Recommendations of diagnosis and treatment of polyarteritis nodosa / 中华内科杂志

Polyarteritis nodosa (PAN) is a rare vasculitis that mainly involves small and medium arteries. It often occurs at the points where the vessels bifurcate, leading to microaneurysm formation, thrombosis, aneurysm rupture and bleeding, and infarction of organs.About a third of cases are associated with hepatitis B virus (HBV) infection.All tissues and organs of the body can be affected, with skin, joints and peripheral nerves being the most common.The pathological changes were fibrinoid necrosis, inflammatory cell infiltration and luminal thrombosis in the acute stage, and fibrous hyperplasia in the chronic stage.Overall outcomes for the disease have improved in recent decades, mainly reflecting early diagnosis and more effective treatments.The main treatments for PAN are glucocorticoid and cyclophosphamide.Patients with HBV-associated PAN should receive antiviral therapy and plasma exchange.

Chinese expert investigation on diagnosis and disease activity evaluation in Takayasu's arteritis / 复旦学报（医学版）

Objective To investigate the current situation in Chinese rheumatologic physicians' clinical diagnosis and evaluation of Takayasu's arteritis (TA).Methods Nineteen rheumatology experts and three vascular surgery specialists in China were invited to make the nationwide investigation for the first time about the diagnosis and disease activity evaluation of TA in China,through the questionnaire survey on the internet.Weighted average was used to calculate the average scores of corresponding problems.Results Chinese experts mainly adopted 1990 American College of Rheumatology (ACR) classification criteria for clinical diagnosis of TA.In details,symptoms of age,limb claudication and amaurosis,signs including pulselessness or pulse weakening,vascular bruits,increasing bilateral pulse pressure and hypertension and acute phase reactants (APR) were critical to the clinical diagnosis of TA.Besides,noninvasive imaging examinations,such as computed tomography angiography (CTA),magnetic resonance angiography (MRA),vascular ultrasonography,and positron emission tomography (PET) were also of great importance.In the aspect of disease activity assessment,Chinese experts mainly used Kerr scoring tool.APR and noninvasive radiological examinations were considered with vital value.Some TA patients with carotid artery involvement were recommended using vascular ultrasonography,while others with pulmonary artery and thoracic/abdominal aorta trunk involvement were preferred CTA other than MRA.Conclusions APR and noninvasive imaging examinations were thought with great help to make clinical diagnosis and evaluation of TA for Chinese physicians.

A cross-sectional study on application of glucocorticoid in systemic lupus erythematosus patients / 中华内科杂志

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.

Primary study on finger joints MRI coil and its value in rheumatoid arthritis / 实用放射学杂志

Objective To study the MR performances of finger joints with health subjects and rheumatoid arthritis (RA)patients,and to detect the imaging quality of a custom-made finger coil.Methods Finger coil,extremity coil,flexible surface coil and 3 inch surface coil were used to scan the self-made water phantom with similar T2 WI sequence,then the signal to noise ratio (SNR)on single image was measured for the comparison.The middle finger specimen (1 finger)was used for the research,5 healthy volunteers (8 fingers)and 18 RA patients (22 fingers)were also scanned.Results The SNR of finger coil was over 1.5 times higher than that of the 3 inch surface coil and 4 times higher than that of the extremity coil and flexible surface coil.For the 22 fingers with confirmed RA,synovitis was found in 1 7,joint effusion in 10,bone marrow edema in 5,bone erosion in 7 and tenosynovitis in 6.Conclusion The finger coil has higher SNR.

An experimental study on half-dose immunosuppressive agents multi-target therapy for lupus nephritis / 中华风湿病学杂志

