Objective:To summarize the clinical manifestations of anti-synthetase syndrome (ASS) with interstitial lung disease (ILD) patients with different antibody subtypes and the skeletal muscle pathology of ASS.Methods:A total of 106 ASS-ILD patients admitted to the First Medical Center of Chinese People′s Liberation Army General Hospital from May 11, 2015 to June 25, 2023 were included. Their intramuscular and extramuscular clinical manifestations were collected. The correlation between different antibody subtypes in patients with ASS and the various subtypes of ILD was investigated. The skeletal muscle pathological characteristics of 13 ASS patients were also summarized.Results:Among the 106 ASS-ILD patients, 56 (52.8%) were anti-JO-1 antibody positive, 19 (17.9%) were anti-PL-7 antibody positive, 11 (10.4%) were anti-PL-12 antibody positive, 14 (13.2%) were anti-EJ antibody positive, and 6 (5.7%) were anti-OJ antibody positive. All the patients had ILD [including nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia (OP), mixed pneumonia]. In all the patients, 46.2% (49/106) had cardiac damage, 37.7% (40/106) had arthritis, 29.2% (31/106) had myasthenia gravis, 24.5% (26/106) had myalgia, and 19.8% (21/106) had Raynaud′s phenomenon. The incidence of NSIP was 75.0% (42/56) in the anti-JO-1 antibody-positive group, significantly higher than other groups (anti-PL-7 antibody-positive group, 8/19;anti-PL-12 antibody-positive group, 3/11;anti-EJ antibody-positive group, 5/14;anti-OJ antibody-positive group, 2/6; P=0.001). UIP was most common in the anti-PL-7 antibody-positive group (8/19). OP was most frequent in the anti-PL-12 antibody-positive group (5/11). The incidence of arthritis was highest in the anti-JO-1 antibody-positive group (51.8%, 29/56). The anti-Ro-52 antibody-positive rate was significantly higher in the anti-EJ antibody-positive group (12/14) than in the other 4 groups [anti-JO-1 antibody-positive group, 33.9% (19/56); anti-PL-7 antibody-positive group, 10/19; anti-PL-12 antibody-positive group, 6/11; anti-OJ antibody-positive group, 0/6; P=0.001]. ASS skeletal muscle pathology was manifested as necrotizing myopathy pattern (6/13), inflammatory myopathy pattern (4/13), and nonspecific myopathy pattern (3/13). All the 106 patients received methylprednisolone as the basic treatment. Among them, 69 patients (65.1%) received methylprednisolone alone, while 37 patients (34.9%) received combination therapy involving immunosuppressants, whose symptoms improved after treatment. Conclusions:A discernible correlation exists between the clinical manifestations of ASS with ILD and specific antibody subtypes. ASS patients generally respond well to immunotherapy. ASS can manifest as 3 distinct skeletal muscle pathological patterns: necrotizing myopathy pattern, inflammatory myopathy pattern, and nonspecific myopathy pattern.

Objective:To summarize the clinical manifestations of anti-synthetase syndrome (ASS) with interstitial lung disease (ILD) patients with different antibody subtypes and the skeletal muscle pathology of ASS.Methods:A total of 106 ASS-ILD patients admitted to the First Medical Center of Chinese People′s Liberation Army General Hospital from May 11, 2015 to June 25, 2023 were included. Their intramuscular and extramuscular clinical manifestations were collected. The correlation between different antibody subtypes in patients with ASS and the various subtypes of ILD was investigated. The skeletal muscle pathological characteristics of 13 ASS patients were also summarized.Results:Among the 106 ASS-ILD patients, 56 (52.8%) were anti-JO-1 antibody positive, 19 (17.9%) were anti-PL-7 antibody positive, 11 (10.4%) were anti-PL-12 antibody positive, 14 (13.2%) were anti-EJ antibody positive, and 6 (5.7%) were anti-OJ antibody positive. All the patients had ILD [including nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia (OP), mixed pneumonia]. In all the patients, 46.2% (49/106) had cardiac damage, 37.7% (40/106) had arthritis, 29.2% (31/106) had myasthenia gravis, 24.5% (26/106) had myalgia, and 19.8% (21/106) had Raynaud′s phenomenon. The incidence of NSIP was 75.0% (42/56) in the anti-JO-1 antibody-positive group, significantly higher than other groups (anti-PL-7 antibody-positive group, 8/19;anti-PL-12 antibody-positive group, 3/11;anti-EJ antibody-positive group, 5/14;anti-OJ antibody-positive group, 2/6; P=0.001). UIP was most common in the anti-PL-7 antibody-positive group (8/19). OP was most frequent in the anti-PL-12 antibody-positive group (5/11). The incidence of arthritis was highest in the anti-JO-1 antibody-positive group (51.8%, 29/56). The anti-Ro-52 antibody-positive rate was significantly higher in the anti-EJ antibody-positive group (12/14) than in the other 4 groups [anti-JO-1 antibody-positive group, 33.9% (19/56); anti-PL-7 antibody-positive group, 10/19; anti-PL-12 antibody-positive group, 6/11; anti-OJ antibody-positive group, 0/6; P=0.001]. ASS skeletal muscle pathology was manifested as necrotizing myopathy pattern (6/13), inflammatory myopathy pattern (4/13), and nonspecific myopathy pattern (3/13). All the 106 patients received methylprednisolone as the basic treatment. Among them, 69 patients (65.1%) received methylprednisolone alone, while 37 patients (34.9%) received combination therapy involving immunosuppressants, whose symptoms improved after treatment. Conclusions:A discernible correlation exists between the clinical manifestations of ASS with ILD and specific antibody subtypes. ASS patients generally respond well to immunotherapy. ASS can manifest as 3 distinct skeletal muscle pathological patterns: necrotizing myopathy pattern, inflammatory myopathy pattern, and nonspecific myopathy pattern.

