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ABSTRACT: OBJECTIVES: We aimed to evaluate the association between polymorphisms of TNFSF4 and primary Sjögren's syndrome (pSS) and primary biliary cirrhosis (PBC) in a Chinese Han population. METHODS: A total of 250 pSS patients, 221 PBC patients, and 393 healthy controls were enrolled. All individuals were ethnic Chinese Han, and each group was matched for gender ratio and age. We identified single nucleotide polymorphisms (SNPs) via the HapMap Han Chinese Beijing databank for a genetic region containing TNFSF4, and then identified haplotype tagging SNPs with the Tagger programme of Haploview. DNA samples were amplified through polymerase chain reaction (PCR) and extension products were differentiated via mass spectrometry. Association analyses were performed using PLINK software. RESULTS: In TNFSF4, T allele and TT genotype of rs2205960, and G allele of rs1234313, were associated with pSS (p<0.05); T allele of rs2205960 was correlated with PBC (p<0.05) as a risk factor. In the haplotype analysis, TAGG and TGGT were correlated with pSS (p<0.05). In genetic additive, dominant, and recessive models analysis, rs2205960 had a significant association with both pSS and PBC, and rs1234313 presented a significant association with pSS (p<0.05). However, no statistically significant difference was found after Bonferroni corrections. CONCLUSIONS: Overall, no association between the allele, or genotype, or haplotype frequencies of TNFSF4 and the risk of pSS or PBC was found. TNFSF4 may have little significance as a common genetic component of pSS and PBC in the Chinese Han population.
Clinical and experimental rheumatology 04/2013; · 2.15 Impact Factor
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ABSTRACT: To examine the clinical features and analyze prognostic factors in a prospective study of primary biliary cirrhosis (PBC) patients.
From 1995 to 2010, PBC patients without hepatic decompensation seen at the Peking Union Medical College Hospital were enrolled. Clinical signs and manifestations (pruritus, persistent fatigue, jaundice and pain in the right hypochondrium), laboratory parameters (auto-antibodies for autoimmune hepatic disease, biliary and hepatic enzymes, immunoglobulin, bilirubin, and albumin) and imaging findings were recorded at entry and at specific time points during follow-up. Cox regression and Kaplan-Meier analyses, respectively, assessed the risk factors for hepatic decompensation and survival.
Two hundred and sixty-two PBC patients were enrolled with a median follow-up of 75.2 mo (range, 21-201 mo). The 240 patients were aged 51.5 ± 10.2 years at diagnosis and 91.6% were female. Two hundred and forty-five (93.5%) were seropositive for anti-mitochondrial antibodies. At presentation, 170 patients (64.9%) were symptomatic, while 96 patients (36.6%) had extra-hepatic autoimmune disease. During the follow-up period, 62 (23.7%) patients developed hepatic decompensation of whom four underwent liver transplantation and 17 died. The cumulative survival rate and median survival time were 83.9% and 181.7 mo, respectively. Cox regression analysis revealed that an incomplete ursodeoxycholic acid (UDCA) response or inconsistent treatment [ < 0.001; hazard risk (HR) 95%CI = 2.423-7.541], anti-centromere antibodies (ACA) positivity ( < 0.001; HR 95%CI = 2.516-7.137), alanine aminotransferase ratio (AAR) elevations ( < 0.001; HR 95%CI = 1.357-2.678), and histological advanced liver disease ( = 0.006; HR 95%CI = 1.481-10.847) were predictors of hepatic decompensation. The clinical features and survival of PBC in China are consistent with those described in Western countries.
Incomplete UDCA response or inconsistent treatment, ACA positivity, AAR elevations, and advanced histological stage are predictors of decompensation.
