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1.
Article in Chinese | WPRIM | ID: wpr-911443

ABSTRACT

Objective:To study clinical characteristics and pregnancy outcomes under anti-coagulation therapy of non-criteria obstetric antiphospholipid syndrome.Methods:Patients suspected of obstetric antiphospholipid syndrome(OAPS) were recruited through Chinese Rheumatism Data Center from 2015 to 2019 consecutively. Patients fulfilling 2006 Sydney revised antiphospholipid syndrome criteria were classified as OAPS. Patients fulfilling definition of non-criteria OAPS(NCOAPS) by expert consensus on diagnosis and management of obstetric antiphospholipid syndrome of China were classified as NCOAPS. Clinical characteristics and laboratory results of two groups were compared. Live birth rates and pregnancy outcomes under anti-coagulation therapy were studied.Results:A total of 88 patients were enrolled, including 56 patients (63.6%) as OAPS, 32(36.4%) as NCOAPS. Live births were only reached in 16.1% (9/56) in OAPS patients and 12.5%(4/32) in NCOAPS. Fetal losses after 10 weeks of gestation and pre-eclampsia before 34 weeks were more common in OAPS group compared to NCOAPS group [78.6%(44/56) vs. 18.8%(6/32), P<0.001; 25.0%(14/56) vs. 3.1%(1/32), P=0.020, respectively]. After enrollment, 15 pregnancies were recorded in OAPS, 10 in NCOAPS, all of whom were treated with low-dose aspirin (LDA) combined with low-molecular weight heparin (LMWH). Live birth rates saw dramatic improvements compared to baseline levels in OAPS [16.1% (9/56) vs. 11/15] along with NCOAPS [12.5% (4/32) vs. 7/10]. Conclusion:Though NCOAPS and OAPS patients differ in antiphospholipid antibody spectrum and pattern of pregnancy morbidities, both groups benefit from LDA combined with LWMH treatment, as live birth rates improve. Non-criteria OAPS patients are recommended to receive anti-coagulation therapy during pregnancy.

2.
Article in Chinese | WPRIM | ID: wpr-799873

ABSTRACT

Objective@#Portal vein thrombosis (PVT) is a rare and severe clinical manifestation of antiphospholipid syndrome (APS), as well as a predictor of poor prognosis. This study was conducted to explore the clinical features and risk factors of PVT in APS patients.@*Methods@#A total of 123 APS patients diagnosed from 2012 to 2019 were retrospectively enrolled. The diagnosis of PVT was made according to the 2009 American College of Liver Diseases (AASLD) criteria. Clinical and laboratory data were collected. A multivariate (MV) logistic regression model was constructed using a stepwise forward selection procedure among those candidate univariables with P values<0.10.@*Results@#A total of 28 cases with PVT, and 95 control cases without PVT were finally enrolled.The 28 APS-PVT patients included 5 males and 23 females with age range from 17 to 63 years. Clinical manifestations included acute thrombosis in 8 patients, chronic thrombosis in 16, and 4 with portal vein spongiform. As to the involved vessels, single portal vein thrombosis was seen in 20 patients, portal combined with superior mesenteric vein (SMV) and splenic vein in one patient, portal plus SMV in 4 and only SMV in 3 patients. Other manifestations were portal hypertension (16/28), esophageal varices (13/28), spleen infarction (7/28) and gastrointestinal bleeding (4/28). Two antiphospholipid antibodies were positive in 13 cases. Triple positive antibodies were seen in 7 cases. Multivariate logistic regression analysis showed that disease duration less than 0.5 years (OR=72.74, 95%CI 7.50-705.45, P<0.001), hypoalbuminemia (OR=356.45, 95%CI 19.19-6 620.14, P<0.001), and elevated erythrocyte sedimentation rate (ESR)/C-reactive protein (CRP) (OR=14.41, 95%CI 1.49-139.20, P<0.001) were independent risk factors for PVT in APS.@*Conclusion@#PVT is usually misdiagnosed due to insidious onset. Short disease duration, hypoalbuminemia and elevated ESR/CRP are risk factors for PVT in APS. Better understanding, early diagnosis and treatment will improve the clinical outcome.

