Objective:Longitudinally extensive transverse myelitis (LETM) could be seen in patients with connective tissue disease (CTD), especially systemic lupus erythematosus (SLE) or primary Sj?gren′s syndrome (pSS). Some patients are combined with neuromyelitis optica spectrum disorders (NMOSD)(termed CTD-LETM-NMOSD) while others without (termed CTD-LETM-non-NMOSD). The aim of this study is to compare the clinical characteristics of CTD-LETM-NMOSD patients to CTD-LETM-non-NMOSD patients.Methods:We retrospectively collected data from 40 CTD patients with LETM who were admitted to the Department of Neurology or Rheumatology at Peking Union Medical College Hospital from Jan, 2006 to Dec, 2016. They were divided into CTD-LETM-NMOSD and CTD-LETM-non-NMOSD two groups. Demographic characteristics, clinical and laboratory features were obtained from the database. Relapse rates and clinical outcome were analyzed by Kaplan-Meier method.Results:Among 40 patients with CTD, 28 (70.0%) were NMOSD while 12 (30.0%) were not. The positivity rates of anti-SSA, antibodies to aquaporin-4 (anti-AQP4) were significantly higher in patients with NMOSD than those in patients with non-NMOSD ( P<0.05). Age, gender, clinical features, disease duration, anti-double-stranded DNA antibody, anti-ribosomal P antibody, antiphospholipid antibodies, expanded disability status scale (EDSS) scores, and magnetic resonance imaging (MRI) features were all comparable between two groups. CTD-NMOSD patients had significantly higher disease relapse rate (75.0% vs. 3/12, P<0.01). Conclusion:Anti-SSA and anti-AQP4 positivity is associated with NMOSD and higher relapse rates, which suggests that NMOSD in CTD-LETM patients may represent distinct characteristics and pathogenesis from patients with CTD-LETM-non NMOSD.

A 29-year-old woman was admitted to the Department of Rheumatology,Peking Union Medical College Hospital due to intermittent rashes,fever and headache.Palpable purpura were symmetrically distributed on the extremities and trunk.Other manifestations included headache with nausea and vomiting.Elevated white blood cell (WBC) count,platelet (PLT) count,erythrocyte sedimentation rate (ESR) and C-reactive protein were the main laboratory findings.Antinuclear antibodies and antineutrophil cytoplasmic antibodies were negative.Examination of the cerebrospinal fluid (CSF) revealed high intracranial pressure,while routine cytology and biochemical tests of CSF were normal.Head MRI scan and PET-CT did not detect remarkable findings.A diagnosis of systemic vasculitis was confirmed by the biopsy of skin lesion which showed inflammatory infiltration of the muscular vessel wall.Combination therapy of corticosteroids and cyclophosphamide lead to a rapid improvement in clinical symptoms and laboratory parameters.The patient was in stable remission till 6 month follow-up.

Objective To explore the relationship among personality traits,aggression,depression,anxiety and suicide ideation by developing a structural equation model.MethodsA total of 2141 participants derived from a stratified random cluster sampling were investigated with Eysenck personality questionnaire(EPQ),aggression questionnaire(AQ),patient health questionnaire depression scale(PHQ-9) ,generalized anxiety disorder scale(GAD-7) and Beck depression inventory(BDI-13).ResultsIn EPQ,the scores of neuroticism,extroversion and psychoticism were (40.00±5.84),(59.84±7.02)and (36.63±6.41) respectively.In AQ,the scores of physical aggression,verbal aggression and anger were (32.41±5.15),(30.43±7.24),and (34.39±6.09) respectively.The median scores of PHQ-9 and GAD-7 were 5 and 6,and the inter-quartile range were 4 and 5.The suicide ideation was correlated with measurement indicator respectively(r=-0.19-0.40,P<0.01).Aggression and depression directly affected suicide ideation(direct effect was 0.27,0.24 respectively) and aggression indirectly affected suicide ideation trough anxiety(indirect effect was 0.02).Personality traits indirectly affected suicide ideation through aggression,anxiety and depression(indirect effect was 0.40).Anxiety indirectly affected suicide ideation through depression(indirect effect was 0.13).ConclusionPersonality traits,aggression,anxiety and depression affect suicide ideation,and these factors have different effecting mechanism.