World Journal of Gastroenterology 02/2013; 19(7):1111-8. · 2.47 Impact Factor
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Li-Na Zhang,
Tian-Yan Shi,
Xu-Hua Shi,
Li Wang,
Yun-Jiao Yang,
Bin Liu,
Li-Xia Gao,
Zong-Wen Shuai,
Fang Kong,
Hua Chen, [......],
Yun-Yun Fei,
Quan-Cai Cui,
Qian Wang,
Min Shen,
Dong Xu,
Wen-Jie Zheng,
Yong-Zhe Li,
Wen Zhang,
Xuan Zhang, Feng-Chun Zhang
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ABSTRACT: The biochemical response to ursodeoxycholic acid (UDCA) in primary biliary cirrhosis (PBC) is a strong predictor of long-term outcome and thus facilitates the rapid identification of patients needing new therapeutic approaches. Numerous criteria for predicting outcome of treatment have been studied based on biochemical response to UDCA at 1 year. We sought to determine whether an earlier biochemical response at 3 or 6 months could as efficiently identify patients at risk of poor outcome, as defined by liver-related death, liver transplantation, and complications of cirrhosis. We analyzed the prospectively collected data of 187 patients with a median follow-up of 5.8 years (range: 1.3-14 years). The survival rates without adverse outcome at 5 years and 10 years were 86% and 63%. Under UDCA therapy, laboratory liver parameters experienced a most prominent improvement in the first 3 months (P < 0.0001) and then stayed relatively stable for the following months. The Paris, Barcelona, Toronto, and Ehime, but not the Rotterdam definition applied at 3, 6, and 12 months significantly discriminated the patients in terms of long-term outcome. Compared to biochemical responses evaluated after 1 year of UDCA therapy, biochemical responses at the third month demonstrated higher positive predictive value (PPV) but lower negative predictive value (NPV) and increased negative likelihood ratio (NLR) by all definitions; biochemical responses at the sixth month showed higher or the same PPV and NPV and lower NLR by all definitions.Conclusion: For the previously published criteria, biochemical responses at the sixth month can be used in place of those evaluated after 1 year of UDCA therapy. Our findings justify a more rapid identification of patients who need new therapeutic approaches. (HEPATOLOGY 2013.).
Hepatology 02/2013; · 11.66 Impact Factor
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ABSTRACT: BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) has been increasingly identified in patients with systemic lupus erythematosus (SLE) owing to the advance in neuroimaging techniques. Prompt diagnosis is pivotal to improve its outcome. To analyze the clinical and radiographic profile of PRES in patients with SLE and search for the appropriate treatment strategy PRES in SLE. METHODS: SLE patients who fulfilled the diagnostic criteria for PRES from August 2008 to January 2011 were evaluated at baseline, and followed to determine clinical outcomes. Data were analysis on clinical characteristics, laboratory abnormalities, treatment details, and outcomes. RESULTS: Ten episodes of PRES in patients with SLE were identified. All patients were female, mean age of onset was 22.93 +/- 2.48 years, and SLEDAI at the onset of PRES were 25.8 +/- 5.7. All cases had acute onset of headache, altered mental status, stupor, vomiting, cortical blindness and seizures. Neurological symptoms were the initial manifestation of SLE in three cases. Head magnetic resonance imaging (MRI) demonstrated posterior white matter edema involving the parietal, temporal and occipital lobes, which were more conspicuous on T2 weighted spin echo and diffusion-weighted MR imaging (DWI) than on computed tomography (CT) scan. Complete clinical and radiographic recovery was observed in 8 patients after prompt treatment with corticosteroids. CONCLUSIONS: PRES might be due to lupus per se besides other traditional causative factors such as hypertension. PRES might be an underestimated variant of "reversible neurological deficits" in SLE. Prompt recognition and timely management is important to prevent permanent neurological deficits.
BMC Neurology 12/2012; 12(1):152. · 2.17 Impact Factor
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ABSTRACT: To investigate the role of different therapeutic regimens in primary biliary cirrhosis (PBC)complicating Sjögren syndrome (SS).
A total of 79 patients diagnosed as PBC complicating SS were enrolled and randomly divided into three groups: Group U (29 patients) received ursodeoxycholic acid (UDCA) alone, Group UP (37 patients) received UDCA and prednisolone, Group UA (13 patients) received UDCA and azathioprine. The clinical and laboratory data were collected at 0, 3, 6 and 12 months after treatment.