3.
Article in Chinese | WPRIM | ID: wpr-755740

ABSTRACT

Objective To summarize the neurological manifestations in patients with primary Sj?gren′s syndrome (pSS). Methods A total of 68 patients were diagnosed as pSS in neurology department of Peking Union Medical College Hospital from March 2014 to February 2018, among whom sixteen cases were excluded due to modified final diagnoses of primary neurological diseases. Therefore 52 pSS patients with neurological involvement were enrolled and retrospectively analyzed. They were divided into two groups as extensive group in which both central and peripheral nervous system were involved, non?extensive group in which either central or peripheral nervous system was involved. Results Neurological manifestations were presented as primary symptoms in 98.1%(51/52) patients, while 35 had neurological involvement as their only extraglandular manifestations. Thirteen cases were in extensive group. The other 39 in non?extensive group including 22 cases with only peripheral nervous system involved and 17 cases with only single central nervous system involved. Compared to non?extensive group, the proportion of woman patients [13/13 vs.71.8% (28/39),P=0.047], serum IgG level [17.73(11.11,22.41)g/L vs. 11.49(9.58,13.40)g/L, P=0.017] and positive rates of oligoclonal band (OB) in cerebral spinal fluid (CSF) [7/13 vs. 22.6%(7/31), P=0.042)] were significantly higher in extensive involvement group. Conclusions Neurological manifestations in pSS patients could be extensive, both central and peripheral nervous system might be associated. Female patients, high serum IgG level and positive OB in CSF are risk factors of extensive neurological involvement, suggesting that the immune system may be generally over?stimulated.

4.
Article in Chinese | WPRIM | ID: wpr-745744

ABSTRACT

Objective To explore the predictive factors for determining the therapeutic response and prognosis of severe thrombocytopenia (TP) in patients with primary Sj(o)gren syndrome(pSS).Method Patients with pSS and severe TP (platelet count ≤ 50× 109/L) admitted between 2010 to 2016 at Peking Union Medical College Hospital were classified according to their therapeutic response and analyzed retrospectively.The response parameters and clinical data including bone marrow aspiration results and laboratory findings were collected.Result Thirty patients were finally analyzed,including twenty with appreciable bone marrow aspiration results.Fourteen and 7 patients achieved a complete response (CR) and a partial response (PR) respectively,other 9 patients with no response (NR).The megakaryocyte counts in bone marrow (BM-MK) counts per slide in each group were 13.0 (9.2,23.5) in CR patients,7.0 (7.0,20.0) in PR patients,and 5.0 (1.0,6.0) in NR patients.BM-MK counts in patients with clinical response (CR+PR)were significantly higher than those with NR (P=0.006).A receiver-operation characteristic analysis revealed a cutoff value of BM-MK counts at 6.5 per slide stratifying patients by different responses with a sensitivity of 13/14,a specificity of 6/7,and area under the curve of 0.879.Univariate analysis indicated a better prognosis as BM-MK counts>6.5 per slide.Conclusion BM-MK count could be a predictive factor of response in patients with pSS and severe TP.Patients with BM-MK counts≤6.5 per slide represent worse platelet improvement..

5.
Article in Chinese | WPRIM | ID: wpr-745737

ABSTRACT

Objective To analyze the clinical characteristics of antiphospholipid syndrome (APS) patients with chronic thromboembolic pulmonary hypertension (CTEPH).Methods A total of 22 APS patients with CTEPH were enrolled in our study,who were admitted in Peking Union Medical College Hospital from January 2012 to August 2018.Diagnoses were confirmed by computed tomographic pulmonary angiography (CTPA),or pulmonary angiography.Demographic characteristics,clinical manifestations,laboratory tests,therapy,World Health Organization (WHO) functional class were retrospectively collected.Results There were 15 females and 7 males with a median age of 29-year-old.Chest pain (6 cases),dyspnea on exertion (22 cases),cough (6 cases) and hemoptysis (9 cases) were the most common clinical manifestations.Lupus anticoagulant (LA),anticardiolipin (ACL) antibodies and anti-beta 2 glycoprotein Ⅰ (anti-β2 GP Ⅰ) antibodies were all positive in 12 patients,two of three antibodies positive in 5 patients,only one positive in 5 patients.The WHO functional classes were Ⅱ-Ⅳ before treatment.Anticoagulants were administrated in all patients.After multidisciplinary evaluation,9 patients underwent pulmonary thromboendarterectomy (PTE),who all had a good outcome.Symptoms in eleven over thirteen patients with only anticoagulants improved.Three patients developed cardiac deterioration while other 3 patients died of right heart failure during follow-up.Conclusion Pulmonary embolism is one of the most common thrombotic events in APS patients.It is important to recognize symptoms and signs related to pulmonary embolism and start anticoagulation as soon as possible.Standard anticoagulation improves symptoms but can't reverse the process of pulmonary hypertension.Some patients may benefit from PTE after anticoagulation and multidisciplinary evaluation.

