Objective To investigate the clinical and prognosis characteristics of SLE patients whose initial clinical presentations were Sj(o)gren's syndrome (SS).Methods Medical charts of 41 consecutive SS/SLE inpatients admitted to Peking Union Medical College Hospital (PUMCH) from February 1998 to February 2008 were systematically reviewed,including demographic data,clinical features,laboratory findings,treatment as well as prognosis.Two hundred and fourteen cases were randomly selected as controls from 2331 non SS-onset lupus (NSs/SLE) inpatients weated in PUMCH at the same time period.Results There were significant differences between SS/SLE and NSS/SLE patients in the following aspects (P＜0.05):①gender composition:(F/M) (41/0 vs 184/30),age at the diagnosis of SLE [(43±41) yrs vs (32±31) yrs)],disease duration L(114±84) mollths vs (45±18) months];②clinical features:xerostomia (85.3% vs 6.1%),xerophthalmia (75.6% vs 2.3%),faeial rash (9.8% vs 46.3%),renal tubular acidosis (21.9% vs 0),nephrotic syndrome (7.3% vs 31.3%),central nervous system invoivement (4.9% vs 19.6%),interstitial lung disease (12.2% vs 2.8%);③labratory findings:ESR[(65±75) mm/1 h vs (46±34) mm/1 h)],patients with elevated IgG level (56.4% vs 29.9%) and IgA level (38.5% vs 20.4%),RF,prevalence of anti-SSA and anti-SSB antibodies (70.8% vs 20.3%,82.9% vs 43.4% and 39.0% vs 7.9%);④SLEDAI score (8±8 vs 10±10),glucocorticosteroid treatment (methylprednisolone bolus/1～2 mg·kg-1·d-1 prednisone/1 mg·kg-1·d-1 prednisone)(8/26/7 vs 91/102/21),and rate of death and/or severe irreversible organ failure (2.4% vs 14.9%).SS/SLE patients were followed up for (33±34) months,40 cases remained stable and only one patient died of acute pulmonary embolism.Conclusion Compared to NSS/SLE,SS/SLE patients ale older and have more insidious disease course.They have higher prevalence rate of anti-SSA and anti-SSB antibodies,renal tubular acidosis and interstitial lung disease,but less severe neuropsychiatric and renal involvements and much better prognosis.

Objective To compare the characteristics of post-stroke depression in patients with and without aphasia.Methods Seventy patients on the first infarction with aphasia and 70 stroke patients without aphasia were recruited.The aphasia deficits in patients were evaluated by using the Aphasia Battery of Chinese ( Aphasia Battery of Chinese, ABC) .The Stroke Aphasic Depression Questionnaire Hospital Version ( Stroke Aphasic Depression Questionnaire Hospital Version, SADQ-H) was applied to analyze the depression in the two groups.Results The sore in SADQ-H of the aphasia group was significantly higher than the control group( (22.03 ±9.55 )vs ( 16.81 ± 10.47 ), P ＜ 0.01 ) .Loss interest, anhedonia, social avoidance, irritability, depression, decreased attention were more serious in patients with aphasia after stroke than that without aphasia.Compared with control group ,the incidence of depression in aphasia group was higher(64.28% vs 50.00% ).The incidence in different types of aphasia: the complete aphasia ( 78.26% ), transcortical mixed aphasia ( 62.50% ), Broca ( 61.53% ),Werincke (62.50%).There was significantly correlation between the incidence of depression and the severity of aphasia.Conclusion The incidence of depression in stroke patients with aphasia is high,especially who have complete aphasia, transcortical mixed aphasia, Broca and Werincke, and it closely relate to the severity of aphasia.

Objective To preliminarily investigate the cutoff value of Stroke Aphasic Depression Questionnaire Hospital Version (SADQ-H) in the diagnosis of post-stroke depression. Methods Using receiver operating characteristic (ROC) curves, the total scores of 17-item Hamilton Depression Rating Scale (HAMD-17) greater than 7 points and Beck Depression Inventory (BDI-13) greater than 4 points were taken as the cutoff values for the assessment of depression. SADQ-H for the assessment of the depression classification standard in patients with post-stroke aphasia was investigated preliminarily by HAMD-17 (8-17 were mild depression; 18-24 were moderate depression, and＞24 was severe depression). Results When HAMD-17 was used as a standard, ROC area under curve (AUC) was 0. 909 (95% confidence interval [CI] 0.814-1.005). When 19.50 was used as a cutoff value, the sensitivity, specificity, positive predictive value, negative predictive value, and κ coefficient of SADQ-H were 82. 6%,91.7%, 87. 0%, 83.3%, and 0. 77, respectively. When BDI-13 was used as a standard, ROC AUC was 0. 916 (95% CI 0. 824-1. 008). When choosing 18.50 as a cutoff value of diagnosis,the sensitivity, specificity, positive predictive value, negative predictive value, and κ coefficient of SADQ-H were 80. 0%, 90. 0%, 84. 0%, 90. 0%, and 0. 68, respectively. The classification standards of SADQ-H for the assessment of depression in patients with post-stroke aphasia were 19, 22 and 26 points. Conclusions When the cutoff value of SADQ-H was 19 points, it had higher sensitivity, specificity, positive predictive value and negative predictive value for the assessment of depression; 19, 22 and 26 points could be used as the classification diagnostic standard of SADQ-H in the assessment of depression in patients with post-stroke aphasia.