Fatigue and pruritus were improved in each group with no difference among them (P > 0.05). The levels of ALT, AST, alkaline phophatase (ALP), gamma-glutamyl transferase (GGT), TBil, DBil, IgG, and IgM in the three groups were all decreased after treatment (P < 0.05), while there were no statistical differences among the three groups (P > 0.05).
The combination therapy of UDCA with prednisolone or azathioprine was not better than UDCA alone. The therapeutic policy of PBC complicating SS involved in the liver should settle PBC mainly.
Zhonghua nei ke za zhi [Chinese journal of internal medicine] 11/2012; 51(11):851-4.
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ABSTRACT: OBJECTIVE: To summarize the clinical characters of refractory primary biliary cirrhosis (PBC) versus typical ones and explore the risk factors of prognosis. METHODS: From 2009 to 2011, 60 PBC patients on the therapy of ursodeoxycholic acid (UDCA) over 1 year at our clinic were recruited. According to the response to UDCA by the Paris criteria, they were divided into refractory group (n = 23) and typical group (n = 37) to analyze the basic clinical conditions, biochemical markers, antibodies and liver biopsies. RESULTS: (1) Compared with the typical patients, the refractory ones had more fatigue (73.9% vs 45.9%), pruritus (60.9% vs 40.5%) and jaundice (43.4% vs 18.9%); (2) the serum level of biomarkers (alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), γ-glutamyl transpeptidase (GGT), total bilirubin (TBIL), direct bilirubin (DBIL) and immunoglobulin M (IgM)) were higher in the refractory group (P < 0.05); (3) no differences existed between two groups in age, gender, antibodies (antinuclear antibody (ANA), antimitochondrial antibody (AMA), AMA-M2, anti-GP210, anti-SP100 and anti-centromere antibody (ACA)) or liver biopsies. CONCLUSION: Some clinical severe manifestation (fatigue, pruritus and jaundice) and higher biochemical markers levels (ALT, AST, ALP, GGT, TBIL, DBIL and IgM) seem to predict a worse response to UDCA and a rapid progression of disease in PBC patients.
Zhonghua yi xue za zhi 11/2012; 92(41):2918-2920.
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ABSTRACT: OBJECTIVES: To assess the clinical characteristics and outcome of patients with cardiac Behçet's disease(BD). METHODS: Medical charts of 20 cardiac BD patients admitted in Peking Union Medical College Hospital from June 1996 to June 2011 were systematically reviewed, including demographic data, clinical features, laboratory and histopathology findings and outcome. RESULTS: Patients age ranged 19~57 yrs[mean (35±10) yrs], included 17 males and 3 females. Six (30%) of them did not fulfill the ISG criteria at cardiac onset, and fourteen (70%) of them experienced heart failure. Echocardiography findings included intracardiac thrombus (n=7), and aortic valve involvement with left ventricular enlargement and severe aortic regurgitation (n=13). Eight patients underwent surgery before efficient immunosuppressant treatment, and five (62.5%) underwent re-operation due to recurrence of thrombus or valvular dehiscence and severe paravalvular leakage. Histopathology findings revealed predominantly inflammatory cells infiltration, thrombus and fibrous tissue formation. After initiation of prednisone plus immunosuppressant, patients were followed up for 6~42 months (mean 14.8±9.9 months), the intracardiac thrombus disappeared or decreased in size in five cases, remained stable after surgery in the other two cases, and the heart failure disappeared in all patients with aortic involvement. CONCLUSIONS: Cardiac BD affects males more than females, and is prone to delayed diagnosis because some patients do not have typical clinical manifestations at cardiac onset; Corticosteroids plus immunosuppressants reduce the thrombus and improve aortic regurgitation and heart failure in cardiac BD, whereas surgery alone does not lead to complete resolution.
