patients in control group. The mean age of both groups matched (47.8 ± 10.9 years vs. 46.2±13.6 years, P 〉 0.05). The sensitivities of the criteria in pSS patients with lower lip biopsy and in pSS patients without lower lip biopsy were 89.2% and 87.2%, respectively; the overall sensitivity was 88.5%. The specificity was 97.3%. A total of 11.3% pSS patients with negative anti-SSA/SSB antibodies were diagnosed as pSS by lower lip biopsy. Coadwion The international classification criteria (2002) for pSS is feasible in Chinese patients. It has high sensitivity and specificity, and may serve as diagnosis criteria in routine clinical practice.展开更多
AIM:To determine the clinical features,diagnosis and treatment of the primary Sjogren syndrome(SS)related optic neuritis.METHODS:The clinical data of 8 patients(12 eyes)with primary SS related optic neuritis were anal...AIM:To determine the clinical features,diagnosis and treatment of the primary Sjogren syndrome(SS)related optic neuritis.METHODS:The clinical data of 8 patients(12 eyes)with primary SS related optic neuritis were analyzed retrospectively.RESULTS:Eight of 128 consecutive patients with optic neuritis resulted from varied causes fulfilled the diagnostic criteria for the primary SS.They presented initially with the signs and symptoms of non-specific optic neuritis,and 5 patients presenting without dryness showed a chronic inflammation of submandibular gland or parotid gland,and lymphocyte infiltration was demonstrated by labial gland biopsy in 2 patients.There were serum positive titers for anti-Sjogren syndrome A(SSA)in 7 patients and anti-Sjogren syndrome B(SSB)in 8 patients.Anti-aquaporin-4(AQP4)antibody was negative in all the 8 patients.Both glucocorticoids and immunosuppressive agent were administered,and visual acuity elevated in 8 eyes(66.7%),3 patients(37.5%)recurred in the follow-up.CONCLUSION:Primary SS related optic neuritis is less common and easily misdiagnosed.The conventional therapies for optic neuritis could not control the recurrence.展开更多
Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with (n = 6) or without primary Sjogr...Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with (n = 6) or without primary Sjogreffs syndrome (n = 10). All patients underwent extensive clinical, laboratory, and MRI evaluations. There were no statistical differences in demographics or first neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sjogren's syndrome. The laboratory findings of cerebrospinal fluid oligoclonal banding, serum C-reactive protein, antinudear autoantibody, anti-Sjogren's-syndrome-related antigen A an- tibodies, anti-Sjogren's-syndrome-related antigen B antibodies, and anti-Sm antibodies were significantly higher in patients with primary Sjogren's syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% (4/6) of patients with primary Sjogren's syndrome and in 60% (6/10) of patients without primary Sj6gren's syndrome. More brain abnormalities were observed in patients without primary Sj6gren's syndrome than in those with primary Sj6gren's syndrome. Segments lesions (〉 3 centrum) were noted in 50% (5/10) of patients without primary Sj6gren's syndrome and in 67% (4/6) of patients with primary Sjogren's syndrome. These findings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sjogren's syndrome are similar. However, neu- romyelitis optica spectrum disorder patients without primary Sjogreffs syndrome have a high frequency of brain abnormalities.展开更多
BACKGROUND Primary Sjögren's syndrome(pSS)concomitant with autoimmune hemolytic anemia(AIHA)but without eye and mouth dryness is exceedingly rare.Iguratimod(IGU)has been widely used in the treatment of pSS.Ho...BACKGROUND Primary Sjögren's syndrome(pSS)concomitant with autoimmune hemolytic anemia(AIHA)but without eye and mouth dryness is exceedingly rare.Iguratimod(IGU)has been widely used in the treatment of pSS.However,there are few reports about the application of IGU in pSS concomitant with AIHA.CASE SUMMARY Here,we present the case of a patient with pSS concomitant with AIHA but without eye and mouth dryness.The patient was initially diagnosed with hyperplastic anemia and AIHA while pSS was missed,and was finally diagnosed with pSS concomitant with AIHA.The patient was treated with IGU along with prednisone and hydroxychloroquine,and her hemoglobin,reticulocytes and IgG returned to normal levels.CONCLUSION IGU was effective for and well tolerated by our patient with pSS concomitant with AIHA,and may be a promising therapy for the treatment of this disease.展开更多
Objective:To investigate the TCM treatment principle of membranous nephropathy and its effect.Methods:A total of 56 patients were selected from the Affiliated Hospital of Shaanxi University of Traditional Chinese Medi...Objective:To investigate the TCM treatment principle of membranous nephropathy and its effect.Methods:A total of 56 patients were selected from the Affiliated Hospital of Shaanxi University of Traditional Chinese Medicine.They were then divided into the control group(western medicine standard therapy)and the study group(Qidi Gushen prescription),with 28 patients in each group.The treatment effect,treatment safety,and patients’satisfaction were observed and compared between the two groups.Results:The results showed that the treatment effect of the study group was 96.43%,which was significantly better compared with the control group(75.00%)(p<0.05);in terms of safety,the probability of adverse events was 7.14%in the study group and 32.14%in the control group,in which the difference was statistically significant(p<0.05);in addition,the study group’s satisfaction with the treatment measures was significantly higher than that of the control group(p<0.05).Conclusion:In treating membranous nephropathy,traditional Chinese medicine can be tailored to its pathogenesis,which is not only beneficial to the treatment effect,but also has a high safety profile.展开更多