Objective To explore the value of bone marrow megakaryocyte counts in predicting clinical response of thrombocytopenia (TP) in systemic lupus erythematosus (SLE) patients. Methods Thirty-one patients of SLE with severe TP (platelet ≤50×109/L) from Peking Union Medical College Hospital during 2007 to 2014 with appreciable bone marrow aspiration results were retrospectively analyzed. Their therapeutic responses were stratified and the correlation with clinical and laboratory findings including the megakaryocyte counts in bone marrow were evaluated with logistic multivariate regression. Results Totally fifteen patients obtained complete response (CR), eight patients obtained partial response (PR) and eight no response (NR). Megakaryocyte counts in bone marrow were (101±26)/slide, (156±48)/slide and (34±15)/slide respectively with statistically significant difference (χ2=6.632, P=0.036). Those NR patients had less megakaryocytes in their bone marrow compared with those with clinical response (CR+PR) (Z=-2.438, P=0.015). By ROC curve method, we found 20/slide might be a good cutoff of megakaryocyte counts in bone marrow for determining the therapeutic response of immunotherapy with a sensitivity of 91% and a specificity of 63% and a AUC (area under the curve) of 0.793. Those with 20/slide or less megakaryocytes in bone marrows only had a clinical effective response rate of 29% verse a response rate of 88% in those with more megakaryocytes in bone marrow. Conclusion Megakaryocyte counts in bone marrow may provide predictive value for therapeutic response of severe TP in SLE patients. Those patients with equal or less than 20/slide megakaryocytes in their bone marrow tend to have poor therapeutic response.

OBJECTIVE:To investigate the effects of TanshinoneⅡA sodium sulfonate injection on levels of P-selectin,glial fi-brillary acidic protein (GFAP),vascular endothelial growth factor (VEGF) and neurological function in patients with acute cere-bral infarction. METHODS:A total of 114 patients with acute cerebral infarction selected from Lianyungang First People's Hospi-tal during Apr. 2013-Apr. 2016 were divided into control group and observation group according to random number table,with 57 cases in each group. Control group was given routine treatment. Observation group was additionally given Tanshinone ⅡA sodium sulfonate injection 40 mg 0.9％ sodium chlonride injection 250 mL,ivgtt,qd. A treatment course lasted for 7 d,and both received 2 courses of treatment. NIHSS scores,the levels of serum P-selectin,GFAP and VEGF were compared between 2 groups before treatment and after 7,14 d of treatment. The occurrence of ADR was also compared. RESULTS:Before treatment,there was no statistical significance in above indexes between 2 groups(P>0.05). Compared to before treatment,NIHSS score,the levels of se-rum P-selectin and GFAP in 2 groups were decreased significantly after 7,14 d of treatment,while the serum level of VEGF was increased significantly. These indexes of 2 groups after 14 d of treatment were significantly better than 7 d of treatment,except for NIHSS score. Above indexes of observation group was significantly better than those of control group during corresponding period, with statistical significance (P<0.05). No obvious ADR was found in 2 groups. CONCLUSIONS:For acute cerebral infarction, Tanshinone ⅡA sodium sulfonate injection can significantly reduce the levels of serum P-selectin and GFAP,improve VEGF level and promote the recovery of neurological damage with good safety.

Objective To establish a more sensitive method to detect free cancer cells in peritoneal washings of gastric cancer cases during surgery. Methods The CEAmRNA levels in peritoneal washings in 65 cases of gastric cancer were detected by Real-time RT-PCR. PLC was applied simultaneously to detect free cancer cells. Negative controls included the peritoneal washings from 5 cases of benign gastric diseases and the blood samples from 5 cases of healthy adult volunteers. Results (1)CEAmRNA was not found in peritoneal washings in benign gastric diseases and in blood of healthy adult volunteers. (2)The positive percentage of free cancer cells detected by Real-time RT-PCR was 47.7%, while PLC′s was only 12.3%.(3)The positive rate of CEAmRNA showed a significant difference between gastric cancer with serosal invasion and without serosa invasion groups, between peritoneal metastasis group and no peritoneal metastasis groups, and also between stages I+II and III+IV diseases(all P

