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.

Objective To investigate clinical relevance of U1-nuclear ribonucleoprotein antibody (anti-U1 RNP) in Chinese patients with systemic sclerosis (SSc). Methods In total, 131 Chinese patients with SSc were prospectively and consecutively recruited into Scleroderma Trials and Research Group sponsored by European League Against Rheumatism (EUSTAR) from clinical database of Peking Union Medical College Hospital ( PUMCH). Their clinical features, visceral lesions and laboratory findings including detection of anti- U1 RNP were recorded. Relevance between existing anti-U1 RNP and clinical characteristics and other laboratory indicators of SSc was analyzed statistically. Results Eighty-seven of the 131 patients presented as diffused SSc (dSSc) , 36 limited SSc ( lSSc) and eight SSc/systemic lupus erythematosus (SLE) overlap syndrome, with positive rate of anti-U, RNP of 28. 2 (37/131) percent in patients with SSc. Positive rate of anti-U1 RNP in dSSc was similar to that in lSSc groups [28. 7% (25/87) and 25. 0% ( 9/36 ) , respectively, P = 0. 673]. But significantly higher prevalence of pulmonary arterial hypertension and cardiac involvement, and less white blood cell and platelets counts were observed in the patients with positive anti-U1 RNP than in those with negative anti-U1 RNP (all P < 0. 05 ). There was no statistically significant difference in skin sclerosis score and prevalence of arthritis, myositis and interstitial lung disease was observed between those with positive and negative anti-U1 RNP ( P > 0. 05 ) . Detection rate of anti-DNA topoisomerase I ( Scl-70 antibody) was higher in SSc patients with negative anti-U, RNP. Conclusions U1 RNP antibody is a common antibody in Chinese patients with SSc, and detection for it combined with other autoantibodies will be helpful for diagnosing of SSc and predicting development of pulmonary arterial hypertension, cardiac and hematological involvements in them.

Objective To investigate the location and the pathogens of systemic lupus erythematosus (SLE) patients who died from infection.Methods Dead cases of hospitalized SLE patients in Peking Union Medical Hospital from January 1986 to May 2011were retrospectively analyzed.Results Severe infection was an important cause of mortality in patients with SLE.There were 252 dead cases of SLE in total from 1986 to 2011,in which 97 cases died from severe infection.The proportion of patients died from infection was gradually increased during the past 26 years,which was 25.7％,24.1％,31.6％,34.9％ and 60.3％ in 1986-1990,1991-1995,1996-2000,2001-2005 and 2006-2011respectively.Lung was the most common site of infec-t ion,accounting for 65％ of all dead cases.Among the 31cases with identified microorganism,14 patients had single microorganism infection and 17 patients had mixed infections.In the single microorganism infection patients,fungal infection contributed to the cause of death in 9 out of 14 (64％) patients,of which 4,2,1,1,and 1 cases were infected with Pneumocystis carinii, Aspergillus fumigates,Fusarium,Candida tropicalis and Cryptococcus respectively.There were 3 cases of bacteria infection,including 2 cases with Methicillinresistant staphylococcus,aureus and 1case with Klebsiella pneumoniae infection.Two patients died from pulmonary tuberculosis.In 17 patients with mixed infection cases, 14 deaths were caused by bacteria infection,including Acinetobacter baumannii, Eschefichia coli, Enterococcus, Klebsiella pneumoniae,Pseudomonas aeruginosa and Enterobacter cloacae.Eleven patients died from mixed fungus infection,including 6 cases of Aspergillus fumigates,3 cases of Candida tropicalis,3 cases of Pneumocystis carinii,2 cases of Aspergillus flavus,1 case of Soil Aspergillus,2 cases of Candida albicans,1 case of Candida glabrata and Candida Krusei.In addition to bacteria and fungi infection,cytomegalovirus infection occurred frequently in SLE death cases.Conclusions Severe infection has been the most frequent cause of death in SLE patients,in which lung infection is the leading cause.Pneumocystis carinii,Aspergillus fumigates,Acinetobacter baumannii and cytomegalovirus are the major pathogens that cause death in SLE patients.

ObjectiveTo investigate the changes of major causes of death of patients with systemic lupus erythematosus(SLE).MethodsDeath cases with SLE from January 1986 to May 2011 in Peking Union Medical College Hospital were retrospectively analyzed.ResultsOut of 3554 patients with SLE,252 patients died,including 223 women and 29 men.The mortality rate was 7.2％ among female and 6.2％ among male,the overall mortality rate was 7.1％.The mortality rate in SLE patients had dropped steadily in the past 25 years,but there was a mild increase of mortality in 2006-2011 compared with that in 2001-2005 (5.7％vs 5.3％ ).In addition to infection,neuropsychiatric lupus and lupus nephritis had become the most common causes of death in SLE patients during the past 25 years.Furthermore,diffuse alveolar hemorrhage,severe pulmonary hypertension,coronary heart disease,thrombocytopenia,interstitial lung disease,lupus pneumonia,gastrointestinal hemorrhage, intestinal obstruction and multiple organ failure were the common causes of death,accounting for 4.4％,4.4％,3.2％,2.8％,2.4％,2.0％,2.0％,1.2％ and 1.2％ of all the death cases respectively.From 1986 to 2005,infection,neuropsychiatric lupus and lupus nephritis were the most common causes of death in patients with SLE,whereas the cases dying from lupus nephritis had decreased obviously and severe pulmonary hypertension had become the third most frequent causes of death during the past 5years.From 1986 to 1990,lupus nephritis,infection and neuropsychiatric lupus accounted for 31.4％,25.7％and 25.7％ of death cases respectively.From 1991 to 1995,lupus nephritis,infection and neuropsychiatric lupus accounted for 27.6％,24.1％ and 24.1％ respectively.From 1996 to 2000,infection,neuropsychiatric lupus and lupus nephritis took up 31.6％,21.1％ and 15.8％ respectively.From 2001 to 2005,infection,neuropsychiatric lupus and lupus nephritis took up 34.9％,20.6％ and 7.9％ respectively.From 2006 to 2011,infection, neuropsychiatric lupus and pulmonary hypertension accounted for 60.3％, 11.8％ and 7.4％ respectively.The mortality in the first year was the highest in the whole disease course,accounting for 32.5％ of patients.Deaths caused by neuropsychiatric lupus and infection happened most frequently during the first year,accounting for 41.9％ and 32.9％,whereas deaths caused by lupus nephritis occurred most frequently 10 years later,accounting for 32.3％.Age and gender had significant association with the major causes of death.The male patients took up 50.0％ of the total patients dying from coronary heart disease,in which 75.0％ of patients were older than 50 years.ConclusionInfection,neuropsychiatric lupus and lupus nephritis are the three most common causes of death in SLE patients fron 1986 to 2005.Severe pulmonary hypertension has become the third most frequent causes of death during the past 5 years instead of lupus nephritis.Severe infection has increased significantly and has been the leading cause of death in SLE patients in recent 5 years.

