Objective To investigate the significance of anticentromere antibody(ACA)in patients with primary biliary cirrhosis(PBC).Method ACA was detected using indirect immunofluorescence(IIF)in 99 patients with PBC,and the difference was compared between patients with and without ACA.Results Fifty-three patients(53.5%)had ACA in serum.The average age of onset was elder in patients with ACA than in patients without ACA[(52.6?1.5)vs(46.2?2.0),P=0.012].There was no significant difference in sex ratio between the two groups.Incidence of gastrointestinal bleeding was higher in patients with than without ACA (13.2% vs 0,P=0.014).Difference was not significant in symptoms such as fatigue,pruritus,icterus,etc. Though it didn't reach statistically significant,the incidence of esophageal varices was higher in patients with ACA than without ACA(45.8% vs 10.0% respectively,P=0.061).There was no significant difference in diam- eter of portal vein,splenomegaly,ascites.Biochemically only serum total protein reached statistical significance between the two groups[(73.3+1.1)g/L vs(78.1+l.7)g/L respectively,P=0.017].Patients with ACA had low- er serum IgG than ACA negative patients[(15.1?0.6)g/L vs(18.4?1.0)g/L respectively,P=0.006].Nuclear envelope pattern(NE),one of the patterns of ANA,was rare in ACA positive group than ACA negative group (16.7% vs 50.0% respectively,P=0.002).Conclusion The prevalence of ACA is high in patients with PBC. Patients with ACA have high risk of oesophageal varices and gastrointestinal bleeding.Nuclear envelope pat- tern of ANA is rare in patients with ACA.

Objective To evaluate the parametric features of image textures on 18F-FDG PET/CT for the differentiation between malignant and benign pulmonary nodules and compare the diagnostic performance of these parameters with SUVmax.Methods 18F-FDG PET/CT images of 170 patients (102 males,68 females,age range:29-81 (mean 59)years) with pulmonary nodules were retrospectively evaluated.Eightynine pulmonary nodules (230 slices) were malignant and 81 (193 slices) were benign.The pulmonary nodules were contoured on CT images and mapped to the co-registered PET images.Thirteen parameters of textural features were extracted and SUVmax was measured.Logistic regression analysis was used to identify the significant texture parameters and create a regression model.The efficacy of the textural features and SUVmax to distinguish between malignant and benign pulmonary nodules was evaluated by ROC curve analysis.The textural features of squamous cell carcinoma and adenocarcinoma were compared via the Mann-Whitney u test.The sensitivity and specificity of the textural features and SUVmax for the differential diagnosis were compared with x2 test.Results Logistic regression model identified 4 textural features (skewness (β =1.7058),kurtosis (β =-1.0989),angular second moment (ASM,3 =-4.4140) and strength (β =0.5626) ; all P ＜ 0.05) to have significant correlation with the malignancy of lung nodules.The AUC of ROC curve was 0.775 (95％ CI0.732-0.819; P＜0.001) with the sensitivity of 89.6％ (206/230) and specificity of 50.8％ (98/193).ASM and strength had statistically significant differences between squamous cell carcinoma and adenocarcinoma [ASM:0.0303 (95％ CI 0.0392-0.0724) vs 0.0594 (95％ CI 0.0721-0.0947) ; strength:2.4714 (95％ CI 2.4632-4.1050) vs 1.5945 (95％ CI 1.9003-2.4652) ; u =3082.0 and 3115.0,both P＜0.01].The AUC of SUVmax-based diagnosis was 0.757 (95％ CI 0.711-0.802 ; P ＜ 0.001) with the sensitivity of 80.9 ％ (186/230) and specificity of 50.3 ％ (97/193) at a cut-off value of 2.5.The sensitivity of the textural features was superior to SUVmax in differentiating malignant from benign pulmonary nodules (x2 =6.903,P ＜ 0.01).Conclusions Image textural parametric features extracted from 18 F-FDG PET/CT are more sensitive to differentiate between malignant and benign pulmonary nodules compared to SUVmax.They might also be useful to discriminate between different pathological types of lung cancers.