Objective: To investigate the value of the trigeminocervical response (TCR) for revealing bulbar involvement in patients with spinal and bulbar muscular atrophy (SBMA). Methods: Thirty patients with SBMA and 30 healthy male controls were included in this study. In all of the normal controls, stimulation of the infraorbital nerve on one side produced bilateral short latency waves consisting of a positive/negative wave, p19/n31, the mean latency of which was measured. The mean square root of the ratio between the amplitude of p19/n31 and the mean rectifi ed surface electromyography (EMG) activity preceding the stimulus, the A value, was estimated. The parameters of the TCR were compared between the two groups. Results: Among the patients with SBMA, 21 (70.0%) had delayed latencies of p19/n31 (P < 0.01) and all (100%) had reduced A values (P < 0.01) relative to the normal controls. Conclusions: All parameters of the TCR were signifi cantly different between the patients with SBMA and the normal controls. T

Objective To identify the clinical features of malignancy mimic vasculitis (MMV),and to increase the knowledge and alertness of MMV to avoid misdiagnosis and mistreatment.Methods Clinical records and laboratory index with test results were collected and retrospective study was employed to analyze twenty-four MMV patients,who were admitted to the department of Rheumatology of Peking Union Medical College Hospital during the period from January 2001 to January 2015.All data were analyzed by Fisher exact probability test.Results ① Twenty-four MMV took up to 2.5％ (24/927) of all the systemic vasculitis,and 0.15‰(24/157 883) of all the malignancy diagnosed during the same period.② The average age at the diagnoses being established was (43±19) years and the male to female was 3:1.③ Skin lesions (16/24),mucous injury (12/24),pulmonary involvement (10/24),thrombosis (7/24) and neurological involvement (7/24) were the mose common clinical manifestations that could mimic systemic vasculitis;Beh(c)et's disease (6/24),granulomatous with polyangiitis (6/24) and polyarteritis nodosa (5/24) were types of systemic vasculitis that MMV most frequently mimic.④ Among 22 MMV with definite pathological evidence,there were sixteen hematological malignancies and fourteen non-Hodgkin's lymphoma (NHL).⑤ For hematological malignancies mimic vasculitis patients,skin lesions (P=0.023),elevated LDH (P=0.046),leukocyte disorder (P=0.015) were more common than those nonhematological malignancy patients.⑥ Those with long disease duration (＞12 months) (n=6) tended to present more skin lesions (P=0.024) especially skin ulcers P=0.038) were than short course patients.Conclusion MMV should be proposed for those systemic vasculitis patients with atypical manifestations and poor response to routine therapy.Even if the disease duration is as long as more than 12 months,MMV should not be excluded imprudently as hematological malignancies could have long disease durations.

Objective To evaluate the safety and efficacy of anti -CD20 monoclonal antibody (rituximab) in treating refractory connective tissue disease (CTD) patients. Methods Seven inpatients with refractory CTD from Peking Union Medical College Hospital (PUMCH) including four cases of SLE, one for primary SS,one for undifferentiated CTD and one for antiphospholipid syndrome (APS), who had been admitted in recent 3 years (Jan 2005～Feb 2008) were treated with rituximab in combination with corticosteroid and immunosuppressive agents. Responses to treatment as well as outcomes were investigated. Results Six patients were female and one was male with the age ranged from 21 to 58 yr. Disease duration ranged from 3 months to 20 years. Rituximab resulted in significant improvement in clinical manifestations such as lupus nephritis, autoimmune hemolytic anemia and thrombocytopenia. Four patients developed secondary infection including lung infections,herpes and septicemia during B cell depletion, and three patients died of severe lung infections and respiratory failure. Conclusion Our study has shown promising efficacy of rituximab therapy in refractory CTD patients, though it increases the risk of fatal infections, especially when in combination with immunosuppressive agents.

Objective To describe the immunological characteristics of refractory primary biliary cirrhosis compared with the typical patients for more than 1 year's administration of UDCA.Methods Sixty patients treated with UDCA for more than 1 year in our clinic were enrolled into this study.According to the response to UDCA by Paris criteria,patients were divided into refractory group (23 patients) and typical groups (37 patients).The recent peripheral lymphocyte subsets and cytokines of the two groups were tested and analyzed.One-way ANOVA and t test were used for statistical analysis.Results ① One-year treatment after diagnosis,there were no differences between the two groups in the distribution of peripheral lymphocytic subsets,meanwhile,the two groups had higher percentage of B cells,CD4+T cells,CD4+CD28+T cells and CD8+ CD28-T cells than healthy controls respectively.② The serum levels of IL-6 [(0.8±0.9) pg/ml vs (0.3±0.4) pg/ml] and HGF were higher in the refractory group than other groups.Conclusion During the plateau phase,refractory PBC patients have higher serum levels of IL-6 and HGF,which probably suggest that the refractory PBC patients may have severe immunologic disturbance in vivo.