Clinical and experimental rheumatology 09/2012; · 2.15 Impact Factor
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Chao-Jun Hu,
Guang Song,
Wei Huang,
Guo-Zhen Liu,
Chui-Wen Deng,
Hai-Pan Zeng,
Li Wang, Feng-Chun Zhang,
Xuan Zhang,
Jun Seop Jeong,
Seth Blackshaw,
Li-Zhi Jiang,
Heng Zhu,
Lin Wu,
Yong-Zhe Li
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ABSTRACT: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease of unknown etiology and is considered to be an autoimmune disease. Autoantibodies are important tools for accurate diagnosis of PBC. Here, we employed serum profiling analysis using a human proteome microarray composed of about 17,000 full-length unique proteins and identified 23 proteins that correlated with PBC. To validate these results, we fabricated a PBC-focused microarray with 21 of these newly identified candidates and nine additional known PBC antigens. By screening the PBC microarrays with additional cohorts of 191 PBC patients and 321 controls (43 autoimmune hepatitis, 55 hepatitis B virus, 31 hepatitis C virus, 48 rheumatoid arthritis, 45 systematic lupus erythematosus, 49 systemic sclerosis, and 50 healthy), six proteins were confirmed as novel PBC autoantigens with high sensitivities and specificities, including hexokinase-1 (isoforms I and II), Kelch-like protein 7, Kelch-like protein 12, zinc finger and BTB domain-containing protein 2, and eukaryotic translation initiation factor 2C, subunit 1. To facilitate clinical diagnosis, we developed ELISA for Kelch-like protein 12 and zinc finger and BTB domain-containing protein 2 and tested large cohorts (297 PBC and 637 control sera) to confirm the sensitivities and specificities observed in the microarray-based assays. In conclusion, our research showed that a strategy using high content protein microarray combined with a smaller but more focused protein microarray can effectively identify and validate novel PBC-specific autoantigens and has the capacity to be translated to clinical diagnosis by means of an ELISA-based method.
Molecular & Cellular Proteomics 05/2012; 11(9):669-80. · 7.40 Impact Factor
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Yang Li,
Li-Dan Zhao,
Lu-Sha Tong,
Su-Ning Qian,
Yan Ren,
Lei Zhang,
Xin Ding,
Yang Chen,
Yan-Xia Wang,
Wen Zhang,
Xiao-Feng Zeng, Feng-Chun Zhang,
Fu-Lin Tang,
Xuan Zhang,
De-Nian Ba,
Wei He,
Xue-Tao Cao,
Peter E Lipsky
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ABSTRACT: CD200 is a type I transmembrane glycoprotein that can regulate the activation threshold of inflammatory immune responses, polarize cytokine production, and maintain immune homeostasis. We therefore evaluated the functional status of CD200/CD200 receptor 1 (CD200R1) interactions in subjects with systemic lupus erythematosus (SLE).
Serum CD200 level was detected by ELISA. The expression of CD200/CD200R1 by CD4+ T cells and dendritic cells (DCs) was examined by flow cytometry, and then compared between SLE patients and healthy controls. Peripheral blood mononuclear cells were stained with carboxyfluorescein diacetate succinimidyl ester and annexin V/propidium iodide for evaluation of the effect of CD200 on cell proliferation and apoptosis. In addition, the effect of CD200 on DC function was determined by transwell migration assay as well as by measurement of binding and phagocytosis of apoptotic cells.
In SLE patients, the number of CD200+ cells and the level of soluble CD200 were significantly higher than in healthy controls, whereas the expression of CD200R1 by CD4+ T cells and DCs was decreased. Furthermore, the increased CD200 expression by early apoptotic cells contributed to their diminished binding and phagocytosis by DCs in SLE. Importantly, the engagement of CD200 receptor on CD4+ T cells with CD200-Fc fusion protein in vitro reduced the differentiation of T-helper type 17 cells and reversed the defective induction of CD4+CD25highFoxP3+ T cells by transforming growth factor beta in SLE patients. Conversely, blockade of CD200-CD200R1 interaction with anti-CD200R1 antibody promoted CD4+ T-cell proliferation.