The recognition of the profound impact of the human gastrointestinal microbiome(GM) on human autoimmune diseases has gradually increased thanks to deeper research efforts. As a systemic autoimmune disease, primary Sjo...The recognition of the profound impact of the human gastrointestinal microbiome(GM) on human autoimmune diseases has gradually increased thanks to deeper research efforts. As a systemic autoimmune disease, primary Sjogren’s syndrome(pSS) cannot be completely cured. Human studies have revealed that GM species and diversity are altered in patients with p SS compared with healthy individuals. Animal studies have provided possible mechanisms for the association between pSS and GM. The potential role of GM in pSS is exerted through several mechanisms. GM dysbiosis leads to increased intestinal permeability, which increases the risk of GM antigen exposure and activates specific autoreactive T lymphocytes via “molecular mimicry”. In addition, GM antigen exposure and intestinal immune tolerance loss caused by GM dysbiosis together induce chronic local gut mucosal inflammation, which deteriorates to systemic chronic non-specific inflammation with the circulation of pro-inflammatory lymphocytes and cytokines. These factors eventually activate autoreactive B lymphocytes and lead to pSS. If GM plays a key role in the pathogenesis of pSS, clarifying the underlying mechanisms will be helpful for the development of new therapies targeting GM for dry eye associated with pSS. This review summarizes the latest knowledge about the relationship between GM and p SS,with the aim of contributing to future research and to the development of new clinical applications.展开更多
[Objectives]To observe the clinical efficacy of Shenling Baizhu Powder in the treatment of primary nephrotic syndrome(PNS)featuring damp abundance due to splenic asthenia.[Methods]40 patients with PNS were randomly di...[Objectives]To observe the clinical efficacy of Shenling Baizhu Powder in the treatment of primary nephrotic syndrome(PNS)featuring damp abundance due to splenic asthenia.[Methods]40 patients with PNS were randomly divided into treatment group(n=19)and control group(n=21).In the treatment group and the control group,methylprednisolone tablets were taken orally and cytoxan(CTX)was given intravenously for treatment;in the treatment group,based on oral administration of methylprednisolone tablets and intravenous drip of cytoxan(CTX),oral administration of traditional Chinese medicine(addition and subtraction of Shenling Baizhu Powder)was added.Urine routine,24-hour urinary protein,renal function and serum albumin were re-examined after 4 weeks of treatment.[Results]The total effective rate of the treatment group was significantly higher than that of the control group(89.47%vs 61.90%,P<0.05).After treatment,both groups could effectively reduce the levels of 24-hour urinary protein(P<0.05),serum creatinine(P<0.05)and urea nitrogen(P<0.05),and increase the level of serum albumin(P<0.05),but the degree of improvement in the treatment group was significantly higher than that in the control group(P<0.05),and the difference was statistically significant(P<0.05).[Conclusions]Methylprednisolone combined with addition and subtraction of Shenling Baizhu Powder could effectively reduce urinary protein,improve edema symptoms,shorten the course of hormone use and reduce adverse reactions in the treatment of PNS featuring damp abundance due to splenic asthenia.展开更多
<div style="text-align:justify;"> <span style="font-family:Verdana;"><strong>Background:</strong> The association of primary Sj<span style="white-space:nowrap;"...<div style="text-align:justify;"> <span style="font-family:Verdana;"><strong>Background:</strong> The association of primary Sj<span style="white-space:nowrap;">ö</span>gren syndrome (PSS) and AA amyloidosis is a rare occurrence. <strong>Objective: </strong>To describe the phenotype of patients with this association through our two cases and a literature review. <strong>Materials and methods:</strong> A report of two cases of AA amyloidosis complicating primary Sj<span style="white-space:nowrap;">ö</span>gren syndrome with a literature review. <strong>Results:</strong> Eight patients of Primary Sj<span style="white-space:nowrap;">ö</span>gren’s Syndrome complicated by AA amyloidosis were studies. Six cases were reported in the literature by consulting several databases. 50% of patients had a positive immunological assessment, three cases with kidney damage, and three cases lung damage. <strong>Conclusion: </strong>The immunological activity in the Primary Sjogren’s Syndrome requires the search not only a lymphoma but also AA amyloidosis apart from any clinical or biological chronic inflammation.</span> </div>展开更多
Antiphospholipid syndrome (APS) is a prothrombotic condition that can affect both the venous circulation and the arterial system. The deep veins of the lower extremities and cerebral blood flow are the most common sit...Antiphospholipid syndrome (APS) is a prothrombotic condition that can affect both the venous circulation and the arterial system. The deep veins of the lower extremities and cerebral blood flow are the most common sites of venous and arterial thrombosis, respectively. Skin ulceration and gangrene may be associated with an active vasculitis in patients with APS. These kinds of ulcers are considered intractable because healing is difficult to achieve. Using steroids as immunesuppressant therapy is still a basic part of managing APS and vasculitis. Nevertheless, in the presence of steroid-induced diabetes mellitus, the priority is to achieve faster wound healing, because of the potential complications that can develop due to metabolic dysfunction and augmented vulnerability to infection. Until recently, there were few reports demonstrating the benefits of the use of adjuvant phosphodiesterase-5 inhibitors like sildenafil citrate in different clinical entities with courses like Raynaud’s phenomenon, scleroderma, and resistance to vasodilator therapy. We present the first report demonstrating the additional benefit of sildenafil citrate for the integration of grafts in a patient with steroid-induced diabetes and APS who had ulcers due to vasculitis resistant to the usual vasodilator therapy.展开更多
