Continuation, reduction, or withdrawal of tofacitinib in patients with rheumatoid arthritis achieving sustained disease control: a multicenter, open-label, randomized controlled trial / 中华医学杂志(英文版)

BACKGROUND@#Rheumatoid arthritis (RA), a chronic systemic autoimmune disease, is characterized by synovitis and progressive damage to the bone and cartilage of the joints, leading to disability and reduced quality of life. This study was a randomized clinical trial comparing the outcomes between withdrawal and dose reduction of tofacitinib in patients with RA who achieved sustained disease control.@*METHODS@#The study was designed as a multicenter, open-label, randomized controlled trial. Eligible patients who were taking tofacitinib (5 mg twice daily) and had achieved sustained RA remission or low disease activity (disease activity score in 28 joints [DAS28] ≤3.2) for at least 3 months were enrolled at six centers in Shanghai, China. Patients were randomly assigned (1:1:1) to one of three treatment groups: continuation of tofacitinib (5 mg twice daily); reduction in tofacitinib dose (5 mg daily); and withdrawal of tofacitinib. Efficacy and safety were assessed up to 6 months.@*RESULTS@#Overall, 122 eligible patients were enrolled, with 41 in the continuation group, 42 in the dose-reduction group, and 39 in the withdrawal group. After 6 months, the percentage of patients with a DAS28-erythrocyte sedimentation rate (ESR) of <3.2 was significantly lower in the withdrawal group than that in the reduction and continuation groups (20.5%, 64.3%, and 95.1%, respectively; P  < 0.0001 for both comparisons). The average flare-free time was 5.8 months for the continuation group, 4.7 months for the dose reduction group, and 2.4 months for the withdrawal group.@*CONCLUSION@#Withdrawal of tofacitinib in patients with RA with stable disease control resulted in a rapid and significant loss of efficacy, while standard or reduced doses of tofacitinib maintained a favorable state.@*TRIAL REGISTRATION@#Chictr.org, ChiCTR2000039799.

Recommendations of diagnosis and treatment of adult-onset Still′s disease in China / 中华内科杂志

Adult-onset Still′s disease (AOSD) is a rare systemic autoinflammatory disorder. In China, standardized diagnosis and treatment for AOSD is insufficient. Based on the evidence from China and other countries, Chinese Rheumatology Association developed standardization of diagnosis and treatment of AOSD in China. The purpose is to standardize the methods for diagnosis of AOSD, treatment strategies, and reduce misdiagnosis, missed diagnosis and irreversible damage.

Recommendations for management of antiphospholipid syndrome in China / 中华内科杂志

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder with vascular, obstetric, and hematological manifestations associated with thrombotic and inflammatory mechanisms orchestrated by antiphospholipid (aPLs) antibodies. Current clinical practice in APS is highly variable duo to lack of high quality of evidence. Here, Chinese Rheumatology Association developed recommendations for management of APS in China. The recommendations cover the early diagnosis, disease evaluation, thrombotic risk assessment, and treatment.

Case reports and clinical analysis of 8 patients with primary Sj?gren's syndrome diagnosed as anti-synthase syndrome / 中华风湿病学杂志

Objective:Anti-synthase syndrome (ASS) is a rare autoimmune disease. To increase the understanding of the disease and reduce the rate of miss diagnosis.Methods:The clinical data of 8 patients with positive anti-synthase antibody afterprimary Sj?gren's syndrome (pSS) were retrospectively analyzed and descriptive statistical analysis was carried out.Results:The diagnosis of Sjogren's syndrome (SS) was in accordance with the revised European criteriaof SS issued by the US-Europe consensus Group in 2002 or the classification criteria of American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) SS in 2016, and the diagnostic ASS was in accordance with the diagnostic criteria of Conners in 2010 or Solomon in 2011. Eight(100%) patients had a history of interstitial lung disease, and 7 (88%) patients had fever (oral temperature >38.5 ℃). All patients were positive for anti-Ro-52 antibody, 4 patients were positive for anti-PL-7 antibody, 2 patients were positive for anti-EJ antibody, 1 patient was positive for both anti-PL-7 antibody and anti-EJ antibody, and 1 patient was positive for anti-PL-12.Conclusion:pSS patients with severe interstitial lung disease or high fever of unknown causes should be screened for anti-synthase antibodies and the possibility of ASS.