Objective To investigate the efficacy and adverse reactions of half-dose glucocorticosteroid and cytoxan,combined with leflunomide for the treatment of lupus nephritis (LN) of MRL/lpr mice,and provide experimental evidences for LN therapy.Methods Twenty-eight 10-week-old MRL/lpr mice were randomly divided into four groups:Group A,blank control group; Group B,classical control group; Group C,full-dose control group; Group D,half-dose treatment group,with 7 mice in each group.The therapeutic efficacy and side reactions in the four groups were observed and compared before and 12 weeks after treatment.Statistical analysis was conducted with one-way ANOVA,q test and Pearson's correlation analysis.Results The serum anti-double stranded DNA (anti-dsDNA) antibody titers (0.43±0.16,0.32±0.09,0.44± 0.18,1.95±0.19) U/ml,serum creatinine level (1.63±0.63,0.40±0.23,0.82±0.21,10.86±2.17) mg,24-hour urine protein excretion level (71±8,60±5,68±3,121±10) μmol/L and renal pathological changes in group B,C,D were significantly improved than those of the group A (P＜0.05) after 12 weeks treatment.There was no significant difference in the efficacy between group B,C,and D (P＞0.05).The incidence of adverse reactions in group D was significantly lower than that in other groups (P＜0.05).Conclusion Multi-target therapy,such as half-dose prednisone and CTX,combined with leflunomide can effectively control lupus disease activity with less side effects.This regimen is cheap,safe and effective for the treatment of LN in MRUL/lpr mice.This study has provided animal evidences for this multi-target therapy for LN.

Mizoribine versus mycophenolate mofetil or intravenous cyclophosphamide for induction treatment of active lupus nephritis / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Lupus nephritis (LN) is one of the most serious manifestations of systemic lupus erythematosus. Although there have been substantial improvements in LN treatment over the last decade, the outcome remains unoptimistic in a considerable percentage of patients. The aim of this study was to evaluate the efficacy and safety of mizoribine (MZR), a novel selective inhibitor of inosine monophosphate dehydrogenase, as induction treatment for active LN in comparison with mycophenolate mofetil (MMF) and intravenous cyclophosphamide (CYC).</p><p><b>METHODS</b>Ninety patients with active LN were observed. Thirty patients were given MZR orally at the dose of 300 mg every other day. Thirty patients took MMF at 2 g per day in two divided doses. Thirty patients received CYC intravenously 0.5 g every 2 weeks. Therapeutic effects and adverse events (AEs) were evaluated at the end of 24-week treatment. One-way analysis of variance (ANOVA) followed by Dunn's test was applied to compare the difference among the groups. For comparing categorical data between two groups, χ(2) test was employed.</p><p><b>RESULTS</b>Early responses at week 12 were achieved by 73.3%, 90.0%, and 96.7% in MZR, MMF, and CYC groups, respectively. There was no significant difference in the complete remission rates (22.7%, 24.0%, and 25.0%, respectively) or overall response rates (68.2%, 72.0%, and 75.0%, respectively) among the three groups at week 24. The most prominent drop-down of Systemic Lupus Erythematosus Disease Activity Index scores was observed in MMF or CYC group, and the decline of health assessment questionnaire scores in MZR or MMF group was more prominent than that in the CYC group at week 12. Serum complement 3 (C3) or C4 levels were elevated in all groups after the treatments. CYC was more effective in inhibiting anti-double-stranded DNA antibody, while MZR was more effective in inhibiting antinuclear antibody. The incidences of AEs in patients treated with CYC were significantly higher than those in patients treated with MZR or MMF (24.2% for CYC vs. 3.3% for MZR, and 2.6% for MMF, P = 0.01).</p><p><b>CONCLUSIONS</b>MZR is well tolerated and has an effect similar to MMF in the induction therapy of active LN. MZR may serve as an alternative approach for LN patients.</p>

Clinical and serological value of complement C3:a retrospective study of 1405 hospitalized patients with systemic lupus erythematosus / 中华风湿病学杂志

ObjectiveTo investigate the association of complement C3 with clinical and serological features of patients with systemic lupus erythematosus.MethodsData was collected by the same methods in the past ten years in fifteen hospitals in Jiangsu Province and then data weres summarized for retrospective analysis.Clinical and laboratory data were selected and then analyzed by Chi-square test,Wilcoxon rank sum test and Logistic regression.ResultsOne thousand four hundred and five patients were investigated.One thousand and forty two had low serum complement C3 level.In this case control study,hospitalization age,disease course,admission times,pleurisy,gastrointestinal involvement,general lymphadenopathy/hepatosplenomegaly,white blood cell count, haemoglobin level,platelet count, serum C-reactive protein level,serum albumin level,serum creatinine level,Urine protein quantification,anti-nuclear antibodies (ANA),anti-dsDNAantibodies, anti-SmantibodiesandSLEDAIscore were possible factors associatedwith complement C3 reduction(P＜0.05).Logistic regression analysis showed that CRP (OR=0.396,0.254-0.617,P=0.000),ANA (OR=2.907,1.267-6.670,P=0.012),urine protein level(OR=1.702,1.043-2.779,P=0.033) and SLEDAI score (OR-0.930, 0.886-0.975,P-0.003) were correlated with complement C3 reduction.Conclusion Complement C3 level is valuable for lupus flare assessment.The complement C3 reduction is a risk factor for renal impairment.