Backgrounds: To observe the effects of aerobic exercise program on the illness symptoms and cardiopulmonary capacity of the patients with primary Fibromyalgia Syndrome (FMS) and to find out whether there is a differen...Backgrounds: To observe the effects of aerobic exercise program on the illness symptoms and cardiopulmonary capacity of the patients with primary Fibromyalgia Syndrome (FMS) and to find out whether there is a difference or not in terms of cardiovascular capacity among the patients with primary FMS and sedentary healthy people. Materials and Methods: The study has been done on 20 female patients with primary FMS. Before the study and after a 6-week controlled aerobic exercise program, a cardiopulmonary exercise test has been applied on all cases. The symptoms, psychological state and life quality of the cases have been evaluated before and after the exercise program. The first cardiopulmonary results of the cases with FMS have been compared with a control group of 15 sedentary healthy. Results: When the patient and control groups are compared, although maksimal oxygen uptake (VO2 max) was lower in the patient group, this was not a significant difference. VO2 max showing the aerobic condition after the exercise increased significantly in the patients. Conclusions: Aerobic exercise programs lasting six to eight weeks are quite effective for the patients with FMS. There is not any limitation in the increase of the cardiopulmonary capacity of the patients after the exercise program.展开更多
Background:Understanding the clinical implications of autoimmune manifestations associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is essential to reduce its consequences. This study was aime...Background:Understanding the clinical implications of autoimmune manifestations associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is essential to reduce its consequences. This study was aimed at determining the activities of new-onset primary Sjögren syndrome (pSS) since the emergence of SARS-CoV-2.Methods:This retrospective cohort study included data from 471 participants with dry mouths and eyes who had been attending Nanjing Drum Tower Hospital since December 2019. By April 2023, patients diagnosed with pSS were sequentially assigned to vaccinated group ( n = 24) or vaccinated and infected group based on exposure ( n = 20). Patients diagnosed with pSS within 3 months of vaccination against SARS-CoV-2 were assigned to a vaccinated group, and those who had been vaccinated and then developed pSS within 3 months of follow up after direct exposure to SARS-CoV-2 were assigned to a vaccinated and infected group. The controls comprised age- and sex-matched patients who had not been exposed to SARS-CoV-2 before December 2019 ( n = 21). We then compared data among the three groups. Results:The vaccinated and infected patients had more fever, malaise, splenomegaly, and weight loss before diagnosis and a higher European Alliance of Associations for Rheumatology Sjögren's syndrome disease activity index at the time of onset than the other two groups. Vaccinated patients had a higher frequency of anti-nuclear antibody (ANA) titers > 1:320 ( n = 12;50%) and anti-phospholipid antibodies (aPL) ( n = 7;29%) than the controls. The frequency of anti-Ro/SSA antibodies (13, 65%), ANA titers > 1:320 ( n = 16;80%), and aPLs ( n = 7;29%) ( n = 5;25%) were all significantly higher in vaccinated patients with infection than those in the controls. Higher doses of glucocorticoids, cyclosporin A, and tacrolimus were administered to the vaccinated and infected than the vaccinated and control groups ( p < 0.05, for all). Conclusions:Patients with new-onset pSS and a history of vaccination and SARS-CoV-2 infection might have more active disease. Further strengthening the assessment of people with a clear history of SARS-CoV infection and the monitoring of potential populations for autoimmune screening should not be overlooked.展开更多
Objective: To observe the relationship of tumor necrosis factor-o (TNF-a) and nitrogen oxide (NO) with the treatment of frequent relapse nephrotic syndrome (FRNS) and to explore the patho-genesis of FRNS and the thera...Objective: To observe the relationship of tumor necrosis factor-o (TNF-a) and nitrogen oxide (NO) with the treatment of frequent relapse nephrotic syndrome (FRNS) and to explore the patho-genesis of FRNS and the therapeutic mechanism of Shenkangling (肾康灵,SKL) Granule in children. Methods: Sixty children suffering from FRNS were randomly divided into the treated group and control group, 30 in each, and the other 30 healthy children were taken as healthy group. The patients were treated with prednisone for a long-term course, and those with no effect or partial effect shown were treated with additional Tripterygium or Cytoxan in the control group, while in the treated group patients were treated with prednisone and additional SKL. The two groups were compared as to their changes of TNF-a, NO before and after treatment, and the relapses after treatment. Results: The levels of TNF-a and NO in the sick children before treatment were markedly higher than those after treatment and normal group (P< 0. 01). The positive correlation between TNF-o of FRNS cases and relapse risk displayed more significance than that between the relapse of FRNS and NO. The difference between treated group and control group was significant (P<0. 01). Conclusion: TNF-a can be regarded as the monitoring parameter of the active phase in FRNS, and the higher the level, the more possible the relapse would occur. SKL could markedly reduce the relapse rate of FRNS in children.展开更多
Primary Sjögren’s syndrome(pSS)is a systemic autoimmune disease with high prevalence and possible poor prognosis.Though the pathogenesis of pSS has not been fully elucidated,B cell hyperactivity is considered as...Primary Sjögren’s syndrome(pSS)is a systemic autoimmune disease with high prevalence and possible poor prognosis.Though the pathogenesis of pSS has not been fully elucidated,B cell hyperactivity is considered as one of the fundamental abnormalities in pSS patients.It has long been identified that Janus kinases-signal transducer and activator of transcription(JAK-STAT)signaling pathway contributes to rheumatoid arthritis and systemic lupus erythematosus.Recently,increasing numbers of studies have provided evidence that JAK-STAT pathway also has an important role in the pathogenesis of pSS via direct or indirect activation of B cells.Signal transducer and activator of transcription 1(STAT1),STAT3,and STAT5 activated by various cytokines and ribonucleic acid contribute to pSS development,respectively or synergically.These results reveal the potential application of Janus kinase inhibitors for treatment of pSS,which may fundamentally improve the quality of life and prognosis of patients with pSS.展开更多