Systemic lupus erythematosus with primary sclerosing cholangitis / 中华风湿病学杂志

Objective:To investigate the clinical manifestations, laboratory and imaging features, treatment and prognosis of systemic lupus erythematosus (SLE) with primary sclerosing cholangitis (PSC).Methods:This is a retrospective case series study describing the clinical, laboratory and imaging manife-stations, treatment and prognosis of 3 SLE patients with PSC. The related literatures were reviewed.Results:In total, 10 patients were included. SLE with PSC, with an average age of (43±17) years old, was more common with hematological and renal involvement, jaundice and arthralgia, positive anti-double-stranded DNA (anti-dsDNA) antibody, hypocomplementemia, elevated erythrocyte sedimentation rate (ESR) and abnormal liver function with predominately elevated alkaline phosphatase (ALP). The classic magnetic resonance cholangio-pancreatography (MRCP) features of PSC include multifocal strictures, beading and areas of dilatation involving the intra- and/or extrahepatic bile ducts. The treatment included glucocorticosteroids, immunosup-pressants and ursodeoxycholic acid (UDCA), and some patients required biliary drainage. Most patients had a good prognosis.Conclusion:Although PSC is rare, attention should be paid to SLE patients with abnormal liver function, especially with elevated ALP, in order to differentiate from PSC.

Treatment with rituximab in 8 cases with systemic sclerosis / 中华风湿病学杂志

Objective:To observe the clinical efficacy and adverse reactions of rituximab in the treatment of systemic sclerosis (SSc).Methods:Eight SSc patients who received rituximab treatment in the Department of Rheumatology of Shanghai Ruijin Hospital from November 2016 to May 2020 were treated with rituximab at week 0, week 2, week 4, week 24 and week 48. The clinical symptoms and laboratory parameters were evaluated at baseline, week 4, week 24 and week 48 respectively. All data were analyzed by Wilcoxon test.Results:All the patients were diagnosed as diffuse SSc, including seven females and one male, with a median disease course of 2.5 years. At week 0, week 24 and week 48, the modified Rodnon skin scores (MRss) were 16.5 (11.8, 29.5) , 14.5 (9.5, 27) ( Z=0.841) and 10.5 (7, 24.3) ( Z=0.420) respectively, which were significantly improved as compared with the baseline ( P<0.05). The patients' self-scores were 60(50, 77.5), 52.5(41.3, 67.5)( Z=0.113) and 47.5(36.3, 57.5)( Z=0.474) respectively, which were significantly improved at week 24 and week 48, and the High Resolution CT (HRCT) scores at baseline and week 48 were 2.7(1.02, 3.7) and 1.6(0.65, 2.95)( Z=0.964) respectively, significantly improved after treatment ( P<0.05). The pulmonary aterial hypertension (PAH) values were 48(41, 58.5) mmHg and 47(38.5, 57) mmHg ( Z=0.315) respectively. There was no significant difference between the two groups. Clinical observation showed that the condition was improved and no adverse reaction occurred at the same time period. Conclusion:The improvement of skin sclerosis, pulmonary interstitial lesion and pulmonary artery pressure can be observed during the treatment with rituximab, which may be a new choice for the treatment of SSc. There is no serious adverse reaction during the treatment, and the patients are well tolerated and safe.