Effects of the different sections of receptor-associated protein on the expression and distribution of TRPC6,synaptopodin and podocalyxin in passive Heymann nephritis / 中华肾脏病杂志

objective To investigate the effects of different sections of receptor associated protein (RAP) on the expression and distribution of TRPC6,synaptopodin and podocalyxin in passive Heymann nephritis(PHN). Methods Male Sprague-Dawley rats were injected with three kinds of antisera (anti-RAP full-length serum,anti-RAP N-terminal serum and anti-RAP C-terminal serum)to establish three kinds of PHN models.The control group was injected with normal rabbit serum.The quatitation of 24 h urinary protein,serum albumin and creatinine were taken before injection and one week after PHN model successfully induced.The histopathologic changes of renal tissues were observed by light microscopy.The expression and distribution of TRPC6,synaptopodin and podocalyxin in glomerular podocytes were observed by laser scanning confocal microscopy and analyzed by fluorescence quantitative software after indirect immunofluorescence double staining.Results The quantities of 24 h urinary protein in the three model groups were significantly higher than those of themselves before injection and control groups (P0.05).The expression of TRPC6 in podocytes was higher in the PHN model groups than that of control group.Fluorescence intensity of TRPC6 in RAP full-length group was stronger than that in RAP N-terminal or C-terminal groups.The expressions of synaptopodin and podocalyxin distributed along the glomerular basement membrane as spot,discontinuous short line and defect of some segments,and were lower in three PHN groups than those of control group.Fluorescence intensity of synaptopodin and podocalyxin among three PHN groups had no differences. Conclusions RAP full-length and N-terminal or C-terminal parts can increase the expression of podocyte TRPC6,but decrease the expressions of synaptopodin and podocalyxin,and alter their distribution,which may be associated with the proteinuria,however,their role in the PHN pathogenesis needs further study.

The relationship between impairment of hematological system and prognosis in systemic lupus erythematosus / 中华风湿病学杂志

Objective To explore the relationship between the impairment of hematological system and disease activity,immunological parameters,and the prognosis of systemic lupus erythematosus (SLE).Methods The clinical and laboratory data of in-patients with SLE in Jiangsu Province were investigated and all patients were hospitalized between 1999-2009.The impairment of hematological system was assessed and the relationship between hematological system damage and disease activity,immunological parameters,mortality rate of patients with SLE were analyzed.Statistic method used was X2 test.Results One thousand nine hundred and fifty eight cases of SLE were included in the study,in which,1836 were female and 122 were male.One thousand five hundred and forty nine (79.1%) patients complicated with hematological system damage,62.3% were anemia,45.5% with leucopenia and 29.4% with thrombocytopenia.There were significant differences in hematological system damage rate among patients with mild activity group,moderate activity group,severe activity group and almost no activity group,compared respectively with almost no activity group.The P values were P=0.01 and P＜0.01 respectively.The incidence of hematological system damage in elevated ESR,low complement C3 level,anti-dsDNA antibody group was higher than that in patients who had normal ESR,complement C3 level and anti-dsDNA group.(P＜0.01).During follow-up,166 patients died,of which the mortality rate(91.6%) in patients had hematological system damage,was obviously higher than those without hematological damage(8.4%)(P＜0.01 ).Among the 166 deceased patients,38.6% died of severe infection,22.9% died ofrenal failure,15.1% died ofnervous system damage,10.2% died of cadiovascular damage and 13.3% died from other causes.Conclusion Hematological system is one of the most commonly involved system in patients with SLE,of which anemia is the most common,and the incidence of leukopenia follows.The impairment of hematological system is closely related to lupus activity.Patients with abnormal immune parameters tend to have secondary hematological system damage.Severe infection is the main cause of death in patients with lupus,followed by nervous system damage and kidney damage.The mortality rate in patients with lupus that complicated hematological system damage is higher than patients who have no hematological system damage.