Objective:To investigate the effect of prostaglandin E1 (PGE1) (Alprostadii injection) on patients with primary nephrotic syndrome(PNS). Methods: 37 patients with PNS were recruited to study the effect of prostaglandi...Objective:To investigate the effect of prostaglandin E1 (PGE1) (Alprostadii injection) on patients with primary nephrotic syndrome(PNS). Methods: 37 patients with PNS were recruited to study the effect of prostaglandin E1 on platelet aggregation function [ PAG (5,) PAG( m ) ], serum total protein (TP) , albumin (Al),blood urea nitrogen(BUN) ,serum creatinine(Scr) ,cholesterol(CHO), triglyceride(TG), protein in 24-hour urine (Pr/24h) and platelet account (PLT). Results: TP, Al, CHO, TG, BUN, Scr, Pr/24h, PAG(5) and PAG(m) in PNS group before treatment were significantly different from those in control group(P<0.05, P<0.01) while no significant difference was found for PLT. When treated with PGE1 , TP,Al,CHO, TG, Pr/24h, ADP- induced PAG(5) ,and Adr- induced PAG(5) and PAG(m) were significantly different from those before treatment (P<0.05). Adr- induced PAG(5) and PAG(m) were significantly different. Adr- induced PAG(5) was xsitively correlated with BUN and Scr in PNS(P<0.01). Similar correlation was found between ADP-induced PAG(5) and Al ,BUN,Scr,Pr/24h(P<0.05), AD- induced PAG(m) and TP,CHO(P<0.05). Conclusions: PGE1 may be an effective drug for the treatment for hypercoagulation in patients with PNS.展开更多
Objective: To provide an objective reference for the syndrome types of Chinese medicine(CM) associated with pediatric primary nephrotic syndrome(PNS).Methods: A cross-sectional study was performed.Data on clinic...Objective: To provide an objective reference for the syndrome types of Chinese medicine(CM) associated with pediatric primary nephrotic syndrome(PNS).Methods: A cross-sectional study was performed.Data on clinical symptoms,CM syndrome types,biochemical indices,and medications used were collected from 98 children with PNS.Then,the correlation between CM syndromes and biochemical indices,as well as medications used,was analyzed.Results: The four most common symptoms in children with PNS were brown urine,red tongue,excessive sweating,and swelling of the face and limbs.The syndromes of qi deficiency of Fei(Lung) and Shen(Kidney)(FSQD) and yin deficiency of Gan(Liver) and Shen(GSYD) were the most common main CM syndrome types.FSQD syndrome score correlated significantly with the total cholesterol level,urine protein/creatinine ratio,and urine Ig G and albumin levels(P〈0.01 or P〈0.05).The use of maintenance glucocorticoids combined with immunosuppressive agents correlated with FSQD syndrome,and the use of maintenance glucocorticoids alone correlated with GSYD syndrome(P〈0.05).Conclusion: Two of the most common CM syndrome types were FSQD and GSYD syndromes.FSQD syndrome may be caused by some factors related to lipid levels,protein loss,and the use of immunosuppressive agents.The use of maintenance glucocorticoids may cause GSYD syndrome.展开更多
基金grants from the National Natural Science Foundation of China(82274454).
文摘Background:Ziwan and Taoren(ZT)is a classic medicine pair in the formula of Mai Dong Di Shao Decoction,has been used to treat primary Sjogren’s syndrome(pSS)for more than 20 years.But its action mechanism is still unknown.This study is aimed to reveal the potential mechanism of ZT treated pSS and discover its active compounds of ZT and therapeutic target for pSS.Methods:Firstly,the potential pathways of ZT for pSS treatment were predicted through network pharmacology and GO and KEGG enrichment analysis.Secondly,the inter-structural relationships between active compounds of ZT and target proteins were visualized using molecular docking techniques.Finally,efficacy and mechanism were conducted through in vivo experiments,such as water intake,spleen index,hematoxylin-eosin staining pathological changes,ELISA,Western Blot analysis,and immunofluorescence staining.Results:Nine active compounds were extracted from network pharmacology,including quercitrin,luteolin,kaempferol,β-sitosterol,isorhamnetin,galangin,hederagenin,diosmetin and gibberellin 7.Seven disease targets were identified:RELA,TP53,AKT1,interleukin(IL)6,MAPK1,ESR1,IL10;with RELA being the most core target.KEGG and GO enrichment analysis indicated that ZT may act through the TLR/NF-κB/RELA inflammatory mechanism process.preliminary results of molecular docking showed that ZT’s active compounds bind well to the RELA(p65)receptor.In vivo results demonstrated that a high dose of ZT significantly improved water intake and reduced lymphocytes infiltration in submandibular gland pathology in NOD mice.The expression content of AQP5 and vasoactive intestinal peptide in the submaxillary gland was significantly increased,while levels of inflammatory factors such as tumor necrosis factor-α,IL-6,and IL-1βalong with protein expressions including toll-like receptor4,p-p65 and p-IKKα/βin NF-κB pathway were reduced.Conclusions:The ZT treatment exhibits a promising efficacy in mitigating dryness symptoms of pSS,potentially attributed to its capacity for suppressing the TLR/NF-κB inflammatory signaling pathway.
文摘Objective: To analyze the epidemiological characteristics of growth, as well as factors associated with growth retardation in children with primary nephrotic syndrome (PNS), and to investigate the effect of glucocorticoid (GC) use duration on growth retardation in these children. Methods: Clinical and laboratory data of 353 PNS children treated at our hospital from July 2014 to June 2015 were collected through the medical record management system. Height, weight, and GC usage were recorded. Follow-up assessments were conducted in August 2022 for the original group, recording height, weight, and GC usage. Height and weight were evaluated using standard deviation scores (SDS). Categorical data were analyzed using chi-square test while continuous measurement data were analyzed using t-test or rank-sum test. Linear regression was used to assess the association between two single independent variables, and logistic regression analysis was used to screen for risk factors related to growth retardation in children with PNS. Results: Among the 353 PNS children enrolled in this study, male-to-female ratio of 2.64:1 (256 males vs 97 females). A total of 119 children exhibited growth retardation, incidence rate of 33.71%. The duration of GC usage among those with growth retardation was significantly longer compared to those without it (762.81 ± 934.50 days vs 263.77 ± 420.49 days;p Conclusion: PNS children treated with GC have a high incidence of growth retardation, and a high proportion of short stature in adulthood, especially in children with growth retardation in childhood, most of them have short stature after grown up. Time of GC usage is a risk factor for growth retardation in children with PNS.