Presence of multiple abnormal immunologic markers is an independent prognostic factor of diffuse large B-cell lymphoma / 医学前沿

Autoimmune diseases (ADs) increase the risk of non-Hodgkin's lymphoma and contribute to poor prognosis of patients. However, the association between immunologic markers and clinical outcome has rarely been investigated. This study aims to analyze the prognostic value of pretreatment immunologic markers in newly diagnosed patients with diffuse large B-cell lymphoma (DLBCL). We retrospectively reviewed the data on 502 patients with DLBCL treated in our institution from January 2013 to March 2018. Survival functions were estimated using Kaplan-Meier method and Cox regression model. The 3-year progression free survival (PFS) and overall survival (OS) rates were 70.2% and 80.9%, respectively, and the complete remission (CR) rate was 78.1%. Among the patients, those with multiple ( ⩾ 3) abnormal immunologic markers had significantly shorter 3-year PFS (52.7% vs. 77.3%, P 60 years for OS (P = 0.014). In conclusion, the immunologic status was closely related to lymphoma progression, and this study provides new insights into the risk stratification of patients with DLBCL.

Significance of mtDNA expression in acute peritonitis induced by monosodium urate in mice / 中华风湿病学杂志

Objective To establish acute peritonitis induced by monosodium urate (MSU) of in mice and observe the significance of mitochondrial deoxyribonucleic acid (mtDNA) expression in the inflammatory processes.Methods The mouse models of acute peritonitis were made by intraperitoneal injection of MSU.Sixty-four male C57BL16 mice were randomly divided into the MSU group which were treated with 0.2 ml of 15 mg/ml MSU solution by i.p.injection and the control group which were treated with 0.2 ml of PBS.Respectively four mice from MSU group and four mice from control group were killed 2 hours, 4 hours, 6 hours, 8 hours 12 hour, 16 hours, 20 hours and 24 hours later and whole blood, peritoneal lavage and peritoneum were collected respectively.Four the mice from the MSU group and four mice from the control group were killed and whole blood, peritoneal lavage and peritoneum were collected.Immunoflourescence study of peritoneum tissues was performed.The levels of interleukin (IL)-1β, IL-18 in plasma and peritoneal lavage were examined by enzyme linked immunosorbent assay (ELISA).DNA was extracted from blood and peritoneal lavage, and mtDNA level was detected by using real-time polymerase chain reaction (PCR).The data was analyszied by multivariate analysis of variance.Results As compared with those killed at other time points from the MSU groups and the control group, the levels of IL-1β [(27.0±2.0) pg/ml vs (26.8±2.1) pg/ml], IL-18 [(673±454) pg/ml vs(752±495) pg/ml] in plasma and peritoneal lavage were increased progressively in those which were killed after i.p.injection of 2 hours and 4 hours from in the MSU group (F=22.778, P＜0.05;F=6.660, P＜0.05).The mtDNA in plasma and peritoneal lavage of the mice began to be expressed 4 hours after i.p.injection 4 hours from in the MSU group.The peak level was detected in those i.p.injected MSU 6 hours later [(9.85±4.59)×106 copies, (7.81±3.43)×106 copies].Then 8 hours later the mtDNA began to slowly decreased.At these three time points, the mtDNA were all increased progressively than those at the other time points of the MSN group or at all time points of the control group (F=6.719, P＜0.05;F=11.181, P＜0.05).By immunoflourescence study, there were neutrophil extracellular traps (NETs) were formed 12 hours later in the MSU group and aggregated NETs were found 24 hours later.Conclusion In the inflammatory processes of acute peritonitis induced by MSU of in mice, with the expression of mtDNA increasing, the inflammation is relieved, and aggregated NETs are formed in the end.Expression of mtDNA may be one for the protective factors of the inflammation induced by MSU.

Value of intravascular ultrasound in the assessment of pulmonary vascular properties and mortality in patients with pulmonary artery hypertension associated with connective tissue diseases / 中华心血管病杂志