6.
Chinese Journal of Rheumatology ; (12): 376-380, 2018.
Article in Chinese | WPRIM | ID: wpr-707866

ABSTRACT

Objective To investigate the value of antineutrophil cytoplasmic antibody (ANCA) in clinical phenotype of eosinophilic granulomatosis with polyangiitis (EGPA).Methods The clinical data of 64 patients with EGPA from Peking Union Medical College Hospital between 2007 to 2016 were retrospectively analyzed,and the patients were followed up.Characteristics of patients with ANCA positive and ANCA negative were compared by independent-samples t test,Mann-Whitney U test and Chi-square test.Results Among 64 patients with EGPA,12(19%) were serum ANCA positive and 52(81%) were negative.The incidence of fever (77% vs 35%,x2=9.403,P=0.002) and renal involvement,including proteinuria (67% vs 25%,x2=7.678,P=0.006),hematuria (58% vs 8%,x2=17.57,P<0.01),renal inadequacy (33% vs 4%,x2=9.978,P=0.002),and the BVAS score higher than 15 (92% vs 60%,x2=4.440,P=0.035) in ANCA positive group were higher than ANCA negative group,while the presence of allergic rhinitis (17% vs 56%,x2=5.969,P=0.015),mucocutaneous lesion (33% vs 65%,x2=4.152,P=0.042) and cardiac involvement (8% vs 44%,x2=3.361,P=0.021) in the ANCA-positive group was lower when compared with ANCA-negative patients.The positive ratio of rheumatoid factor (RF) (100% vs 42%,x2=7.723,P=0.006),and the level of erythrocyte sedimentation rate (ESR) (50 vs 35.5 mm/1 h,P=0.034) in ANCA-positive group were higher than in ANCA negative group.There was no significant difference in pathological characteristics between the two groups.According to the treatment and prognosis,there were no significant differences between the two groups in the usage and dosage of steroids and immunosuppressant,the remission rate and recurrence rate of the disease,and the death rate due to the primary disease.Conclusion The clinical manifestations of EGPA are complicate.Whether ANCA is positive or not may be related to the clinical phenotypes.More attention should be paid to renal involvement in ANCA positive patients while cardiac involvement in ANCA negative patients.

7.
Article in Chinese | WPRIM | ID: wpr-710116

ABSTRACT

A 45-year-old woman was admitted to the Department of Rheumatology and Immunology, Peking Union Medical College Hospital, due to weakness of the upper limbs, fever, and blurred vision. She was clinically diagnosed as systemic lupus erythematosus overlapped primary biliary cirrhosis, with renal, retinal, hematological and musculoskeletal involvement, combined with severe pulmonary infection and respiratory failure. Treated with glucocorticoids, ursodeoxycholic acid, antibiotics and respiratory support, the patient got better. A couple of days later, her fever recurred and platelets count dropped to 30×109/L, hemoglobin to 78 g/L, fibrinogen to<1.5 g/L, ferritin to 1 640 ng/ml, natural killer (NK) cell count to 8/μl, the activity of NK cells 2% (reference value 9.5%-23.5%), considering the occurrence of hemophagocytic lymphohistiocytosis (HLH). Cytomegalovirus pp65 antigenemia test: 13 positive cells/2×105 WBC. Considered the possibility of HLH caused by cytomegalovirus infection and treated by 250 mg ganciclovir intravenous drip twice a day for a full course. The temperature of the patient was gradually reduced to 36.5℃, the count of platelets were increased to 229×109/L, the hemoglobin was increased to 94 g/L, and the fibrinogen was increased to 3.26 g/L. When there were unexplained critical signs of the primary disease during systemic lupus erythematosus treatment, severe complications such as infection, HLH, thrombotic thrombocytopenic purpura should be taken into account.

8.
Article in Chinese | WPRIM | ID: wpr-663412

ABSTRACT

An 61-year-old woman presenting deep vein thrombosis and persistent positive anticardiolipin antibodies was diagnosed as antiphospholipid syndrome and treated with low molecular weight heparin. Before and after anticoagulant therapy, continuous positive fecal occult-blood was found asymptomatically. Colonoscopy confirmed rectal cancer. Antiphospholipid autoantibodies are non-specially positive in some malignances, especially in elder onset patients. Thus, routine screening of malignancies is strongly suggested.