Objective To investigate the effect of combination therapy by observing the salivary glands function and related organ pathology after given methotrexate (MTX) and cyclophosphamide (CTX) intermittently in induced mice model of Sjogren's syndrome (SS). To further explore the synergistic effect of combination therapy by detecting the immunological regulatory factor B cell activation factor belonging to the TNF family (BAFF) expression. Methods The ingredients of Lewis rat's exocrine glands homogenate were injected into female C57BL/6 mice to set up the mice model of SS. After established the SS mice model successfully, they were randomly divided into six SS model group, including low-dose MTX treatment group (0.02 mg/w), high-dose MTX treatment group (0.06 mg/w), CTX pause treatment group (1.2 mg/3 w), CTX alternate day treatment group (0.6 mg/2 d), MTX+CTX combination treatment group (MTX 0.02 mg/3 w+ CTX 1.2 mg/3 w). Treatment effects were assessed both clinically and histologically. Results Eighteen weeks after the first treatment, the improvement of the salivary secretion of the CTX alternate day treatment group and MTX+CTX combination treatment group were higher than other groups, which showed statistically significant difference (P<0.01). Compared with the SS model control group, HE staining showed that the lymphocytic infiltration of exoerine glands was decreased in the treatment group. In the CTX alternate day treatment group and MTX+CTX combination treatment group, few amount of inflammatory cell infiltration were found, and the expression intensity of BAFF mRNA and protein were decreased markedly in salivary gland than others by RT-PCR and immunohistochemistry assay (P<0.01). Conclusion MTX and CTX can inhibit lymphocytic infiltration of the salivary glands, inhibit BAFF transcriptional level and production of BAFF protein, leading to an increase of fluid production. It suggests that modulation of signaling via BAFF pathways may be a mechanism of action. MTX and CTX combination therapy is more effective than single-agent therapy. The inhibitory effects of MTX and CTX on BAFF-mediated inflammatory pathways are primarily synergistic.

Objective To summarize the clinical characteristics of SLE patients complicated with Listeria monocytogenes meningitis (LMM).Methods The clinical manifestations,systemic involvements,laboratory tests,cerebrospinal fluid (CSF) examinations,treatment and prognosis of SLE inpatients combined with LMM in Peking Union Medical College Hospital during Janurary 2000 to December 2015 were retrospectively analyzed.Results Eight SLE patients with LMM accounted for 0.14％ of the 5 675 hospitalized SLE patients.Seven were female and 1 was male,with the mean age of (35±16) year.Headache (7/8) was the most common neurological symptom followed by positive meningeal irritation sign (5/8),unconsciousness (3/8) and nausea or vomiting (2/8).Increased intracranial pressure was found in 7 cases and 4 of them were even higher than 330 mmH2O.CSF protein elevated in 6 cases and CSF glucose decreased in 6 cases.LMM often occurred in inactive or mild active SLE with the average SLE disease activity index (SLEDAI) score of 4.7±1.5.Systemic manifestations included hematologic changes (6/8),rashes (4/8),renal involvement (4/8) and arthritis (3/8),etc.Treated by autibiotics such as penicillin and medications for SLE,7 patients achieved clinical improvements,1 patient died.Conclusion LMM is one of the opportunistic infections in SLE patients and tends to occur in inactive lupus cases.There are unspecific clinical and laboratory features and CSF changes which maybe overlap and mix up with systemic manifestations of SLE.That's why it is necessary to take continuous cultures of CSF.Early identification and appropriate treatment lead to prognostic improvement of these patients.

Objective To investigate the clinical features of patients with microscopic polyangiitis (MPA) and alveolar hemorrhage (AH),so as to raise the physicians' understanding of this condition.Methods The clinical data of 15 MPA patients combined with AH,who were hospitalized in Peking Union Medical College Hospital from January 2008 to January 2014,were retrospectively analyzed.Results These 15 subjects accounted for 18.1％ of all MPA patients,including 4 males and 11 females with mean age of (59.4 ± 17.9) years old and mean disease duration of 7.5 (1-48) months.Hemoptysis or bloody sputum (14/15) and dyspnea (11/15) were predominant pulmonary manifestations.Hypoxemia appeared in 6 patients (40.0％).An average 8-46 (22.7 ± 4.4) g/L drop of hemoglobin level was seen in all 15 patients.Imaging revealed new bilateral infiltrations or opacities in 13 cases,and bloody bronchoalveolar lavage fluid were found in 6 cases.Compared with MPA patients without AH,the incidence of myalgia [2/15 vs 50.0％ (34/68) ; P =0.009,OR =0.154] and hemoglobin level [(83.2 ± 21.0) g/L vs (102.5 ± 18.7) g/L; P =0.001] were statistically lower in MPA patients with AH.There were no statistical differences in age,sex,interstitial lung disease,renal involvement,peripheral neuropathy and the five factor score between two groups.The proportion of patients who received corticosteroid pulse treatment in MPA with AH group was significantly higher than that of MPA patients without AH [9/15 vs 19.1％ (13/68) ; P =0.003,OR =6.346].After treatment,13 cases achieved remission,while 2 died.Conclusion As an important pulmonary manifestation of MPA,AH could be insidious but fatal.The treatment with steroid,cyclophosphamide and plasma exchange could improve the prognosis.