CD200 and CD200R1 expression and function are abnormal in SLE and may contribute to the immunologic abnormalities in SLE.
Arthritis research & therapy 05/2012; 14(3):R123. · 4.27 Impact Factor
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ABSTRACT: Previous studies indicate that CD43 plays a role in regulating the adhesion of lymphocytes, cell mutation and activation, however, little is known about its effect on systemic lupus erythematosus (SLE). This study was designed to explore the clinical significance of CD43 in SLE patients.
We used microarray and real-time PCR to detect the mRNA and protein expression of magnetic bead sorted T cells and B cells from peripheral blood mononuclear cells (PBMCs) of SLE patients, and analyzed the relationship between CD43 and the clinical indexes.
Both microarray and real-time PCR results showed that CD43 mRNA was significantly decreased in PBMCs of SLE patients compared with healthy controls (P < 0.001). There were no significant differences between lupus nephritis and non-lupus nephritis patients, and neuropsychiatric and non-neuropsychiatric patients. CD43 mRNA expression was significantly reduced in T cells but not in B-cells in SLE patients compared to healthy controls (P < 0.01). Compared with healthy controls, the percentage of CD43(+) cells in the PBMCs of SLE was significantly decreased (P = 0.004), and the CD43 fluorescence intensity in CD3(+)/CD43(+) cells and CD19(+)/CD43(+) cells was also significantly weaker than in healthy controls (P = 0.039 and 0.003). There was no significant difference in the percentage of CD3(+)/CD43(+) cells, CD19(+)/CD43(+) cells between the two groups. The CD43 fluorescence intensity in CD3(+)/CD43(+) cells was inversely correlated with the levels of IgG and IgM (r = -0.8 and -0.6).
Compared to healthy controls, both CD43 mRNA and protein expressions were reduced in T cells from patients with SLE, and were inversely correlated with IgG.
Chinese medical journal 04/2012; 125(7):1266-71. · 0.86 Impact Factor
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ABSTRACT: Adamantiades-Behcet's disease (ABD) is a chronic, relapsing, systemic vasculitis of unknown etiology. It is more prevalent in populations along the ancient Silk Road from Eastern Asia to the Mediterranean Basin, and most frequently affects young adults between the second and fourth decades of life. ABD-complicated gastroenteropathy is a significant cause of morbidity and mortality, with abdominal pain as the most common symptom. The ileocecal region is affected predominantly, with ulcerations that may lead to penetration and/or perforation, whereas other parts of the gastrointestinal system including the esophagus and stomach can also be affected. Endoscopy is useful to locate the site and extent of the lesions, and tissue biopsy is often warranted to examine the histopathology that is often suggestive of underlying vasculitis of small veins/venules or, alternatively in some cases, nonspecific inflammation. Bowel wall thickening is the most common finding on computed tomography scan. Treatment is largely empirical since well-controlled studies are difficult to conduct due to the heterogeneity of the disease, and the unpredictable course with exacerbation and remission. Corticosteroids with or without other immunosuppressive drugs, such as cyclophosphamide, azathioprine, sulfasalazine, tumor necrosis factor α antagonist or thalidomide should be applied before surgery, except in emergency.
World Journal of Gastroenterology 02/2012; 18(7):609-15. · 2.47 Impact Factor
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ABSTRACT: To explore the clinical manifestations of muscular tuberculosis (MT) and analyze its risk factors.
Twenty MT patients were recruited from our department during 2000 - 2010. There were 9 males and 11 females with an average age of 43.5 years old. And their clinical manifestations were recorded and analyzed.
All patients had local masses. And 19 patients had the involvement of single muscle and multiple muscles were involved in 1 patient. Gastrocnemius was affected in 9 patients. Nine patients had a previous history of tuberculosis or suffered concurrent tuberculosis of other body parts. Three patients with immune system disease received glucocorticoid therapy. And 11 patients underwent PPD (purified protein derivative) test and only 1 was strongly positive while 10 others were negative. MT was confirmed by pathological examinations in 20 cases. All patients underwent muscle biopsy and received effective chemotherapy.