Objective Immune and inflammatory mechanisms could play a significant role in genesis or progression of interstitial lung disease and pulmonary arterial hypertension,especially in patients with connective tissue diseases.Specific antibodies may predict the occurrence of this condition.Methods The plasma of patients with systemic sclerosis(SSc)or mixed connective tissue disease(MCTD)were screened with enzyme linked immunoserbent assay(ELISA)and Western blotting using recombinant mocsin.The clinical data were recorded and pulmonary function tests(PFTs)were performed in 78 consecutive individuals in order to identify the difference in clinical manifestations between anti-mocsin positive group and negative group.Results Our results showed high titers of anti-mocsin antibodies in 21(53%)of 40 patients with SSc,and 15(39%)of 38 patient with MCTD.The presence of anti-moesin antibodies was significantly correlated with pulmonary involvement(ILD and PAH)in SSc and MCTD patiens(P=0.001).Comparing with the antibody negative group,the total lung capacity[(82±10)% vs(90±14)%,P=0.027),forced vital capacity [(76±13)% vs(85±17)%,P=0.040]and diffu-sing capacity of the lung for carbon monoxide[(58±16)% vs (72±23)%,P=0.014]of PFTs in anti-moesin antibodies positive group were noted to be significantly decreased(P＜0.05).Conclusion Specific antibodies to moesin is prevalent in patients with SSc and MCTD,so it may be an early predictor of pulmonary involvement in patients with SSc or MCTD.

Objective To identify a novel auto-antibody in sera of systemic sclerosis (SSc) patients and to analyze its relevance with SSc-associated interstitial lung disease (ILD). Methods The anti-moesin antibody in the sera of 62 SSc patients, who had participated the European League Against Rheumatism's Scl eroderma Trial and Research Group (EUSTAR), were tested by enzyme linked immunosorbent assay (ELJSA). Patients were grouped by high resolution computerized tomography (HRCT) features, pulmonary function test (PFT) abnormalities, inflammatory markers and disease course. The prevalence and titer (Optical density value) of anti-moesin antibody were compared between groups with t and χ2 test. Results The titer of anti-moesin antibody was significantly higher in the SSc-ILD group than non-ILD group (0.156±0.062 vs 0.107± 0.026, P=0.005). Among SSc patients, the diagnostic sensitivity and specificity of the anti-moesin antibody for ILD was 44.0% and 91.7% respectively (Kappa=0.2, P=0.022). Anti-rnoesin antibody was more prevalent in SSc patients with HRCT features of honeycomb-like lesion, lobular septal thickening and mediastinal lymphadenopathy (P<0.05). SSc patients with deteriorated total lung volume (TLC %) had higher titer of anti-moesin antibody significantly (0.172±0.067 vs 0.133±0.039, P=0.011), as the same tendency in patients with decreased diffusing capacity of the lung for carbon monoxide (DLco% ) but without statistical significant difference (0.153±0.580 vs 0.120±0.340, P=0.089). The anti-moesin antibody was equally prevalent between abnormal ESR, C reactive protein, immunoglobulin and complements groups and their normal controls (P> 0.05). Group of patients who had SSc courses more than or less than 5 years demonstrated similar anti-moesin antibody titers (0.146±0.047 vs 0.164±0.077, P=0.272). However, patients with ILD courses less than 12 months had higher liter of the antibody than controls (0.182±0.073 vs 0.138±0.049, P=0.040). Conclusion This study suggests that the novel anti-moesin antibody has comparatively high specificity for SSc-associated ILD patients, which may contribute to further understanding the pathogenesis of ILD in SSc patients. Further investigations are deserved to evaluate the application of anti-moesin antibody in facilitating early screening and evaluation of ILD.

ObjectiveTo study the characteristics and the impact of lymph node metastasis on radical resection for pancreatic head cancer to provide a theoretical basis for lymphadenectomy in radical resection.To study the reliability of using a surgical microscope to detect lymph nodes in radically resected specimens of pancreatic head cancer.MethodsLymph nodes in the specimens after radical pancreaticoduodeneetomy (pancreaticoduodenectomy + D2 regional lymphadenectomy) were identified using a surgical microscope and they were grouped using the JPS standard.The position and the frequency of the lymph nodes retrieved,and their association with other clinicopathologic factors were analysed.The results were compared with the data published in 2004 on 46 patients to evaluate the reliability of using a surgical microscope.ResultsLymph node metastasis was detected histopathologically in 101 patients (67.3％).The median number of lymph nodes retrieved in the specimens as detected using the surgical microscope was 38.2.The most commonly involved lymph node groups were No.13 (64.5％),No.14 (51.7％),No.17 (38.6％),No.12 (25.8％),No.16 (20.8％).Lymph node metastasis was significantly associated with tumour T stage,tumour invasion and differentiation,preoperative serum level of CA19-9 and CA72-4,but not with patient age,sex,or tumour location.There were no significant differences between the results and the data of the previous study in 2004.ConclusionsExtended lymphadenectomy is necessary because extensive lymph node metastasis was common.Surgical microscopy is an effective and reliable method to detect lymph nodes in resected specimens of pancreatic head cancer for accurate pathologic staging.