9.
Article in Chinese | WPRIM | ID: wpr-612277

ABSTRACT

A 54-year-old man was admitted to the Department of Rheumatology in Peking Union Medic.al College Hospital due to polyarthritis.back pain and multiple subcutaneous masses.He had symmetrical polyarthritis at onset.which was diagnosed as theumatoid arthritis.His follow-up and treatment were irregular.Back pain and multiple subcutaneous masses developed 2 year ago.Erythrocyte sedimentation rate and C-reac.tive protein were elevated.Rheumatoid fac.tor and Anti-cylic citrullinated peptide antibody were positive.No evidence of infection or malignancy was detected.Chest CT suggested pulmonary fibrosis and osteolytic lesions of stemum.Spinal MRI showed paravertebral soft tissue.Biopsy of the soft tissue suggested the pathologic.al finding of a theumatoid nodule.The patient was diagnosed as theumatoid arthritis complic.ated with multiple theumatoid bursal cysts.Combination therapy of methylprednisolone (40mg/d) and cyclophosphamide (0.4g/w) lead to a rapid improvement of clinical symptoms and laboratory parameters.The patient was still in remission at 6-month follow-up.

10.
Article in Chinese | WPRIM | ID: wpr-611675

ABSTRACT

Objective To evaluate the clinical performance of chemiluminescent immunoassay (CLIA) on anti-nuclear antibody(ANA) specific autoantibodies testing.Methods A multi-center clinical study A total of 811 Sera samples were collected from 6 collaborating hospitals during the period of April to July 2016, and tested with CLIA and line immunoassay (LIA) in parallel for autoantibodies to ribonucleoprotein(RNP), smith antigen(Sm), SSA/Ro60,SSB/La, centromere protein B(CENPB), double-stranded DNA(dsDNA), nucleosome(Nuc), and ribosome P protein(Rib-P).The positive rate,specificity and qualitative coincidence rate for each antibody between CLIA and LIA methods were analyzed.All discrepant samples for systemic lupus erythematosus (SLE) highly specific autoantibodies (including anti-Sm, dsDNA, Nuc and Rib-P) were retested by enzyme linked immunosorbent assay (ELISA) and further analyzed with SLE disease cohort using McNemar test.Results The positive rate and specificity of CLIA and LIA for antibodies to ANA specific antigens were comparable.Excellent qualitative coincidence were found between CLIA and LIA for the detection of anti-RNP, SSA/Ro60, SSB/La and CENPB (Kappa>0.75), while the coincidence rate foranti-Sm, dsDNA, Nuc and Rib-P detection were moderate (0.4

11.
Article in Chinese | WPRIM | ID: wpr-511089

ABSTRACT

To investigate the etiology and differential diagnoses of patients with systemic lupus erythematosus (SLE) and fever of unknown origin (FUO).From January 2012 to December 2014,a total of 928 SLE patients were admitted to Peking Union Medical College Hospital.Only 50 patients were combined with FUO (5.4%).The most common reason of fever was caused by infections (33 cases,66.0%),including bacterial infection in 17 cases with 5 tuberculosis,viral infection in 11 cases,and fungal infection in 5 cases.The second reason offever was due to poor disease control or recurrence in 17 patients (34.0%).No fever was caused by malignant tumor.When clinical data was compared between 17 non-infected patients versus 33 infected patients,C reactive protein and procalcitonin in the infected group were significantly higher than those in the non-infected group.In SLE patients combined with FUO,infection is the most common etiology which is necessary to be paid attention to.