As a kind of systemic disease, MT is mainly characterized by painful or painless muscle mass. The patients with a history of tuberculosis, tuberculosis of other body parts and immune system disease are susceptible to MT. Diagnosis is mainly made through biopsy. And chemotherapy is effective.
Zhonghua yi xue za zhi 01/2012; 92(3):206-8.
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Xue-Feng Sun,
Qing-Jun Wu,
Ya-Lan Bi,
Yong Hou,
Meng-Tao Li,
Wen Zhang,
Xuan Zhang,
Yan Zhao,
Xiao-Feng Zeng, Feng-Chun Zhang,
Fu-Lin Tang
The Journal of Rheumatology 05/2011; 38(5):959-60. · 3.69 Impact Factor
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ABSTRACT: To investigate clinical features of cutaneous sarcoidosis.
A retrospective analysis was carried out based on the clinic data of 35 patients with cutaneous sarcoidosis who were hospitalized in Peking Union Medical College Hospital during 1980 - 2009. They were divided into two groups, the group without systemic involvement (skin group) and the group with systemic involvement (systemic group).
(1) The ratio of men and women with cutaneous sarcoidosis was 1:3.38, and the average incident age was (47.5 ± 10.0) years old. The average incident age of skin group and systemic group were (41.8 ± 12.5) years old and (50.5 ± 7.1) years old, respectively. (2) The most common skin manifestation was subcutaneous nodule, followed by maculopapular and erythema nodosa. The most common involved sites were limbs. (3) The common involved systems extra-skin included the lung, joints and lymph nodes. The involvement rate of lung in cutaneous sarcoidosis of our present data was lower than those of foreign reports. However, the involvement rates of joints, lymph nodes, kidney, muscles and nervous system showed higher in our data. (4) The incidences of fatigue and weight loss in systemic group were higher than those in skin group (P < 0.05). The indexes of erythrocyte sedimentation rate, C-reactive protein and rheumatoid factor in systemic group were higher than those in skin group (P < 0.05). More patients in systemic group were treated with corticosteroid than that in skin group (95.7% vs 66.7%, P < 0.05).
Subcutaneous nodules are the most common and the involvement rate of lung is lower in cutaneous sarcoidosis of our present data. Compared to the patients without systemic involvement, the average incident age of systemic ones is older, the indexes of inflammation markers and the usage of corticosteroid are higher.
Zhonghua nei ke za zhi [Chinese journal of internal medicine] 05/2011; 50(5):397-400.
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ABSTRACT: To analyze the characteristics of vascular involvement in Takayasu's arteritis (TA) patients and explore the differences between the involvements of whole range versus origin of vessels and those whose origin of vessels were involved.
A total of 57 TA patients were divided into the origin and whole range groups according to their CTA (computed tomography angiography) examination reports. Then a retrospective analysis was performed on their clinical data.
(1) TA had a female dominance of 1:10.4. The TA-extensive type was the most common type. And carotid and subclavian arteries were the most common sites of involvement. (2) The ratio of whole range and origin groups was 1.28:1. The delay of whole range group from the onset to a correct diagnosis was longer than that of origin group (P < 0.05). The rate of aortic arch type in whole range group was less than that in origin group (P < 0.05). (3) More patients in whole range group had the initial symptom of headache than those in origin group, as well as the subsequent symptoms of claudication, weight loss and urination problems (P < 0.05). (4) 37.5% of patients in whole range group and 64% in origin group had an elevated level of C-reactive protein (CRP) (P < 0.05).
Patients with whole range of arteries involved in TA are common. With atypical clinical manifestations and a low positive rate of CRP, a diagnostic delay is an important issue. Thus due attention should be paid to its clinical diagnosis and treatment.
Zhonghua yi xue za zhi 04/2011; 91(15):1055-7.