文摘Objective Primary Sjogren’s syndrome(pSS)is a systemic autoimmune disease that mainly affects the exocrine gland,especially in women.Currently,the results of studies on the menstruation or fertility of pSS patients remain controversial.This study aimed to examine the menstrual and reproductive characteristics of pSS patients.Methods Clinical data of 449 pSS patients who were admitted to Tongji Hospital in Hubei,China,from January 2015 to November 2021 were obtained and their menstrual and reproductive information analyzed.In addition,the clinical features of pSS patients with premenopausal or postmenopausal onset were compared.Results The spontaneous abortion rate of pSS patients was not higher than the reported rate of the general population and that the age of menarche,menstrual cycle,and menstrual period of pSS patients did not significantly differ from those reported in the general population;however,early menopause seemed to be more common in pSS patients.Skin involvement(27.96%vs.15.00%,P=0.005)and hyperglobulinemia(10.64%vs.4.16%,P=0.033)were more common in patients with premenopausal pSS onset,but patients with postmenopausal onset had a significantly greater incidence of interstitial lung disease(32.50%vs.17.02%,P=0.0004).Also,erythropenia(47.00%vs.31.25%,P=0.002),hypoalbuminemia(19.49%vs.8.22%,P=0.0009),and prevalence of high hypersensitive C-reactive protein levels(21.67%vs.10.94%,P=0.005)were more common in pSS patients with postmenopausal onset.Notably,the rate of abnormal pregnancy was significantly greater in patients with premenopausal onset(9.72%vs.2.50%,P=0.011).Conclusion Patients with pSS onset before or after menopause may have different risks in pulmonary involvement and laboratory manifestations.
基金Supported by the Shaanxi Provincial People’s Hospital Science and Technology Development Incubation Fund Project,No.2021YJY-33.
文摘BACKGROUND Primary membranous nephrotic syndrome with chylothorax as the first manifestation is an unusual condition.To date,only a few cases have been reported in clinical practice.CASE SUMMARY The clinical data of a 48-year-old man with primary nephrotic syndrome combined with chylothorax admitted to the Department of Respiratory and Critical Care Medicine of Shaanxi Provincial People's Hospital were retrospec-tively analysed.The patient was admitted to the hospital for 12 d due to shortness of breath.Imaging showed pleural effusion,laboratory tests confirmed true chylothorax,and renal biopsy revealed membranous nephropathy.After primary disease treatment and early active symptom treatment,the prognosis of the patient was good.This case suggests that chylothorax is a rare complication of primary membranous nephrotic syndrome in adults,and early lymphan-giography and renal biopsy can assist in the diagnosis when there are no contrain-dications.CONCLUSION Primary membranous nephrotic syndrome combined with chylothorax is rare in clinical practice.We report a relevant case to provide case information for clinicians and to improve diagnosis and treatment.
文摘Objective: To explore better therapy and reduce the rate of re-relapse of primary nephritic syndrome in children who had been treated with corticosteroids but relapsed. Methods: Eighty relapsers were enrolled from Jan. 1994 to Apr. 2000, who were randomly divided into two groups. The treatment group (n=39) had been treated with tripterysium glucosides for three months,with the control group (n=41) members were treated with cyclophosphmide (CTX) by intermission intravenous pulse, with total dose of CTX not being more than 150 mg/kg. Prednisone, meanwhile, was given to both groups. The total treatment period of prednisone was prolonged by 12-18 months. Results: After following up for 3-7 years, the re-relapse rates of both groups were observed. The re-relapse rate of the treatment group was 28.2% to 29.3% in the CTX-controlled group. The re-relapse rates between two groups were almost similar, and with no observed significant difference (P>0.05). The side effect of tripterysium glucosides was less than that of CTX. Conclusion: For the treatment of relapsing nephritic syndrome in children, the combination of tripterysium glucosides and prolonged corticosteroid therapy is as effective as the regimen of CTX plus prolonged use of prednisone.
基金Supported by the National Natural Sciences Foundation of China(30300164 ).
文摘Objective To evaluate the sensitivity and specificity of international classification criteria (2002) for primary Sjogren's syndrome (pSS) and the role of lower lip biopsy in diagnosis of pSS in Chinese patients. Mothoda Patients who were diagnosed by the experts/rheumatologists as pSS during 1990-2002 from the Department of Rheumatology, Peking Union Medical College Hospital were retrospectively collected as experimental group. Patients who were diagnosed as non-pSS connective tissue diseases or non-connective tissue diseases served as control group. Those with a history of head-neck radiation, hepatitis C virus infection, AIDS, lymphoma, sarcoidosis, graft versus host disease (GVHD), and anti-acetylcholine drug use were exempted. Both groups were required to complete questionnaires about symptoms such as dry eyes and dry mouth, and complete the objective tests of keratoconjunctivitis and xerostomia including Schirmer test, corneal staining, unstimulated salivary flow, sialography, lower lip biopsy, and antinuclear antibodies (including anti-SSA/SSB antibodies) test. Results A total of 330 pSS patients were included in experimental group and 185 non-pSS patients in control group. The mean age of both groups matched (47.8 ± 10.9 years vs. 46.2±13.6 years, P 〉 0.05). The sensitivities of the criteria in pSS patients with lower lip biopsy and in pSS patients without lower lip biopsy were 89.2% and 87.2%, respectively; the overall sensitivity was 88.5%. The specificity was 97.3%. A total of 11.3% pSS patients with negative anti-SSA/SSB antibodies were diagnosed as pSS by lower lip biopsy. Coadwion The international classification criteria (2002) for pSS is feasible in Chinese patients. It has high sensitivity and specificity, and may serve as diagnosis criteria in routine clinical practice.
