Objective To study the characteristics of the serum free light chain ( sFLC ) and cytokines levels and the effects of cyclophosphamide (CTX) on patients with primary Sj(0)gren's syndrome (pSS) complicated with amyloidosis.Methods Nine patients of pSS with amyloidosis who were admitted to Department of Rheumatology of the hospital from Jan 2005 to Mar 2011 were selected.The serum levels of sFLC and cytokines TNF-α,TGF-β1,IL-4 were measured before and 3,6,12 months after the treatment respectively.Meanwhile,Twelve patients with pSS without complications were selected as controls.Repeated measures analysis of variance was used for data analysis.Results The sFLC in serum of pSS patients with amyloidosis was λ type,the κ type was not found.The level of serum sFLC after 12 months treatment [(22±42) mg/L]was significantly lower than before treatment [(180±15) mg/L].The levels of TNF-α,TGF-β1,IL-4 after 12months treatment [(24.0±1A),(58±5),(38.8±3.2) ng/L,respectively] were significantly lower than before treatment [(31.1±L0),(65±5),(61.2±3.8) ng/L,respectively].Conclusion The levels of serum sFLC and eytokines are abnormally increased in patients of pSS with amyloidosis.After treatment,the levels of cytokines are decreased initially which indicates that these cytokines may play a key role in the pathogenesis of pSS.CTX therapy may lead to better efficacy and more favorable prognosis.

Objective To investigate the correlation between serum tumor markers levels with the disease condition activity and the tumor occurrence in the patients with rheumatoid arthritis(RA).Methods Serum tumor marker CA199,CA153 levels were de-tected in 35 patients with RA (RA group)and 31 healthy controls(normal control group).ESR and CRP were examined and the disease activity (DA)was assessed by the DAS28 score in the RA group.The serum tumor markers levels and the positive rates were compared between the RA group and the normal control group.The correlation between the serum tumor markers levels with ESR,CRP and DAS28 score was analyzed.Results Serum levels and positives rate of CA199,CA153 in the RA group were higher than those in the control group(P <0.05).The serum CA153 level had a positive correlation with the ESR,CRP and DAS28 score in the RA group(P <0.05),but the serum CA199 level had no significant correlation with them.Conclusion The serum levels and positive rates of CA199 and CA199 could be increased in the patients with RA,moreover the increase of serum CA153 level is corre-lated with DA of RA.Therefore the serum tumor marker CA153 level may be used as one of reference indexes for evaluating the DA in the patients with RA.

ObjectiveTo assess the efficacy of low-dose intravenous cyclophosphmide (IV CTX) in treatmerit of old patients with systemic lupus erythematosus(SLE).MethodsTwenty-three old patients with newly diagnosed, untreated SLE were included.Patients received 3 times fortnightly IV CTX pulses at a fixed dose of 400mg followed by 3 monthly pulses.All patients were orally administered 0.8mg/kg of prednisone.The changes of the indexes were observed before and after 12 weeks treatment including the scores of SLE disease activity index(SLEDAI) and the levels of C3, C4 ,24-hour urinary protein and anti-dsDNA antibody.ResultsAmong 23 patients, 19 were followed up to 24 weeks, SLEDAI 4 weeks after treatment were all significantly lower than those before the treatment (P ＜0.01).At week 8, SLEDAI continued to decrease(P ＜0.05).At week 4,the level of urine protein, the levels of complements C3 and C4 and ds-DNA decreased significantly compared with that before the treatment (P ＜0.05).ConclusionIntensive low-dose CTX plus prednisone was effective in newly diagnosed, untreated old patients with SLE, and could reduce adverse effect.

Objective To explore the relationship between life events and depression of patients with primary sjgren's syndrome (PSS).Methods 80 patients with PSS complicating depressive disorder were tested by self-rating depression scale(SDS) and life event scale (LES),and compared with the control group including 80 PSS patients with non-depressive disorder matched with age and sex.Then the correlation between SDS and LES were analyzed.Results The total score of life events (32.72 ± 13.93),the score of negative life events (24.36 ±11.24),the score of SDS(58.60 ± 16.78) and the frequency of life events(91.3％) in the research group were higher than those in control group(P ＜ 0.05),but the positive life events score (7.15 ± 5.84) was lower than that in control group (9.76 ± 7.29),and there was statistically significant difference between the two groups (P ＜ 0.05),there were more negative life events,especially on the aspects of family,work and study in the research group.There was positive correlation among the negative life events score,total score of life events and SDS score in the research group(P＜ 0.01),meanwhile,negative correlation was revealed between the score of positive life events and SDS score in the research group(P ＜ 0.05).Conclusion Patients with PSS complicating depressive disorder experienced more negative life events which play an important role in depression of patients with PSS.