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ABSTRACT: The objective of the study was to assess the safety and effectiveness of the chloroform/methanol extract of Tripterygium wilfordii Hook F (T2) plus methotrexate (MTX) in treating patients with rheumatoid arthritis (RA).
One hundred sixty-six patients with RA, who started the combination therapy of T2 (20 mg b.i.d. or t.i.d.) and MTX (10-12.5 mg/wk), were enrolled, and these patients were followed up for at least 1 year. Demographics, disease severity, markers of disease activity before and after the combination therapy, and incidence of adverse events were evaluated.
The patients were predominantly female (n = 134, 81%) with a mean age of 58.0 (SD, 7.9) years (range, 39-79 years) and a mean disease duration of 55.0 (SD, 72.2) months (range, 0-456 months). A total of 161, 161, 146, and 85 patients had received at least 1, 3, 12, and 24 months of the combination of T2 and MTX, with a total of 4162 patient-months' exposure to the combination therapy. The combination therapy reduced tender and swollen joint counts, morning stiffness, inflammatory indices such as ESR and CRP, and improved disease activity as measured by the DAS28 significantly by 3 months as well as 12 months (P < 0.05). Most of the adverse events noted during this study were mild. Menstrual irregularity occurred in 72.7% (16/22) of premenopausal female. Only 10 (6.0%) and 8 (4.8%) subjects withdrew because of adverse events or lack of efficacy, respectively. Severe infections were very rare.
T2 plus MTX is an effective and relatively safe treatment for RA patients.
Journal of clinical rheumatology: practical reports on rheumatic & musculoskeletal diseases 11/2010; 16(8):375-8. · 1.19 Impact Factor
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ABSTRACT: Primary Sjögren's syndrome (pSS) is one of the autoimmune diseases with high incidence. There were several clinical investigations in Caucasian but seldom in Chinese. The aim of this study was to compare the difference of clinical manifestations, immunological features and prognosis of pSS between Caucasian and Chinese pSS patients.
Five hundred and seventy-three patients who fulfilled the 2002 international classification (criteria) for pSS from Peking Union Medical College Hospital between 1985 and 2006 were screened retrospectively and compared with other populations.
(1) The study consisted of 524 (91%) female and 49 (9%) male patients (female: male = 10.7:1). Mean age at the onset of the disease was (39.0 ± 13.7) years and in 169 (29.5%) patients the disease onset occurred before the age of 30 years. The average duration from disease onset to pSS diagnosis was 48 months (range, 1 - 552 months). It had been shortened during the recent five years. (2) Dry mouth (84.5%) and dry eyes (70.0%) were the most common symptoms, significantly lower than foreign patients (P = 0.000). Two hundred and seventy-two (47.5%) patients presented with rampant caries, 160 (27.9%) with parotidomegaly. The positivity of xerostomia, xerophthalmia and salivary gland biopsy were 91.9%, 94.8% and 90.7%, respectively. (3) Systemic involvement occurred in 91.4% patients. Compared with studies done outside China, higher prevalence of fever 41.0%, myositis 4.9%, pericardial effusion 14.8%, pulmonary involvement 42.3%, renal involvement 33.5%, thyroid involvement 32.7%, pancrease involvement 5.6% (P < 0.01) and lower prevalence of fatigue, lymphadenectasis and Raynaud's phenomenon (P < 0.01) were seen. (4) Risk factors of death include pulmonary artery hypertension, liver damage and interstitial lung disease.
Chinese pSS differs significantly from the non-Chinese cases in terms of the age of onset, systemic involvement, autoantibodies and proportional mortality rate. Lung and liver damage were found to be the highest risk factors of the disease prognosis.