基金The 12~(th)Five-Year Plan National Science and Technology Support Program,China(No.2012BAI08B06)
文摘AIM:To determine the clinical features,diagnosis and treatment of the primary Sjogren syndrome(SS)related optic neuritis.METHODS:The clinical data of 8 patients(12 eyes)with primary SS related optic neuritis were analyzed retrospectively.RESULTS:Eight of 128 consecutive patients with optic neuritis resulted from varied causes fulfilled the diagnostic criteria for the primary SS.They presented initially with the signs and symptoms of non-specific optic neuritis,and 5 patients presenting without dryness showed a chronic inflammation of submandibular gland or parotid gland,and lymphocyte infiltration was demonstrated by labial gland biopsy in 2 patients.There were serum positive titers for anti-Sjogren syndrome A(SSA)in 7 patients and anti-Sjogren syndrome B(SSB)in 8 patients.Anti-aquaporin-4(AQP4)antibody was negative in all the 8 patients.Both glucocorticoids and immunosuppressive agent were administered,and visual acuity elevated in 8 eyes(66.7%),3 patients(37.5%)recurred in the follow-up.CONCLUSION:Primary SS related optic neuritis is less common and easily misdiagnosed.The conventional therapies for optic neuritis could not control the recurrence.
文摘Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with (n = 6) or without primary Sjogreffs syndrome (n = 10). All patients underwent extensive clinical, laboratory, and MRI evaluations. There were no statistical differences in demographics or first neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sjogren's syndrome. The laboratory findings of cerebrospinal fluid oligoclonal banding, serum C-reactive protein, antinudear autoantibody, anti-Sjogren's-syndrome-related antigen A an- tibodies, anti-Sjogren's-syndrome-related antigen B antibodies, and anti-Sm antibodies were significantly higher in patients with primary Sjogren's syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% (4/6) of patients with primary Sjogren's syndrome and in 60% (6/10) of patients without primary Sj6gren's syndrome. More brain abnormalities were observed in patients without primary Sj6gren's syndrome than in those with primary Sj6gren's syndrome. Segments lesions (〉 3 centrum) were noted in 50% (5/10) of patients without primary Sj6gren's syndrome and in 67% (4/6) of patients with primary Sjogren's syndrome. These findings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sjogren's syndrome are similar. However, neu- romyelitis optica spectrum disorder patients without primary Sjogreffs syndrome have a high frequency of brain abnormalities.
基金Supported by the Lanzhou Science and Technology Plan Project,No.2018-3-48Lanzhou Talent Innovation and Entrepreneurship Project,No.2019-RC-35and 2019 Special Fund for Doctoral Training of Lanzhou University Second Hospital,No.YJS-BD-15.
文摘BACKGROUND Primary Sjögren's syndrome(pSS)concomitant with autoimmune hemolytic anemia(AIHA)but without eye and mouth dryness is exceedingly rare.Iguratimod(IGU)has been widely used in the treatment of pSS.However,there are few reports about the application of IGU in pSS concomitant with AIHA.CASE SUMMARY Here,we present the case of a patient with pSS concomitant with AIHA but without eye and mouth dryness.The patient was initially diagnosed with hyperplastic anemia and AIHA while pSS was missed,and was finally diagnosed with pSS concomitant with AIHA.The patient was treated with IGU along with prednisone and hydroxychloroquine,and her hemoglobin,reticulocytes and IgG returned to normal levels.CONCLUSION IGU was effective for and well tolerated by our patient with pSS concomitant with AIHA,and may be a promising therapy for the treatment of this disease.
文摘Objective:To investigate the TCM treatment principle of membranous nephropathy and its effect.Methods:A total of 56 patients were selected from the Affiliated Hospital of Shaanxi University of Traditional Chinese Medicine.They were then divided into the control group(western medicine standard therapy)and the study group(Qidi Gushen prescription),with 28 patients in each group.The treatment effect,treatment safety,and patients’satisfaction were observed and compared between the two groups.Results:The results showed that the treatment effect of the study group was 96.43%,which was significantly better compared with the control group(75.00%)(p<0.05);in terms of safety,the probability of adverse events was 7.14%in the study group and 32.14%in the control group,in which the difference was statistically significant(p<0.05);in addition,the study group’s satisfaction with the treatment measures was significantly higher than that of the control group(p<0.05).Conclusion:In treating membranous nephropathy,traditional Chinese medicine can be tailored to its pathogenesis,which is not only beneficial to the treatment effect,but also has a high safety profile.
基金Supported by the Science and Technology Program for Basic Research in Shenzhen (No.JCYJ20200109140412476)Clinical Research Project of Shenzhen Second People’s Hospital in 2019 (No.20193357009)。
文摘The recognition of the profound impact of the human gastrointestinal microbiome(GM) on human autoimmune diseases has gradually increased thanks to deeper research efforts. As a systemic autoimmune disease, primary Sjogren’s syndrome(pSS) cannot be completely cured. Human studies have revealed that GM species and diversity are altered in patients with p SS compared with healthy individuals. Animal studies have provided possible mechanisms for the association between pSS and GM. The potential role of GM in pSS is exerted through several mechanisms. GM dysbiosis leads to increased intestinal permeability, which increases the risk of GM antigen exposure and activates specific autoreactive T lymphocytes via “molecular mimicry”. In addition, GM antigen exposure and intestinal immune tolerance loss caused by GM dysbiosis together induce chronic local gut mucosal inflammation, which deteriorates to systemic chronic non-specific inflammation with the circulation of pro-inflammatory lymphocytes and cytokines. These factors eventually activate autoreactive B lymphocytes and lead to pSS. If GM plays a key role in the pathogenesis of pSS, clarifying the underlying mechanisms will be helpful for the development of new therapies targeting GM for dry eye associated with pSS. This review summarizes the latest knowledge about the relationship between GM and p SS,with the aim of contributing to future research and to the development of new clinical applications.
基金Natural Science Research Project of Guangxi University of Chinese Medicine(2015LX037).