12.
Chinese Journal of Rheumatology ; (12): 148-153, 2016.
Article in Chinese | WPRIM | ID: wpr-670164

ABSTRACT

Objective To investigate the levels, phenotypes and functions of regulatory T cells (Treg) and Th17 cells in systemic sclerosis (SSc) patients and assess the role of imbalance between Treg and Th17 cells in the pathogenesis of SSc. Methods Peripheral blood mononuclear cells (PBMCs) of 31 SSc patients and 33 healthy controls were analyzed for the expression of CD4, CD25, CD45RA, the cytotoxic T-lymphocyte antigen-4 (CTLA-4), FoxP3, and IL-17 using flow cytometry. Treg immunosuppression capacities were measured in co-cultured experiments. The expressions of IL-17A, IL-22, IL-6 and IL-1β were measured by FlowcytoMix. The expression of FoxP3, CTLA-4, IL-17A, and RORC mRNA were measured by real-time polymerase chain reaction (PCR). The independent samples t-test was used to compare data between the groups. Results The frequency of Treg cells was significantly elevated in SSc [(3.6 ±1.1)%] compared with controls [(2.0±0.8)%, t=6.88, P<0.01)] and the expression of CTLA-4 was lower in Treg cells of SSc (P=0.034). The expression of CTLA-4 and FoxP3 mRNA was lower in SSc compared with controls (P<0.05), the immunosuppressive capacity of Treg cells were diminished in SSc (P=0.034). Th17 cells were increased in SSc (P<0.01); the levels of IL-17A, IL-22, IL-6 and IL-1β in the serums of SSc were higher than the controls (P<0.05). In SSc, CD25+FoxP3+IL-17+cells were increased[0.075%±0.032)%vs (0.049±0.027)%, t=3.52, P=0.029], but no difference of CD25+FoxP3+IL-17- was observed when compared with control. The expression of IL-17A and RORC mRNA were increased in SSc (P<0.01, respectively). Conclusion Both Treg and Th17 cells are increased in patients of SSc. Treg increases along with the deficiency of phenotype and function. FoxP3+IL-17+co-express cells are increased, and the balance between Treg and Th17 cells is broken.

13.
Article in Chinese | WPRIM | ID: wpr-502477

ABSTRACT

Objective To investigate the clinical features of macrophage activation syndrome (MAS) associated with systemic lupus erythematosus (SLE).Method The clinical data of 15 patients with SLE-induced MAS diagnosed in Peking Union Medical College Hospital from July 2011 to December 2014 were retrospectively analyzed.Results Fourteen patients were female.The average age was 28.07.When MAS occurred,the average duration of SLE was 20.47 months,and the average SLE disease activity index (SLEDAI) was 18.4.All 15 patients developed fever,hematocytopenia and impaired liver function in the course of MAS,while patients with splenomegaly,coagulation disorders and neuropsychiatric symptoms were 11,14 and 8,respectively.All 15 patients presented leukocpenia and thrombocytopenia.Hypofibrinogenemia,elevated ferritin and hemophagocytosis in bone marrow were respectively observed in 7,11 and 12 patients.Glucocorticoids were used in all patients,among whom eight received pulse methylprednisolone therapy.Thirteen patients were treated with immunosuppressants,including cyclosporine A,tacrolimus,cyclophosphamide and mycophemolate mofetil.Complete remission was achieved in 14 patients.One patient died of MAS.Conclusions In patients with SLE,MAS was most commonly seen in young females with short SLE duration and active disease.Fever,splenomegaly,hematocytopenia,coagulation disorders and liver damage are the most remarkable clinical manifestations.Early diagnosis and intensive therapy are the key parts to improve clinical outcome.

14.
Article in Chinese | WPRIM | ID: wpr-488782

ABSTRACT

Objective To investigate the clinical characteristics in patients with primary antiphospholipid syndrome (PAPS) and to identify potential predictors of thrombotic events.Methods A total of 107 patients with PAPS were enrolled in our study, who were admitted in Peking Union Medical College Hospital from January 2004 to December 2014.Demographic data, age at onset, disease duration, past history of hypertension and regular cigarette smoking, clinical manifestations, imaging characteristics, management and prognosis were retrospectively collected.Bivariate statistical analysis and logistical regression test were performed to compare the discrepancy between patients with or without thromboembolic events.Results In 107 patients, there were 65 female and 42 male patients, with mean age (39.8 ± 15.8) years old, median disease duration 10.5 (2.0, 48.0) months.A total of 72(67.3%) patients reported episodes of thromboembolic events, including 72 venous thromboses and 29 arterial thromboses.The most frequent venous thromboses were deep vein thromboses (35.5%), pulmonary embolism the second common (29.9%), with cranial venous sinus thromboses the following (8.4%).In arterial thromboembolic events, the incidence of transient ischemic attack (TIA) and ischemic stoke was the highest (14.0%), embolism of lower extremities the second (6.5%) ,and 4 patients (3.7%) with acute myocardial infarction.Sixty seven patients (62.6%)had positive lupus anticoagulant, 60 patients (56.1%)with positive anticardiolipin antibody,32 patients (29.9%, 32/74) with positive β2 glycoprotein Ⅰ (β2GP I).Forty patients(37.4%)had double positive antibodies, while 19 cases (17.8%)with triple positive.In logistical regression, aging (per 10 years) and hypocomplementemia were significantly related to venous thrombosis (OR =1.421, 95% CI 1.066-1.894, P < 0.05, and OR =6.435, 95% CI 1.374-30.130, P < 0.05, respectively).Cigarette smoking and triple positive antibodies were independent risk factors of arterial thrombosis (OR =3.996, 95% CI 1.079-14.795, P < 0.05 and OR =3.166, 95% CI 1.102-9.097, P < 0.05, respectively).Conclusion Alas is an autoimmune disorder characterized by recurrent arterial and venous thromboembolic events.Venous thromboembolism is more common than the arterial.Age and hypocomplementemia are predictors of venous thromboembolism;while smoking and triple positive antibodies are independent risk factors of arterial thromboembolism.