Objective:To summarize the clinical, thigh magnetic resonance (tMRI) and electromyographic (EMG) characteristics in patients with immune-mediated necrotizing myopathy (IMNM).Methods:A total of 32 IMNM patients who were admitted to the Department of Neurology from April 2019 to April 2021 were enrolled at the First Medical Centre of Chinese PLA General Hospital. According to the type of antibody, the patients were divided into anti-SRP antibody positive (SRP +) group, anti-HMGCR antibody positive (HMGCR +) group and seronegative (SN) group. The gender, age, course of disease, myositis antibodies, extramuscular manifestations, EMG were collected and analyzed among three groups. The characteristics of skeletal muscle were assessed by tMRI inflammatory edema and fat infiltration scores. Analysis of variance, Kruskal-Wallis test and Chi-square test were used to compare the differences in different clinical characteristics and tMRI scores among the three groups. When there was a statistical difference among the three groups, the comparison between the two groups was corrected by the Bonferroni method. Result:(1) Of the 32 patients, 20 were females (62.5%).The median age of onset was 47±14 years, 25 (78.1%) patients had an acute or subacute course.There were 17 (53.1%) with SRP +, 8 (25.0%) with HMGCR +, and 7 (21.9%) with MSAs (myositis specific antibodies) negative. Anti-Ro52 antibody was the most common combined antibody (12/32, 37.5%), among which 10 were in SRP +group.(2) The CK of all patients were elevated, median was 5 948 (4 229, 7 664) U/L. There was no statistical difference of MMT scores among three groups. The proximal limb score was lower than distal limb ( P<0.01). The axial muscle score was lower than the distal limb score ( P<0.05).(3) Extramuscular manifestations of HMGCR + group were lower than those of the other two groups (12.5% vs. 71.4% and 76.5%, P<0.017). Rash (60.0% vs.14.3%, P<0.05) and interstitial pulmonary diseases (70.0% vs. 14.3%, P<0.05) were more common in patients with anti-SRP coexistence with anti-Ro52 than those with isolated anti-SRP. Connective tissue disease was more common in SN group (57.1% vs. 11.8% and 0, P<0.017).(4) tMRI showed fascial edema of SN group was more obvious than that of the other two groups ( P<0.017). There was no statistical difference in the degree of fat infiltration and inflammatory edema among three groups, but SRP + group had more cases of early fat infiltration.(5) Myotonic potentials (25.0% vs. 0 and 0, P<0.017) and compound repetitive discharges (CRDs) (50.0% vs. 5.9% and 0, P<0.017) were common in HMGCR + group. Proteomic analysis found significantly different expressed proteins in skeletal muscle of patients with myotonic potentials or CRDs were associated with cytoskeleton, cell junction and extracellular matrix. Conclusion:IMNM with pure anti-SRP antibody positive and anti-HMGCR positive were mainly affected by skeletal muscles. Those who were co-positive for anti-SRP antibody and anti-Ro52 antibody had more extramuscular manifestations, which might be a special subtype of SRP + group. This study proposed for the first time that myofascial inflammatory edema is an early sign of SN-IMNM injury. EMG of HMGCR +group were more prone to myotonia potential and CRDs.