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.

Objective To assess the clinical features of primary biliary cirrhosis(PBC)associated with systemic sclerosis(SSc)in order to facilitate recognition of this overlap syndrome(SSc-PBC). Methods The clinical data of 9 patients with SSc-PBC in Peking Union Medical College Hospital were retrospectively studied with literature review. Results ① Nine patients including 8 female were at a mean age of(54±8)years. Sevene patients initially presented with SSc and developed PBC over(4.3±2.3)years. ② Eight patients were diagnosed limited cutaneous SSc(leSSc), which included 7 patients with CREST syndrome. The most frequent manifestations were Raynaud's phenomenon(8/9)and esophageal dysfunction(8/9). Four patients with PBC manifested subclinically, but laboratory results revealed increased ALP/GGT in most patients(8/9).Two patients received liver biopsies, which confirmed the diagnosis of PBC. ③ Antinuclear antibodies(ANA)and anti-mitochondrial antibody(AMA)were detected in all the patients, among them, 8 with positive anticentromere antibody(ACA)and 8 with positive AMA-M2. ④ UDCA and glucocorticosteroid might help early stage patients with SSc-PBC, but lack efficacy in patients complicated with interstitial lung disease, pulmonary hypertension or cirrhosis. Conclusion PBC might be overlapped with SSc, especially lcSSc(CREST syndrome). Screening of autoantibodies, such as ACA, AMA and AMA-M2, could help rheumatologists early recognition of SSc-PBC and improve the prognosis of this overlap syndrome by early intervention.

Objective : To measure and compare condyle-related indexes(volume, surface area, anterior space, upper space and posterior space) in adult female patients with different sagittal malocclusions with the aim of providing a reference for clinical treatment. Methods : The CBCT database of Qingdao Stomatology Hospital was searched to identify patients with high-angle adult skeletal malformations with the following classifications: classⅠ(n=30), class Ⅱ(n=30), and class Ⅲ(n=30). The CBCT images associated with the sample population were stored according to DICOM3.0 medical digital image communication standards. Mimics10.01 software was used to read and prepare three-dimensional reconstructions of the condylar, and the above-listed indicators were measured and statistically analyzed. Results : There were no significant differences between the right and left sides among the three groups (P > 0.05). For parameters including condylar anterior joint space and post-joint space, the differences among the three groups were significant (P < 0.05). There was no significant difference in parameters related to condylar articular space between classⅠand class Ⅱ(P > 0.05). The differences between skeletal classesⅠand Ⅲ and between skeletal ClassesⅡ, and Ⅲ were statistically significant (P < 0.05). Conclusion Condylar position varies among high-angle adult woman with skeletal malocclusions with different sagittal skeletal patterns.

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.

Objective:To describe a series of systemic sclerosis (SSc) patients with the combination of scleroderma renal crisis (SRC) and pulmonary arterial hypertension (PAH).Methods:The medical records of 472 SSc patients in Peking Union Medical College Hospital between January 2012 and October 2020 were reviewed and a retrospective analysis of the characteristics of patients with SRC and PAH among SSc patients was conducted.Results:Thirteen patients suffered from SRC and PAH in the SSc patients, 1 case was limited cutaneous SSc, and 12 cases were diffuse cutaneous SSc. Five patients had renal crisis before pulmonary arterial hypertension, 4 patients had pulmonary arterial hypertension before the occurrence of renal crisis, and the remaining 4 patients were found at the same time. Among them, 11 patients had Raynaud's phenomenon, 7 had gastrointestinal bleeding, 6 had pulmonary edema and 3 had telangiectasias. Twelve cases were positive for anti-nuclear antibodies and 4 cases were positive for anti-Scl-70 antibodies. N-terminal pro-brain natriuretic peptide (NT-proBNP)>1 400 ng/L in 11 patients. Two patients had thrombotic microangiopathy (TMA). Among the 13 patients, 3 patients died during hospitalization, 2 patients were lost to follow-up, and 2 patients died within 5 years of follow-up. Six patients survived, and 1 of the 4 patients with regular dialysis were discharged from dialysis.Conclusion:In patients with scleroderma, SRC can occur earlier, later than, or at the same time with SSc-PAH. Patients may have a higher incidence of gastrointestinal bleeding and higher level of NT-proBNP. PDE5i or ERAs may be beneficial.