15.
Chinese Journal of Cardiology ; (12): 56-61, 2015.
Article in Chinese | WPRIM | ID: wpr-303767

ABSTRACT

<p><b>OBJECTIVE</b>To observe the prevalence and related risk factors of arterial stiffness measured by brachial-ankle pulse wave velocity (baPWV) in patients with systemic lupus erythematosus (SLE).</p><p><b>METHODS</b>The study population included 135 patients currently enrolled in the Chinese SLE Treatment and Research group registry (CSTAR). All traditional cardiovascular risk factors and SLE-related disease factors were collected on the day of the baPWV examination.</p><p><b>RESULTS</b>(1)Significant differences were observed in age (P < 0.000) , family history of cardiovascular disease (P = 0.003), mean blood pressure (P = 0.000) and hemoglobin A1c (P = 0.023) between SLE patients with normal and abnormal arterial stiffness. In addition, SLE patients with abnormal arterial stiffness had lower creatinine clearance rates [85.9 (65.5-108.8) ml/min vs. 106.4 (86.8-124.6) ml/min, P = 0.011], longer disease and hydroxychloroquine duration (P = 0.002 and P = 0.022, respectively), and higher proportion of intravenous cyclophosphamide use (OR = 3.04, 95%CI:1.230-7.514, P = 0.013) as compared to patients with normal arterial stiffness. (2)After adjustment of all confounding factors, age (OR = 4.56, 95%CI: 1.863-11.133, P = 0.001), mean blood pressure (OR = 1.12, 95%CI: 1.055-1.196, P = 0.000) , disease duration (OR = 1.12, 95%CI: 1.050-1.367, P = 0.007) and the proportion of intravenous cyclophosphamide use (OR = 2.86, 95%CI: 1.364-5.979, P = 0.005) remained as independent risk factors for abnormal arterial stiffness in SLE patients.</p><p><b>CONCLUSION</b>Both traditional cardiovascular risk factors and SLE-related factors are associated with the risk of increased arterial stiffness.</p>


Subject(s)
Ankle , Ankle Brachial Index , Asians , Blood Flow Velocity , Cardiovascular Diseases , Humans , Incidence , Lupus Erythematosus, Systemic , Prevalence , Pulsatile Flow , Pulse Wave Analysis , Risk Factors , Vascular Stiffness
16.
Chinese Journal of Rheumatology ; (12): 335-338, 2015.
Article in Chinese | WPRIM | ID: wpr-466192

ABSTRACT

Objective To investigate the clinical characteristics and related pathogenetic factors in Takayasu arteritis with myocardial involvement.Methods We retrospectively analyzed clinical data of Takayasu arteritis patients with myocardial involvement of Peking Union Medical College Hospital from 1987 to 2013.Results Myocardial involvement was diagnosed in 23 patients from 86 Takayasu arteritis patients.Sixty-five percent (15/23) of patients presented with congestive heart failure.Fifteen patients were without coronary artery involvement.Sixty-five percent (15/23) of patients with mild to moderate hypertension.Seventyfour percent(17/23) patients had pulmonary hypertension.Left ventricular ejection fraction was (34.70 ±0.09)%.Glucocorticco costeroid and immunosuppressive therapy could significantly increase left ventricular ejection fraction (t=4.302,P<0.05).Conclusion We emphasize that myocardial involvement is common in Takayasu arteritis.It is an independent risk factor that impacts the prognosis.Early detection of myocardial involvement and effective therapy can improve the prognosis.