The clinical data of 13 patients diagnosed as Takayasu's arteritis with cardiomyopathy in Peking Union Hospital were reviewed.Echocardiography showed cardiomegaly (left chambers mainly),diffuse weakened myocardial wall movement and reduced left ventricular ejection fraction.Dilated cardiomyopathy was diagnosed in two patients.Glucocorticoid and cyclophosphamide were the essential medication for treatment.One patient died,1 patient was aggravated and the disease condition in remaining 11 patients was stable or improved.Takayasu's arteritis with cardiomyopathy is a rare disease and it attacks younger people.It is difficult to identify at onset,and the disease deteriorates rapidly.Echocardiography should be performed earlier to evaluate the heart function for early diagnosis and treatment.Administration of large dose of glucocorticoid and cyclophosphamide at early stage may improve the prognosis.

Objective To investigate the growth and exocrine function of BM-MSC derived from PBC patients.Methods To compare the growth patterns and cytokines secretions between PBC patients and healthy controls by student's t test.Results ① There was no difference in growth profile and speed between PBC patients and healthy controls.② The level of TGF-β1 was much lower in the supernatant of BM-MS from OBC patients than health controls [(2.6±1.9)vs (8.2±6.7)ng/ml,t=-3.641,P=0.001].There were no other differences between two groups' BM-MSC.③ The super natant concentration of interlukin-10 of the third BM-MSC subculture from healthy controls was lower than that of the primary subculture [(18.5±5.0) vs (12.4±3.1) pg/ml,t=2.368,P=0.045],and that of hepatic growth factor from the second subcuhure was higher than the primary subculture [(0.21±0.07) vs (0.35±0.08) ng/ml,t=-2.874,P=0.021].There were seldom discrepancies in other cytokines between different generations of BM-MSC.Conclusion BM-MSC from PBC patients may have almost the similar characters in growth pattern and cytokines secretion as,except the TGF-β1,which was much lower than those from healthy controls.The second subculture of BM-MSC might be more suitable for the treatment to patients with PBC.

Objective To study the clinical profile of patients with multicentric reticulohistiocytosis (MRH).Methods The clinical manifestations,image and histologic characteristics of ten patients with MRH were collected,and the relevant literatures were reviewed.Results Seven cases were women.The median (quartile) age was 46.5 (44.5-55.3) years.Clinical symptoms included papulonodular lesions on the skin (ten cases),erosive polyarthritis (ten cases),myositis (four cases),lung nodules (three cases),larynx nodules (one case) and serositis (one case).Biopsy demonstrated infiltration of multinucleated giant cells with CD68 positive and glassy cytoplasm.All of the ten patients were treated with corticosteroids and disease-modifying antirheumatic drugs,skin lesions of seven patients were ameliorated and arthritis was improved in three patients.Conclusion MRH is a systemic disease that has characteristic clinical manifestations and unique histological findings.Deep understanding of this disease can avcid.isdiagnose.Further studies are needed to explore effective treatment.

Objective To synthesize 18 F-DPA-714 and to study its labeling rate, radiochemical purity, stability and biological characteristics. Methods 18F-was reacted with K2CO3/K2.2.2 and then en-gaged in nucleophilic substitution with DPA-714. The crude product was purified by aluminum column and semi-preparation HPLC. The stability of 18 F-DPA-714 was identified in PBS and plasma. The lipid-water partition coefficient (LogP) was determined. Biodistribution analysis and microPET imaging were performed on mice and rats respectively. Results It took about 25 min for synthesizing 18 F-DPA-714, the radiochemi-cal yield was 31.6% (decay not corrected), and the radiochemical purity was ≥99%. The product re-mained stable within 4 h. The LogP of 18 F-DPA-714 was 2.71. Pharmacokinetics of 18 F-DPA-714 was more in line with the two compartment model, with the distribution half-life ( T1/2α) of 2.40 min and the elimina-tion half-life( T1/2β) of 69.15 min. 18 F-DPA-714 was quickly uptaken by tissues after the tail vein injection. It mainly distributed in the lungs, kidneys, and heart, with the radioactive uptake values of (17.85±7.52)%ID/g, (15.41±1.80) %ID/g and (10.56±0.94) %ID/g at 30 min post-injection, respectively. 18F-DPA-714 was mainly metabolized through the liver, and excreted by the kidneys. The uptake in bones was stable. PET dynamic scanning showed that 18 F-DPA-714 accumulated in the brain of aged rats and cleared slowly within 60 min. Conclusions 18 F-DPA-714 prepared in this study has high labeling rate, short synthesis time and small precursor dosage. It displays good biological distribution and blood-brain barrier permeability characteristics.