文摘[Objectives]To observe the clinical efficacy of Shenling Baizhu Powder in the treatment of primary nephrotic syndrome(PNS)featuring damp abundance due to splenic asthenia.[Methods]40 patients with PNS were randomly divided into treatment group(n=19)and control group(n=21).In the treatment group and the control group,methylprednisolone tablets were taken orally and cytoxan(CTX)was given intravenously for treatment;in the treatment group,based on oral administration of methylprednisolone tablets and intravenous drip of cytoxan(CTX),oral administration of traditional Chinese medicine(addition and subtraction of Shenling Baizhu Powder)was added.Urine routine,24-hour urinary protein,renal function and serum albumin were re-examined after 4 weeks of treatment.[Results]The total effective rate of the treatment group was significantly higher than that of the control group(89.47%vs 61.90%,P<0.05).After treatment,both groups could effectively reduce the levels of 24-hour urinary protein(P<0.05),serum creatinine(P<0.05)and urea nitrogen(P<0.05),and increase the level of serum albumin(P<0.05),but the degree of improvement in the treatment group was significantly higher than that in the control group(P<0.05),and the difference was statistically significant(P<0.05).[Conclusions]Methylprednisolone combined with addition and subtraction of Shenling Baizhu Powder could effectively reduce urinary protein,improve edema symptoms,shorten the course of hormone use and reduce adverse reactions in the treatment of PNS featuring damp abundance due to splenic asthenia.
文摘<div style="text-align:justify;"> <span style="font-family:Verdana;"><strong>Background:</strong> The association of primary Sj<span style="white-space:nowrap;">ö</span>gren syndrome (PSS) and AA amyloidosis is a rare occurrence. <strong>Objective: </strong>To describe the phenotype of patients with this association through our two cases and a literature review. <strong>Materials and methods:</strong> A report of two cases of AA amyloidosis complicating primary Sj<span style="white-space:nowrap;">ö</span>gren syndrome with a literature review. <strong>Results:</strong> Eight patients of Primary Sj<span style="white-space:nowrap;">ö</span>gren’s Syndrome complicated by AA amyloidosis were studies. Six cases were reported in the literature by consulting several databases. 50% of patients had a positive immunological assessment, three cases with kidney damage, and three cases lung damage. <strong>Conclusion: </strong>The immunological activity in the Primary Sjogren’s Syndrome requires the search not only a lymphoma but also AA amyloidosis apart from any clinical or biological chronic inflammation.</span> </div>
文摘Antiphospholipid syndrome (APS) is a prothrombotic condition that can affect both the venous circulation and the arterial system. The deep veins of the lower extremities and cerebral blood flow are the most common sites of venous and arterial thrombosis, respectively. Skin ulceration and gangrene may be associated with an active vasculitis in patients with APS. These kinds of ulcers are considered intractable because healing is difficult to achieve. Using steroids as immunesuppressant therapy is still a basic part of managing APS and vasculitis. Nevertheless, in the presence of steroid-induced diabetes mellitus, the priority is to achieve faster wound healing, because of the potential complications that can develop due to metabolic dysfunction and augmented vulnerability to infection. Until recently, there were few reports demonstrating the benefits of the use of adjuvant phosphodiesterase-5 inhibitors like sildenafil citrate in different clinical entities with courses like Raynaud’s phenomenon, scleroderma, and resistance to vasodilator therapy. We present the first report demonstrating the additional benefit of sildenafil citrate for the integration of grafts in a patient with steroid-induced diabetes and APS who had ulcers due to vasculitis resistant to the usual vasodilator therapy.
文摘Backgrounds: To observe the effects of aerobic exercise program on the illness symptoms and cardiopulmonary capacity of the patients with primary Fibromyalgia Syndrome (FMS) and to find out whether there is a difference or not in terms of cardiovascular capacity among the patients with primary FMS and sedentary healthy people. Materials and Methods: The study has been done on 20 female patients with primary FMS. Before the study and after a 6-week controlled aerobic exercise program, a cardiopulmonary exercise test has been applied on all cases. The symptoms, psychological state and life quality of the cases have been evaluated before and after the exercise program. The first cardiopulmonary results of the cases with FMS have been compared with a control group of 15 sedentary healthy. Results: When the patient and control groups are compared, although maksimal oxygen uptake (VO2 max) was lower in the patient group, this was not a significant difference. VO2 max showing the aerobic condition after the exercise increased significantly in the patients. Conclusions: Aerobic exercise programs lasting six to eight weeks are quite effective for the patients with FMS. There is not any limitation in the increase of the cardiopulmonary capacity of the patients after the exercise program.
基金Jiangsu Provincial Key Research and Development Program,Grant/Award Number:BE2020621.
文摘Background:Understanding the clinical implications of autoimmune manifestations associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is essential to reduce its consequences. This study was aimed at determining the activities of new-onset primary Sjögren syndrome (pSS) since the emergence of SARS-CoV-2.Methods:This retrospective cohort study included data from 471 participants with dry mouths and eyes who had been attending Nanjing Drum Tower Hospital since December 2019. By April 2023, patients diagnosed with pSS were sequentially assigned to vaccinated group ( n = 24) or vaccinated and infected group based on exposure ( n = 20). Patients diagnosed with pSS within 3 months of vaccination against SARS-CoV-2 were assigned to a vaccinated group, and those who had been vaccinated and then developed pSS within 3 months of follow up after direct exposure to SARS-CoV-2 were assigned to a vaccinated and infected group. The controls comprised age- and sex-matched patients who had not been exposed to SARS-CoV-2 before December 2019 ( n = 21). We then compared data among the three groups. Results:The vaccinated and infected patients had more fever, malaise, splenomegaly, and weight loss before diagnosis and a higher European Alliance of Associations for Rheumatology Sjögren's syndrome disease activity index at the time of onset than the other two groups. Vaccinated patients had a higher frequency of anti-nuclear antibody (ANA) titers > 1:320 ( n = 12;50%) and anti-phospholipid antibodies (aPL) ( n = 7;29%) than the controls. The frequency of anti-Ro/SSA antibodies (13, 65%), ANA titers > 1:320 ( n = 16;80%), and aPLs ( n = 7;29%) ( n = 5;25%) were all significantly higher in vaccinated patients with infection than those in the controls. Higher doses of glucocorticoids, cyclosporin A, and tacrolimus were administered to the vaccinated and infected than the vaccinated and control groups ( p < 0.05, for all). Conclusions:Patients with new-onset pSS and a history of vaccination and SARS-CoV-2 infection might have more active disease. Further strengthening the assessment of people with a clear history of SARS-CoV infection and the monitoring of potential populations for autoimmune screening should not be overlooked.