<p><b>OBJECTIVE</b>To investigate the value of intravascular ultrasound (IVUS) on assessing pulmonary vascular properties (PVPs) and its relationship with hemodynamics, and mortality rate in patients with pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD).</p><p><b>METHODS</b>Patients (n=51) with highly suspected PAH-CTD were prospectively enrolled in our department between July 2011 and March 2014. All patients underwent right heart catheterization (RHC) and IVUS, and were divided into 3 groups: PAH-CTD (n=25), PAH due to other reasons (n=15), and non-PAH control group (n=11). Based on IVUS, PAH patients were divided into distal (n=22) and proximal (n=18) remodeling subtypes.A total of 408 pulmonary segments were detected by IVUS, and all patients were followed up to (19 ± 10) months.</p><p><b>RESULTS</b>IVUS evidenced higher mean wall thickness (MWT) ((0.30 ± 0.02) mm and (0.33 ± 0.02) mm vs. (0.21 ± 0.02) mm) and percentage of MWT (WTP) ((13.62 ± 0.59)% and (14.39 ± 0.77)% vs. (9.57 ± 0.97)%) values in PAH patients compared to control patients (all P<0.01). Pulmonary vascular mechanical properties (PVMPs) including compliance ((8.85 ± 0.82) × 10(-2)mm(2)/mmHg(1 mmHg=0.133 kPa) and (6.28 ± 0.65) × 10(-2)mm(2)/mmHg vs. (41.59 ± 5.02) × 10(-2) mm(2)/mmHg, all P<0.01), distensibility ((0.83 ± 0.09)%/mmHg and (0.55 ± 0.06)%/mmHg vs. (3.16 ± 0.38) %/mmHg, all P<0.01), elastic modulus ((169.25 ± 15.10) mmHg and (253.00 ± 22.11) mmHg vs.(43.78 ± 4.27) mmHg, all P<0.01) and stiffness index β (4.19 ± 0.41 and 5.18 ± 0.34 vs. 2.39 ± 0.27, P<0.05 or 0.01) in PAH groups were all significantly worse than in control group (all P<0.01). An inverse exponential association was found between PVMPs and hemodynamics with R(2) ranging from 0.544 to 0.777 (P<0.001). PVMPs tended to be better in group PAH-CTD than in PAH group due to other reasons.Mortality rate was similar between the two PAH groups, while PAH with distal remodeling subtype was linked with significantly higher mortality rate than PAH with the proximal remodeling subtype (23 % vs. 0, HR=10.14, P<0.05).</p><p><b>CONCLUSIONS</b>IVUS plays an important role in the assessment of PAH-CTD patients in terms of evaluating PVPs and predicting mortality rate. PAH patients have deteriorated PVPs, but PVMPs tended to be better in PAH-CTD than in PAH patients due to other reasons. The mortality rate was similar between PAH groups, while PAH patients with the distal remodeling subtype is linked with a higher mortality rate than PAH patients with the proximal remodeling subtype.</p>

Risk factor analysis of antineutrophil cytoplasmic antibody-associated systemic vasculitis with pulmonary infection / 中华风湿病学杂志

Objective To analyze the risk factors of ANCA-associated systemic vasculitis with pulmonary infection to improve the alertness of clinicians to imprave the survival of patients.Methods Seventyfive AASV patients in Shanghai Renji Hospital from January 2010 to June 2013 were selected,and logistic regression models was applied to analyze the correlation.Results Of 75 AASV patients,39 (52％) cases were males,36(48％) cases were females,the average ages were (56±19),56(75％) cases were MPA,17(23％) cases were WG and 2(3％) cases were CSS.42(56％) cases were diagnosed to have pulmonary infection.Among the patients with pulmonary infection,23 (55％) were males,19 (45％) were females,24 (57％) were older than 60 years old,17(12％) had WG,37(88％) had MPA,and 8(19％) died.Meanwhile,11(26％) had bacterial infection,2(5％) had tuberculosis,2(5％) had fungi infection,and 27(64％) had mixed infections respectively.8(19％) cases died.Multivariate logistic regression analysis showed that those who were older than 60 years (OR=15.13,95％CI 2.16-106.04,P＜0.01),who were diagnosed as MPA (OR=9.00,95％CI 1.37-59.14,P＜0.05),who had hypoproteinemia (OR=17.61,95％CI 3.14-98.74,P＜0.01) and interstitial lung disease (OR=11.83,95％CI 2.19-63.85,P＜0.01) were more likely to complicate with pulmonary infection.Conclusion Those who are elders,diagnosed as MPA,with hypoproteinemia and interstitial lung disease have higher risk to have pulmonary infection among AASV patients,which underscores that clinicians should pay more attention to this complication of AAV patients.