17.
Chinese Journal of Rheumatology ; (12): 724-729,后插1, 2015.
Article in Chinese | WPRIM | ID: wpr-603007

ABSTRACT

Objective To figure out the effect of resveratrol (Res) in skin and lung pathology of systemic sclerosis (SSc) animal model and find a new target of anti-fibrosis therapy in SSc.Methods First, we establish ed SSc animal model by daily subcutaneous injection of bleomycin (BLM) for 4 weeks in BALB/c mice.Then we fed the mice with Res.We observed the pathological changes in skin and lung and the expression of the deacetylase SIRT1.We observed the following parametrs.The pathologicalchanges in injected skin and lung which shown by hematoxylin-eosin (HE) staining, the expression of α-smooth muscle actin (α-SMA) in injected skin and lung which measured by immunohistochemistry, the expression of SIRT1 and pro-collagen Ⅲ mRNA which assessed by Real Time polymerase chain reaction (PCR).For the homosce dasticity data.We used one-way analysis of variance (ANOVA) and LSD-t test to compare between the groups.Results Daily subcutaneous injection of BLM for 4 weeks in Balb/c mice could successfully establish a mouse model of SSc.The thickening of skin and alveolar septum, the infiltration of inflammatory cells in lung, and even fibrosis insome area of lung could be observed.The number of α-SMA positive cells and the expression of pro-collagen Ⅲ mRNA were increased (P<0.05).Meanwhile, the expression of SIRT1 mRNA was decreased [the number of α-SMA positive cell: skin 26.4±5.9 vs 4.4±2.2, lung 14.6±4.6 vs 2.4±1.1, cells per view, P<0.01;the expression of pro-collagen Ⅲ mRNA: 1.06±0.24 vs 0.45±0.14, relative to glyceraldehyde phosphate dehydrogenase (GAPDH), P<0.05].Meanwhile, the expression of SIRT1 mRNA was decreased (1.01±0.51 vs 5.03±1.59, relative to GAPDH, P<0.05).Treated with Res, the pathological changes in skin and lung were alleviated and the number of α-SMA positive cells in lung and skin was decreased [skin 26.4±5.9 vs 10.0±3.5 (high dosage group), 26.4±5.9 vs 13.4±4.4 (medium dosage group);lung 14.6±4.6 vs 8.8±3.5 (high dosage group), cells per view, P<0.05].There was were no significant differences in the expression of SIRT1 and pro-collagen Ⅲ mRNA in lung after treated by Res.Conclusion Daily subcutaneous injection of BLM for 4 weeks could successfully establish a mouse model of SSc.Res could be a new medicine for the treatment of SSc.

18.
Chinese Journal of Immunology ; (12): 1074-1076,1081, 2015.
Article in Chinese | WPRIM | ID: wpr-602270

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Objective:To investigate the possibility of establishing a new animal model of pulmonary arterial hypertension by immunizing rat with moesin, detecting the anti-moesin antibody and measuring the mean pulmonary arterial pressure ( mPAP ) . Methods:35 male SD rats were divided into 5 groups, namely, positive control group, blank control group, adjuvant control group, low-dose moesin(250 μg per time) immunized group and high-dose moesin(500 μg per time) immunized group.Each control group had 5 rats, while the 2 immunized groups had 10 rats respectively.The mPAP was measured and the pathological changes of the lung were studied at the endpoint of the study.Results:All the rats immunized by moesin produced anti-moesin antibody.At the endpoint, low-dose and high-dose immunized group had a significantly higher mPAP(20.6±2.9 mmHg, 20.7±2.3 mmHg, respectively) than blank control(15.5 ±0.6 mmHg, P=0.002, 0.001, respectively) and adjuvant control (17.2 ±1.6mmHg, P=0.03, 0.013, respectively).There was no difference between adjuvant and blank control(P=0.93).The mPAP of both immunized groups was not as high as the positive control(33.9±4.7 mmHg,P<0.001,P=0.001, respectively).Pathological study indicated that the immunized rats showed arterial wall thickening and muscularization, as well as inflammation around the vessel, which was similar to the positive control.Conclusion:Our pilot study showed moesin could induce rat to develop PAH.Moesin immunized rat could be a new animal model, which could mimic better the pathogenesis of connective tissue disease associated pulmonary arterial hypertension.