文摘Objective: To observe the relationship of tumor necrosis factor-o (TNF-a) and nitrogen oxide (NO) with the treatment of frequent relapse nephrotic syndrome (FRNS) and to explore the patho-genesis of FRNS and the therapeutic mechanism of Shenkangling (肾康灵,SKL) Granule in children. Methods: Sixty children suffering from FRNS were randomly divided into the treated group and control group, 30 in each, and the other 30 healthy children were taken as healthy group. The patients were treated with prednisone for a long-term course, and those with no effect or partial effect shown were treated with additional Tripterygium or Cytoxan in the control group, while in the treated group patients were treated with prednisone and additional SKL. The two groups were compared as to their changes of TNF-a, NO before and after treatment, and the relapses after treatment. Results: The levels of TNF-a and NO in the sick children before treatment were markedly higher than those after treatment and normal group (P< 0. 01). The positive correlation between TNF-o of FRNS cases and relapse risk displayed more significance than that between the relapse of FRNS and NO. The difference between treated group and control group was significant (P<0. 01). Conclusion: TNF-a can be regarded as the monitoring parameter of the active phase in FRNS, and the higher the level, the more possible the relapse would occur. SKL could markedly reduce the relapse rate of FRNS in children.
基金supported by grants from the Chinese National Key Technology R&D Program,Ministry of Science and Technology(Nos.2017YFC0907601,2017YFC0907605)CAMS Innovation Fund for Medical Sciences(CIFMS)(No.2021-I2M-1-005)
文摘Primary Sjögren’s syndrome(pSS)is a systemic autoimmune disease with high prevalence and possible poor prognosis.Though the pathogenesis of pSS has not been fully elucidated,B cell hyperactivity is considered as one of the fundamental abnormalities in pSS patients.It has long been identified that Janus kinases-signal transducer and activator of transcription(JAK-STAT)signaling pathway contributes to rheumatoid arthritis and systemic lupus erythematosus.Recently,increasing numbers of studies have provided evidence that JAK-STAT pathway also has an important role in the pathogenesis of pSS via direct or indirect activation of B cells.Signal transducer and activator of transcription 1(STAT1),STAT3,and STAT5 activated by various cytokines and ribonucleic acid contribute to pSS development,respectively or synergically.These results reveal the potential application of Janus kinase inhibitors for treatment of pSS,which may fundamentally improve the quality of life and prognosis of patients with pSS.
基金This work is supported by grant from Beijing Tide Pharmaceutical Co,Ltd.
文摘Objective:To investigate the effect of prostaglandin E1 (PGE1) (Alprostadii injection) on patients with primary nephrotic syndrome(PNS). Methods: 37 patients with PNS were recruited to study the effect of prostaglandin E1 on platelet aggregation function [ PAG (5,) PAG( m ) ], serum total protein (TP) , albumin (Al),blood urea nitrogen(BUN) ,serum creatinine(Scr) ,cholesterol(CHO), triglyceride(TG), protein in 24-hour urine (Pr/24h) and platelet account (PLT). Results: TP, Al, CHO, TG, BUN, Scr, Pr/24h, PAG(5) and PAG(m) in PNS group before treatment were significantly different from those in control group(P<0.05, P<0.01) while no significant difference was found for PLT. When treated with PGE1 , TP,Al,CHO, TG, Pr/24h, ADP- induced PAG(5) ,and Adr- induced PAG(5) and PAG(m) were significantly different from those before treatment (P<0.05). Adr- induced PAG(5) and PAG(m) were significantly different. Adr- induced PAG(5) was xsitively correlated with BUN and Scr in PNS(P<0.01). Similar correlation was found between ADP-induced PAG(5) and Al ,BUN,Scr,Pr/24h(P<0.05), AD- induced PAG(m) and TP,CHO(P<0.05). Conclusions: PGE1 may be an effective drug for the treatment for hypercoagulation in patients with PNS.
基金Supported by Shanghai Municipal Commission of Health and Family Planning(No.2010QL031B)
文摘Objective: To provide an objective reference for the syndrome types of Chinese medicine(CM) associated with pediatric primary nephrotic syndrome(PNS).Methods: A cross-sectional study was performed.Data on clinical symptoms,CM syndrome types,biochemical indices,and medications used were collected from 98 children with PNS.Then,the correlation between CM syndromes and biochemical indices,as well as medications used,was analyzed.Results: The four most common symptoms in children with PNS were brown urine,red tongue,excessive sweating,and swelling of the face and limbs.The syndromes of qi deficiency of Fei(Lung) and Shen(Kidney)(FSQD) and yin deficiency of Gan(Liver) and Shen(GSYD) were the most common main CM syndrome types.FSQD syndrome score correlated significantly with the total cholesterol level,urine protein/creatinine ratio,and urine Ig G and albumin levels(P〈0.01 or P〈0.05).The use of maintenance glucocorticoids combined with immunosuppressive agents correlated with FSQD syndrome,and the use of maintenance glucocorticoids alone correlated with GSYD syndrome(P〈0.05).Conclusion: Two of the most common CM syndrome types were FSQD and GSYD syndromes.FSQD syndrome may be caused by some factors related to lipid levels,protein loss,and the use of immunosuppressive agents.The use of maintenance glucocorticoids may cause GSYD syndrome.