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.

Objective:To improve the understanding of the relationship between antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE).Methods:The clinical characteristics and process of diagnosis and treatment of a case was reported and analyzed. This patient was initially diagnosed as antiphospholipid syndrome and later developed new skin lesion and positive anti-dsDNA antibody, which made the diagnosis of systemic lupus erythematosus.Results:A 15-year-old girl suffered acute pulmonary embolism, lower extremity deep vein thrombosis, and high titer of anti-phospholipid antibody, but negative for other autoantibodies. So primary antiphospholipid syndrome was diagnosed. Symptoms were improved after thrombolysis and anticoa-gulation treatment. During the follow-up period, the patient developed malar erythema, lymphocytopenia, proteinuria, positive ANA, anti-dsDNA antibody, and reduced complement level. So she was diagnosed with systemic lupus erythematosus. After glucocorticoid pulse therapy and immunosuppressants treatment, the symptoms were relieved and lupus disease activity was decreased.Conclusion:A few primary APS patients can progress into SLE. Patients with risk factors such as a younger age of onset, positive ANA and positive Coomb's test results should be closely followed up.

Pulmonary arterial hypertension (PAH) is a clinicopathological syndrome caused by the increase of pulmonary artery, and it is the most serious complication of connective tissue disease (CTD). In recent years, a lot of progress has been made in the diagnosis, treatment and evaluation of PAH. Chinese Rheumatology Association formulated this recommendation on the basis of current experience and guidelines, in order to promote early screening, early diagnosis and early intervention of CTD-PAH, as well as patient follow-up and management, to improve the prognosis of CTD-PAH patients.

Objective:To investigate the effects of NOD-like receptor protein 3(NLRP3) inflammasome activation on the proliferation, migration and extracellular matrix desposition of activated pancreatic stellate cells(PSCs).Methods:The rat PSCs were isolated, cultured and identified, and were divided into control group or LPS group based on the pretreatment with LPS (10 μg/ml for 24 hours) or without. The expression of NLRP3 inflammasome associated molecules in PSCs culture medium was detected by ELISA. The PSCs with NLRP3 inhibition were constructed by shRNA carrying lentivirus infection and were divided into LPS+ negative control group and LPS+ lentivirus group based on whether the cells were treated with LPS and infected by lentivirus or not. The alteration in cell proliferation and migration were detected by CCK-8 kit and transwell chamber method. The expression of extracellular matrix α-SMA and collagen in PSCs was detected by immunofluorescence staining and the expression of TGF-β mRNA was analyzed by RT-qPCR.Results:The cytoplasm of PSCs which were cultured for 24 hours was rich in bright annular lipid droplets, and the cells expressed desmin. After 7 days of culture, the cell became larger in size, the lipid droplets basically disappeared, and the cells were activated and expressed α-SMA. The expression of caspase-1, IL-1β and IL-18 in the supernatant of PSCs culture medium in LPS group were significantly higher than those in control group (1.55±0.04 vs 0.65±0.03), (2.02±0.04 vs 1.05±0.05) and (1.70±0.05 vs 0.97±0.03), respectively. After inhibiting by lentivirus infection, the expression of NLRP3 in the lentivirus group (0.25±0.04) was significantly lower than that in negative control group (0.68±0.05). In control group, LPS group, LPS+ negative control group and LPS+ lentivirus group, the A490 values was 0.61±0.02, 1.15±0.06, 0.96±0.05, and 0.56±0.01, respectively; the migrating PSCs number was (64.12±4.58), (121.67±8.02), (111.67±4.67) and (69.67±8.08)/HF, respectively; the relative expression of α-SMA was 0.78±0.05, 4.12±0.04, 3.81±0.06 and 0.88±0.05, respectively; the relative expression of collagen was 0.65±0.03, 3.43±0.02, 2.67±0.02 and 0.48±0.03, respectively; and the expression of TGF-β mRNA was 0.22±0.03, 0.89±0.01, 0.86±0.03 and 0.43±0.02, respectively. The A490 value, the migrating cells number, the expression of α-SMA, collagen and the expression of TGF-β mRNA in LPS group and LPS+ negative control group was significantly higher than those in control group and LPS+ lentivirus group, and all the differences were statistically significant (all P value <0.05). Conclusions:NLRP3 inflammasome activation may accelerate the extracellular matrix deposition and pancreatic fibrogenesis by promoting PSCs proliferation and migration ability via regulating the biological functions.