19.
Article in Chinese | WPRIM | ID: wpr-468598

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Objective To analyze the clinical features,laboratory tests,treatments and outcome of patients with scleroderma renal crisis (SRC).Methods We retrospectively reviewed the clinical and laboratory data of 16 patients with scleroderma renal crisis in Peking Union Medical College Hospital from May 2004 to May 2013.The treatment and outcome of SRC patients were also retrospectively analyzed.Results There were a total of 16 SRC patients including 5 male patients and 11 females.The median age at SRC onset was (49.9 ± 12.3) years.It usually took 3.2 years from the diagnosis of systemic sclerosis(SSc) to SRC attack.Ten SRC patients belonged to diffuse cutaneous systemic sclerosis (dcSSc),and 6 patients were limited cutaneous systemic sclerosis (lcSSc).Among SRC patients,16/16 were negative of anticentromere antibodies(ACAs).All these 16 patients had hypertension and renal insufficiency,including 8 dialysis dependent after the onset of SRC and 7 with thrombotic microangiopathy.There were 3 patients receiving renal biopsy.The pathological findings were mainly summarized as intimal thickening and stenosis of renal arterioles.Among 13 patients with long-term followed-up,11 patients received angiotensin converting enzyme inhibitors(ACEI),5 patients died,2 patients were dialysis dependent.Only 1 patient stopped dialysis after the combination treatment of ACEI and endothelin receptor antagonist.Another 5 patients didn't need dialysis.Conclusion SRC usually occurred at the early course of SSc.dcSSc was more frequent than lcSSc.ACAs were rarely found in SRC patients.The immediate and sufficient use of ACEIs was still the cornerstone of SRC treatment.Future studies are needed to evaluate the efficacy of endothelin receptor antagonist in the treatment of SRC.

20.
Chinese Medical Journal ; (24): 3557-3561, 2014.
Article in English | WPRIM | ID: wpr-240728

ABSTRACT

<p><b>BACKGROUND</b>Systemic sclerosis (SSc) is an autoimmune disease that has three major components: inflammation, fibrosis, and vasculopathy. T-helper 17 cell (Th17) and regulatory T cell (Treg) are considered to be critical for autoimmune disease pathogenesis. The role of Th17 and Treg in SSc is still unclear. The aim of this study was to detect the presence of Th17s and CD4(+)CD25(+) Tregs in peripheral blood samples from SSc patients and to investigate the possible roles of these two T cell subsets in SSc pathogenesis.</p><p><b>METHODS</b>Th17s (CD4 and IL-17 positive) and CD4(+)CD25(+) Tregs (CD4, CD25 and Foxp3 positive) in the peripheral blood mononuclear cells of 53 SSc patients and 27 healthy controls were counted by flow cytometry. The differences between SSc and control patients were analyzed. Clinical parameters, including disease duration, duration of the second symptoms, Modified Rodnan Skin Score (MRSS), anti-topoisomerase I antibody, anti-U1 ribonucleoprotein (RNP) antibody, systemic involvements, pulmonary function test (PFT) and high resolution computed tomography (HRCT) score were prospectively collected following EUSTAR (EULAR scleroderma trial and research group) protocols. The correlations between the experimental and clinical data were investigated.</p><p><b>RESULTS</b>The ratio of Th17 in SSc patients was significantly elevated compared to healthy controls (8.74% vs. 4.41%, P < 0.001). The amount of Th17 was positively correlated with disease duration (R = 0.531, P = 0.013) and duration of the second symptoms (R = 0.505, P = 0.023). The ratio of CD4(+)CD25(+) Treg in SSc patients also significantly differed from the healthy controls (3.04% vs. 2.24%, P = 0.018). Elevated Tregs were more frequently observed in patients with a high interstitial lung disease (ILD) score on computed tomography (24/36) compared with patients with normal ILD scores (4/12, P = 0.043). Elevated Tregs were also more often observed in patients with low carbon monoxide diffusing capacity (DLCO) (24/34) compared with patients with normal DLCO (4/11, P = 0.042).</p><p><b>CONCLUSIONS</b>T cell abnormalities are remarkable in systemic sclerosis. Th17s proliferate and their numbers increase with lengthened disease duration. Th17s might participate in both inflammation and fibrosis by secreting IL-17. CD4(+)CD25(+) Tregs also proliferate in SSc and may play important roles in promoting fibrosis.</p>


Subject(s)
CD4-Positive T-Lymphocytes , Metabolism , Cells, Cultured , Flow Cytometry , Humans , Interleukin-2 Receptor alpha Subunit , Metabolism , Scleroderma, Systemic , Allergy and Immunology , Metabolism , T-Lymphocytes, Regulatory , Allergy and Immunology , Metabolism , Th17 Cells , Metabolism
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