Invasive fungal infection in diffuse connective tissue diseases: a retrospective case-control study / 中华风湿病学杂志

ObjectiveInvasive fungal infection(IFI) can be a lethal complication in patients with diffuse connective tissue diseases(DCTD).The aim of this study was to determine the characteristics of hospitalized DCTD patients with IFI,and identify the risk factors.MethodsData from 33 DCTD in patients with IFI at Shanghai Renji Hospital between Jan 2007 and Jan 2011 were collected retrospectively.DCTD patients with either active M.tuberculosis (n=33) or other bacterial infections (n=34) at the same period were taken as controls.Systemic lupus erythematosus (SLE) inpatients with IFI (n=11 ) from Jan 2002 to Dec 2006 were also considered as a historical control group.The method of univariate analysis of data depended on the data distribution type.Variables that suggested association in the univariate analysis P＜0.1 were entered into a stepwise logistic regression model.ResultsThe leading underlying diseases of DCTD with IFI were SLE(n=18,55％),systemic vasculitis(n=4,12％),and inflammatory myopathy(n=4,12％).The most frequent pathogen was Candida spp(n=13,39％ ),followed by Cryptococcus neoformans(n=10,30％ ),and Aspergillus (n=3,9％).The infection locations included lung (n=19,58％),central nervous system (n=9,27％ ),and disseminated IFI(n=4,12％ ).Six patients(18％) died from IFI.Compared with non-IFI infections,patients with IFI infection had a shorter duration of underlying disease and were exposed to high doses of prednisolone prior to infection.More patients with IFI infection had elevated alanine aminotransferase,higher fasting glucose and lower C-reactive protein levels when compared to patients with non-IFI infections.Compared with the two historical SLE-IFI groups, the short-term survival improved in lupus patients complicated with IFI infection over time(64％ vs 83％).ConclusionUnderstanding disease spectrums and risk factors of IFI in DCTD,along with advances in antifungal treatment,will help clinicians to manage those patients with invasive fungal infection effectively to achieve favourable prognosis.

The expression of IKB kinase in the peripheral blood and its correlation with the production of interferon-α in systemic lupus erythematosus patients / 中华风湿病学杂志

Objective To investigate the mRNA expression of IKB kinase (IKK-α) and interferon-α (IFN-α) in the peripheral blood leukocytes of patients with systemic lupus erythematosus (SLE), and to explore the role of IKK-α in the production of IFN-α in SLE patients. Methods SYBR green dye I based real-time quantitative PCR was used to detect the mRNA expression levels of IKK-α and IFN-α in the peripheral blood leucocytes of SLE patients and healthy controls. Serum levels of IFN-α were measured with ELISA method. Results IKK-α mRNA expression levels in SLE patients were significantly higher than those of normal controls (P<0.05). IKK-α mRNA expression levels in SLE patients with active disease were significantly higher than patients with stable disease (P<0.01). IFN-α mRNA expression level in SLE patients was significantly lower than that of the normal controls (P<0.01). IFN-α mRNA expression levels in SLE patients with active disease were significantly higher than patients with stable disease (P<0.01). Serum levels of IFN-α in SLE patients with active disease was significantly higher than that of the normal controls and patients with stable disease (P<0.05). The anti-dsDNA antibody correlated positively, and complement C3 correlated negatively with serum concentration of IFN-α. IKK-α mRNA expression levels in SLE patients correlated positively with serum concentration of IFN-α. Conclusion IKK-α correlates positively with serum concentration of IFN-α. The IFN-α level is significantly correlated with disease activity, This suggests that IKK-α may play an important role in the pathogenesis of SLE.