Objective To survey the prevalence and the related factors of depression in patients with primary Sj(o)gren's syndrome (pSS),and provide references for early mental intervention.Methods The depression state of 217 patients with pSS was screened with self-rating depression scale (SDS) first and then assessed by Hamilton depression scale (HAMD,17 index).The prevalence of depression was determined based on the diagnosis of psychiatrist based on the 3rd edition of Chinese classification of mental disorders (CCMD Ⅲ).The personality was evaluated by Eysenck personality questionnaire (EPQ),and the life events were questionnaired by life events scale (LES).The general condition was analyzed by self-designed questionnaire.Finally,step-wise multiple factor regression analysis was carried out to determine the risk factors that best accounted for the depression.Results The prevalence rate of depression in patients with pSS was 39.2％ (85/217).There were 5 factors entered the regression equation by multifactor regression analysis.They were severe pain,concerns of doctor and nurse,known of the fact by patients,adverse life events,and neuroticism of EPQ,and the concern of doctor and nurse was negatively correlated.Conclusion More attention from clinicians should be paid to the depression of pSS.Patients with severe pain,lack of attention from doctors and nurses,known of the disease itself,adverse life events and neurotic personality traits are factors related with depression.Recognizing the risk factors early can provide guidance for early psychological intervention.

Objective To investigate the prognosis of patients with Kawasaki's disease complicated with coronary artery lesion and to provide evidence for diagnosis and treatment of these patients. Methods This study was conducted during January 2002 to June 2007. All patients diagnosed as Kawasaki's disease complicated with coronary artery lesions were from the Affiliated Hospital of Guilin Medical College, the Second People's Hospital of Guangxi Province and Guilin Women and Childrens' Hospital. All cases were echocardiogram examined in month 3, 6, 12, 24 and 36 in the purpose of observing the morphology of coronary artery. The study subjects were re-categorized to the groups of mild, moderate dilatation of coronary artery and giant coronary aneurysm, based on the severity of coronary artery lesion. The results of these results of the three groups were compared. Results Eighty-four cases in the mild group, 27 cases in the moderate group and 8 cases in the giant coronary aneurysm. The recovery cases were 23 (27%), 3 (11%) and 0 in the 3 groups respectively at month 3. The above numbers were 44 (52%), 8 (30%) and 0 respectively at month 6.The numbers were 69(82%), 13 (48%) and 1 (13%) at month 12. The numbers were 78 (93%), 19 (70%), 3 (38%) at month 24. The numbers were 82(98%), 20(74%) and 4(50%) at month 36. Thirteen patients were treated with adenosine-triphosphate (ATP) stress echocardiography examiantion, 5 patients were evaluated by coronary angiography,and 4 patients were tested by 64-slice CT coronary reconstruction. Part of the patients were found to have coronary stenosis or occlusion. Conclusion Patients of Kawasaki's disease often have concurrent coronary artery lesions. Patients with mild dilatation of the coronary artery are the most commonly seen and have the best prognosis. On the contrast, patients with giant coronary aneurysm are the lest common situation and is the worst in prognosis. Part of them will develop coronary artery stenosis or occlusion in late stage which may lead to ischemic heart disease. We should stress on close follow-up of patients with Kawasaki's disease complicated with coronary artery lesion. Appropriate and timely treatment will increase their clinical outcomes.

Objective:To evaluate the safety and efficacy of belimumab(BLM) in patients with SLE.Methods:Clinical data were collected for SLE patients who were diagnosed and treated with BLM in the Department of Rheumatology and Immunology (inpatient and outpatient department) of Guilin Medical College Affiliated Hospital from 1 December, 2019 to 12 May, 2023. BLM + standard of care (SOC) for the BLM group and SOC only for the SOC group. The primary clinical endpoint was adverse events (AE) occurring in groups, and the secondary clinical endpoint was disease activity index including SLEDAI-2000, clinical indicators, glucocortoid dosage reduction, and disease flare in the two groups. Propensity score matching method, independent sample t-test, non-parametric rank-sum test, variance analysis were used for statistical analysis. Results:Among the 79 BLM patients included, 48 had hematological impairment (61%), 53 had renal impairment (67%), 11 had skin and mucosal impairment (14%), 20 had joint impairment (25%), and 2 had neurological impairment(3%). There were no serious adverse events during the treatment in both groups. In the BLM group, with 14 cases experienced respiratory system infection, 1 with urinary system infection, 3 with skin infection, 4 with herpes virus infection, and 16 with liver function impairment. In the SOC group, there were 26 cases experienced respiratory system infection, 1 with urinary system infection, 6 with digestive system symptoms, 6 with skin infection, 4 with herpes virus infection, 10 with liver function impairment, 2 with thrombosis, and 1 with sepsis. Patients tolerated BLM generally well, with fewer adverse events occurring [in patients with the long course treatment, the number of AE cases in the BLM group vs SOC group 11 [(33%) vs 22 (68%), ( χ2=3.74, P=0.053)]. From all study groups and high-dose glucocorticoid (≥20 mg) groups, it was observed that the BLM group had a more significant reduction in glucocortoid dose [baseline glocucorticoid reduction in the BLM group decreased from 20 (12, 40)mg/d to 6 (4, 10)mg/d ( Z=0.12, P=0.01). In the renal injury group, after treatment, serum creatinine level decreased in the BLM group glomerular filtration rate increased from 72.37 (41.97, 95.74) to 97.03 (71.18, 114.34) ( Z=-4.62, P<0.001). There was less flares in the BLM group [7 cases (28%) vs 18 cases (72%), χ2=5.58, P=0.018] when compared with the soc group. Conclusion:BLM is safe and effective for the treatment of SLE, which can reduce disease activity, improve clinical parameters, reduce glocucorticoid dosage, and have a low flare rate.