Chinese medical journal 11/2010; 123(22):3252-7. · 0.86 Impact Factor
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Yin Su,
Yi Zhao,
Xu Liu,
Jian-Ping Guo,
Quan Jiang,
Xiang-Yuan Liu, Feng-Chun Zhang,
Yi Zheng,
Xiao-Xia Li,
Hui Song,
Ci-Bo Huang,
Yan-Hong Huang,
Tian Wang,
Si-Si Pan,
Chun Li,
Xia Liu,
Lei Zhu,
Chun-Fang Zhang,
Zhan-Guo Li
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ABSTRACT: Recent studies have identified signal transducer and activator of transcription 4 (STAT4) as a susceptibility gene for systemic lupus erythematosus (SLE) in different populations. In order to examine whether the allele distribution of the single nucleotide polymorphism (SNP) in gene STAT4 rs7574865 in patients with SLE is different from those of healthy controls in Chinese Northern Han population, we investigated whether the variants of STAT4 rs7574865 were associated with any specific clinical features of SLE.
We genotyped SNPs in STAT4 rs7574865 in 252 patients with SLE and 497 healthy controls. All subjects were from the Northern part of Chinese Han population. The genotypes in rs7574865 were determined by polymerase chain reaction (PCR) and consequence direct sequencing of PCR products in the DNA samples.
There was a significant difference in distribution of the SNPs in rs7574865 between the SLE patients and healthy controls. Compared with healthy controls, there was a significant correlation between TT genotypes in rs7574865 and the risk of SLE when GG genotype was used as a reference genotype after adjusting for gender and age. The frequency of T allele in the SLE patients was strongly significantly higher than that of healthy controls. Furthermore, there was a significant difference in the distribution of SNP in rs7574865 between male and female SLE patients, when compared with healthy controls. The frequency of T allele in rs7574865 in male patients was significantly higher than that of male healthy controls or female patients. There was no significant correlation between the frequencies of T allele in STAT4 rs7574865 and the clinical features of SLE.
The SNP rs7574865 in STAT4 is strongly associated with risk of SLE in the Chinese Northern Han population. The TT genotype and T allele in STAT4 rs7574869 are susceptibility factors for SLE, especially for male SLE patients.
Chinese medical journal 11/2010; 123(22):3173-7. · 0.86 Impact Factor
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ABSTRACT: To address the clinical features of malignant lymphoma (ML) occurring in primary Sjögren's syndrome (pSS), understand their similarities and differences and explore their risk factors.
Medical records of 17 cases of pSS/ML inpatients admitted to our hospital from January 1980 to August 2010 were systemically reviewed. And 163 cases were randomly selected as controls from 4485 pSS/nML inpatients at our hospital during the same period.
There were 14 females and 3 m ales with the diagnostic age of pSS at (53 ± 13) years old. And the disease duration was (8 ± 9) years. There was no significant difference between two groups in three general aspects (P > 0.05). The gap between pSS and ML was (4 ± 4) years. Several significant differences existed between two groups (P < 0.05) in clinical features and laboratory findings: lymphadenopathy, parothydomegalia and leucopenia. The pathologic categories of ML in pSS/ML included NHL (n = 15) (14 cases of B-cell origin), HL (n = 1) and lymphosarcoma (n = 1).
During the course of pSS, the occurrence of such manifestations as lymphadenopathy, parothydomegalia and leucopenia, etc.tends to have a complication of ML with a dominance of NHL. And a worse prognosis is expected.
Zhonghua yi xue za zhi 10/2010; 90(39):2773-5.
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ABSTRACT: Primary biliary cirrhosis (PBC) is a chronic, progressive, cholestatic, organ-specific autoimmune disease of unknown etiology. It predominantly affects middle-aged women, and is characterized by autoimmune-mediated destruction of small- and medium-size intrahepatic bile ducts, portal inflammation and progressive scarring, which without proper treatment can ultimately lead to fibrosis and hepatic failure. Serum autoantibodies are crucial tools for differential diagnosis of PBC. While it is currently accepted that antimitochondrial antibodies are the most important serological markers of PBC, during the last five decades more than sixty autoantibodies have been explored in these patients, some of which had previously been thought to be specific for other autoimmune diseases.
World Journal of Gastroenterology 08/2010; 16(29):3616-29. · 2.47 Impact Factor