Clinical characteristics of 174 Chinese patients with antiphospholipid syndrome and comparison with European patients / 中华风湿病学杂志

Objective To analyze the clinical characteristics of antiphospholipid syndrome (APS) in a cohort of Chinese patients. Methods From January 1996 to October 2009, 174 patients with defined APS were retrospectively studied,χ2 test was selected. Results The cohort consisted of 151(86.8%) female and 23 (13.2%) male. Mean age at study was (42±13) years (range: 14-75 years). Thirty-one (17.8%) patients had primary APS, 143 (82.2% ) patients had APS associated with other diseases, including 138 cases of systemic lupus erythematosus (SLE). One hundred and forty-one (81.0%) had thrombosis episodes, among which the most common presenting manifestations were stroke (27.6%), deep venous thrombosis (27.6%) and pulmonary embolism (6.3% ). Stroke was more prevalent in Chinese than European patients. Forty-five patients (31.9%) had thrombotic recurrences and 62 patients (44.0%) had more than two sites of thrombosis. Patients with primary APS had higher rates of rethrombosis than those with secondary APS (P<0.05). Fetal morbidity rate of 126 married women in our study was 50.0%. Seven of APS patients with APS nephropathy were characterized by thrombotic microangiopathy. The presence of anticardiolipin antibodies was detected in 112 patients (64.4% ), anti-beta-2 glycoprotein Ⅰ antibodies in 103 patients (59.1%) and lupus anticoagulant in 50 patients (28.7%). Conclusion APS is characterized by recurrent thrombosis, pregnancy morbidity and positive antiphospholipid antibodies tests. Stroke, deep venous thrombosis and pulmonary embolism are the most common manifestations both in Chinese and European patients. Rethrombosis is more prominent in primary APS. Thrombotic microangiopathy is one of the most common histological changes of APS nephropathy. Multiple tests for aPL are proposed.

The significance of antibodies to anti-coagulation related factors and anti-phospholipid antibodies in glomerular microthrombosis in lupus nephritis:a prospective study on 124 patients / 中华风湿病学杂志

Objective To investigate the prevalence of glomerular microthrombosis in lupus nephritis (LN) and the significance of antibodies to anti-coagulation related factors and anti-phospholipid antibodies in glomerular microthrombosis (GMT).Methods Kidney biopsy specimens and plasma samples were obtained consecutively from 124 patients with LN. Kidney biopsy specimens were examined for the presence of glomerular microthrombi.Plasma samples from 25 LN patients with GMT (LN-GMT group) and 99 LN patients without GMT (LN-non-GMT group) were tested for lupus anticoagnlant (LA) and antibodies to cardiolipin (ACL),β2 glycoprotein I (β2GP I ),plasmin,thrombin,tissue plasminogen activator (t-PA) and Annexin A II.Results The prevalence of GMT in LN patients was about 20.2%.Compared to LN-non-GMT group,LN-GMT group had elevated SLE disease activity indices (SLEDAI),elevated activity and chronicity indices of kidney tissue injury,and elevated serum creatinine,blood urea nitrogen and proteinuria levels,and also had a higher frequency of hypertension (P＜0.01).The positive rates of LA,IgG class anti-β2GP I and anti-thrombin antibodies were higher in LN-GMT group than in LN-non-GMT group (P＜0.05).The positive rates of IgG class antibodies to ACL,plasmin,t-PA and Annexin A II in LN-GMT group were not statistically different from those in LN-non-GMT group (P＞0.05).No difference was found in the positive rate of any IgM class antibody between the two groups (P＞0.05).Conclusion This study has shown that GMT occurs approximately in 20.2% of the LN patients.Patients with GMT have more severe kidney tissue injury and more poor renal outcomes than patients without GMT.LA and antibodies to β2GP I and thrombin play a role in glomerular microthrombosis in lupus nephritis.