Retrospective study on the initial clinical manifestations of 1958 hospitalized patients with systemic lupus erythematosus / 中华风湿病学杂志

Objective To investigate the initial manifestation and disease onset feature of systemic lupus erythematosus(SLE) in the past ten years in fifteen hospitals in Jiangsu Province.Methods Data was collected by the same Methodsin all the participated hospitals and then it was summarized for retrospective analysis.Two groups were compared by chi-square test.Results ① One thousand nine hundred and fifty eight patients were investigated and the male-to-female ratio was 1∶15.0.② One thousand seven hundred and ninty eight patients had clear initial manifestations.The most common initial manifestations were skin and mucosal lesions(769 cases,42.8％ ) and arthritis (697 cases,38.8％ ).The main skin lesion was malar rash (706 cases).Arthritis was found to be more common in female than male.③ All hospitalized patients at their first admission showed multiple organ/system involvement:the most common involvement was skin and mucous membrane (82.3％),hematologic damage (74.0％),in which at least one series of blood cells were involved,arthritis (1156 cases,56.5％ ) much more than myositis (51 cases),proteinuria 1046 cases and hematuria in 385 cases.Renal biopsy pathology showed type Ⅳ glomerulonephritis.Conclusion ① SLE patients are mainly female and the male to female ratio is 1∶15.0.② The most common initial manifestations are skin and mucosal lesions.③ The most commonly involved organ/system are skin and mucous membrane,blood,joint and kidney.The most common pathological changes shown in renal biopsy is type Ⅳ glomerulonephritis.

Study of podocyte slit diaphragm protein NEPH1 and Nephrin in membranous lupus nephritis / 中华风湿病学杂志

Objective To investigate the expression of slit diaphragm proteins of glomerular podocyte,such as NEPH1 and Nephrin in type Ⅴ lupus nephritis (V-LN). Methods Twenty-five patients with V-LN and 18 patients with idiopathic membranous nephritis (IMN) were enrolled into the study, and 5 normal renal samples were the normal control group. Twenty-four hours urine protein excretion, serum albumin, creatinine, triglyceride, total cholesterol, serum C3, C4, urine C3 and NAG were tested respectively.Glomerular lesions were measured by light microscopy. The expressions of NEPH1 and Nephrin were determined by indirect immunofluorescent staining. The statistical treatment was used t-test. Results Compared to the IMN group, the 24 hours urine protein excretion and the concentrations of serum albumin, creatinine, urine C3 were not significantly different while the triglyceride, total cholestorel, serum C3, C4 were significantly decrease in the V-LN group (P<0.05). Urine NAG was increased in the V-LN group (P<0.01). By indirect immuno-fluorescent histochemitry examination, the glomerular expressions of NEPH1 and Nephrin were significantly decreased in both V-LN and IMN. Compared with the IMN group, the decrease of NEPH1 and Nephrin expression was more remarkable in the V-LN group. Conclusion The expression changes of NEPH1 and Nephrin may play an important pathogenic role in proteinuria of Ⅴ lupus nephritis. Renal tubular epithelial cell damage may play a role in proteinuria of V-LN.

A clinical analysis of male patients with systemic lupus erythematosus / 中华风湿病学杂志

Objective To analyze the clinical characteristics of male patients with systemic lupus ery thematosus in a cohort of Chinese patients.Methods 325 lupus nephritis patients (38 male and 287 female patients) admitted to our hospital from January 1997 to December 2006 were summarized in this cohort.The difference in manifestations and laboratory features were analyzed between male and female lupus nephritis patients.Results ① Male lupus nephritis patients had more frequent episodes of serositis (P＜0.05),while fe male lupus nephritis patients more frequently had arthralgia,malar rash,oral ulcers and neuropsychiatric lu pus.② The kidney was involved with early stage of the disease in male patients.The rate of misdiagnosis was higher in male lupus nephritis patients than that of the female patients.③ The prevalence of ANA positivity and thrombocytopenia was higher in male patients than that of the female patients.The prevanee of positive anti-ENA antibody,anti-Sin antibody,anemia and hypergammaglobulinemia was lower in male patients than those of female patients.No difference was found in the presence of positive anti-RNP antibody,hypocom plementemia,elevated erythrocyte sedimentation rate and leucoeytopenia between the two groups.Conclusion The clinical pictures of male systemic lupus erythematosus patients are atypical except for kidney involvement.The kidney involvement of male lupus patients is characterized by the early onset,rapid progression and high rate of misdiagnosis.Physicians should familiar with these differences.Early diagnosis and appropriate treat ment are essential for controlling disease progress.