The role of anti-annexin A2 antibodies in antiphospholipid syndrome / 中华风湿病学杂志

Objective This study has explored the role of antibody against annexin A2 in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). Methods Using purified recombinant annexin A2, IgG anti-annexin A2 antibody was measured by ELISA in 101 APS patients, 41 SLE patients with thrombosis, 124 SLE patients without thrombosis and 120 healthy controls. Results The positive rate of IgG anti-annexin A2 antibody in APS patients and SLE patients with thrombosis was 21.8%, 26.8%, respectively, they were all significantly higher than in SLE patients without thrombosis (6.5%). IgG anti-annexin A2 antibody was associated with thrombosis and/or pregnancy morbidity (P<0.01). Conclusion Anti-annexin A2 antibody is associated with thrombosis and/or pregnancy mnrbidity. It suggests that anti-annexin A2 antibody may be helpful in identifying in some potential AIRS.

Causes of fever in systemic lupus erythematosus: a retrospective study of 487 hospitalized patients / 中华风湿病学杂志

Objective To investigate the causes of fever in systemic lupus erythematosas (SLE)patients and identify the characteristics of the fever caused by SLE fever infection after steroid therapy.Methods From January 2002 to May 2007, 487 SLE hospitalized patients with fever were admitted and their data were retrospectively studied. The clinical manifestations and medications were followed up. A case control design was carried out to identify the differences between fever caused by lupus and infection. Results Among all the 487 patients, 265 had infection, 206 had fever that was caused by SLE per se, fever caused by both lupus and concurrent infection was confirmed in 8 patients, malignancy and miscellaneous were the causes of fever in 4 respectively. The most common sites of infection were respiratory tract (61.3%), skin and mucosa (10.9%) and central nervous system (9.1%). The main microorganisms isolated were bacteria (77.4%), fungi (13.5%) and virus (7.8%). Prednisene ≤ 100 mg/d could suppress fever caused by lupus in 81.0% patients, usually within 1 to 5 days. In the case control study, lower C3, C4 and CH50, anemia, lymphocytosis, higher immunoglobulin levels, elevated anti-dsDNA antibodies, elevated anti-nuclear antibodies and higher SLEDA1 score were associated with SLE fever. Long SLE duration, old age, elevated CRP levels,leukocytosis, neutrophilia, high accumulated doses of prednisone and ever use of immunosuppressive medications were factors associated with infection. Conclusion The fever of in-patient SLE patients is mainly caused by infections and disease flare. Moderate dose of steroid therapy is effective to control the fever caused by lupus flare. The fever caused by SLE has different clinical features and laboratory results when compared to that caused by infection although both responses to steroid therapy.

Clinical characteristics of 115 Chinese patients with antiphospholipid syndrome and the analysis of different classification criteria / 中华风湿病学杂志

Objective To analyze three different classification criteria, the clinical characteristics of antiphospholipid syndrome(APS)in a cohort of Chinese patients. Methods From January 1996 to October 2006, APS patients diagnosed with different classification criteria were retrospectively studied. Results There were totally 120 APS patients fulfilled at least one criterion, One hundred and one patients fulfilled the 1988 Asherson criteria, 96 patients fulfilled the 1999 Sapporo criteria, and 115 patients fulfilled the 2006 Sydney criteria. The ratio of male to female in a cohort of 115 definite APS patients was 1 to 10.5. The mean period of the disease until entry into the study was 82.6 months, the mean age at study entry was(41?12)years. Ninety patients had thrombosis episodes, among which the most common presenting manifestations were deep venous thrombosis, stroke and skin vasculitis. Forty-six of 92 married women in our cohort had fetal morbidity. Catas- trophic APS occurred in 7 patients. The presence of anticardiolipin antibodies(aCL)was detected in 86 pa- tients, anti-beta-2 glycoproteinⅠantibodies in 58 patients and lupus anticoagulant(LA)in 27 patients. Conclusion The most common presenting manifestations are deep venous thrombosis, stroke and cutaneous manifestations. The sensitivity of Sydney classification criteria is improved by adding anti-beta-2 glycopreteinⅠantibody as one of the laboratory criteria. However, primary APS patients who only presented with thrombo- cytupenia and positive laboratory tests could not satisfy this criterion. In addition, the significance of autoanti- bodies to some coagulant factors in APS needs further study.