Axial myopathy is a general term for a group of myopathy involving the axial muscles. It refers to a group of skeletal myopathy in which paraspinal muscles are individually or significantly affected, with or without the involvement of whole body skeletal muscles. Axial muscle weakness is often ignored in clinical practice. The evaluation of axial muscle is mainly the evaluation of the paraspinal muscles (erector spinae) in current literature. The clinical manifestations of paraspinal muscle weakness are dropped head syndrome and camptocormia. Physical examination and skeletal muscle magnetic resonance imaging, especially the whole body muscle magnetic resonance scan, are vital for the evaluation of axial muscle. It is of great clinical significance to increase attention to the diagnosis and differentiation of axial myopathy, which is helpful to avoid missing treatable diseases and improve the understanding and early recognition of associated myopath.

Objective? To construct the critical illness patient inter-hospital referral process of primary hospitals based on Delphi method so as to provide a basis for the inter-hospital critical illness patient standardized referral process in the medical alliance model. Methods? The inter-hospital referral process was designed by retrieving literatures at home and abroad, and was demonstrated and revised with the modified Delphi method. Results? We constructed a total of 30 items from four aspects including work preparation of medical staff as well as reception medical staff before inter-hospital referral, risk-prevention during referral and patients' condition after referral. Among two rounds of expert consultation, the active coefficients were all 100% and the authority coefficients were all 0.843 respectively. Conclusions? The critical illness patient inter-hospital referral process of primary hospitals based on modified Delphi method accords with the principle of scientific nature and operability which has positive a meaning for standardizing clinical inter-hospital critical illness patient referral.

Objective To investigate the oncologic and reproductive outcomes after progestin treatment of complex endometrial hyperplasia(CEH) and grade 1 endometrial carcinoma(EC).Methods In a retrospective study, data were obtained for patients with CEH or grade 1 EC at presumed stage IA(without myometrial invasion) who wished to preserve fertility and were treated at the Peking Union Medical College Hospital, China, between January 1, 2000 and December 31, 2011.Patients had received oral medroxyprogesterone acetate(250-500 mg/d) or megestrol acetate(160-480 mg/d) for at least 6 months.Results Among 55 included patients, median age was 32 years(range 21-41 years).41(75%) achieved complete response after a median period of 6(3-24) months.Complete response was less frequent among obese than nonobese patients(4/12 [33%] vs 37/43 [86%];P=0.001).Disease recurrence was recorded in 10(24%) patients with complete response;the 5-year recurrence-freesurvival rate was 71%.Among the 33 patients who retained a desire to conceive, 17(52%) became pregnant.Conclusions Fertility-sparing management with oral progestin is effective and safe.Obesity is associated with a lower probability of long-term success.

Objective: To investigate the utilization status of epidermal growth factor receptor tyrosine kinase inhibitor (EGFR-TKI) in 11 hospitals of Zhejiang province from 2009 to 2015, and to analyze the use rationality.Methods: The doctor's advice in 40 days annually was collected in 11 hospitals of Zhejiang province from 2009 to 2015, and the drug consumption, frequency of utilization (DDDs), defined daily cost (DDC) and drug utilization index (DUI) were analyzed for the patients with lung cancer treated with EGFR-TKI.Results: Icotinib, erlotinib and gefitinib were the three prevalent EGFR-TKIs used in Zhejiang province, and icotinib started to be used in clinics in 2013.The overall cost of EGFR-TKIs increased year by year during 2009 and 2015, and the total amount of sales increased by 4.67 times in 2015 when compared with that in 2009.Generally, the DDDs value of erlotinib showed a decreasing trend, however, that of icotinib and gefitinib rose year by year during 2009 and 2015.Erlotinib had the highest DDC followed by gefitinib and icotinib.The mean value of DUI of the three targeted drugs was about 1.Conclusion: The utilization of EGFR-TKI is reasonable in 11 hospitals of Zhejiang province with increasing comsuption.

OBJECTIVE:To provide reference for rational use of leucocyte increasing drugs in patients with lung cancer. METHODS:The application of drugs in lung cancer patients in 40 d each year were collected from 11 Zhejiang hospitals during 2009-2014,and then analyzed retrospectively in consumption sum,DDDs,DDC and department distribution,etc. RESULTS:The proportion of consumption sum of leucocyte increasing drugs in total consumption sum decreased generally in 11 Zhejiang hospital during 2009-2014,decreasing from 781 995.50 yuan in 2009(3.28%)to 626 792.80 yuan in 2014(1.53%). Top 3 departments in the list of consumption sum of leucocyte increasing drugs were oncology department(29.00%),radiotherapy department(27.08%) and respiratory medicine department(9.93%). Top 3 drugs in the list of consumption sum during 2009-2014 were recombinant hu-man granulocyte colony-stimulating factor,Coenzyme complex for injection and Leucogen tablets;top 3 drugs in the list of DDDs were Leucogen tablets,Berbamine tablet and recombinant human granulocyte colony-stimulating factor;Coenzyme complex for in-jection,recombinant human granulocyte colony-stimulating factor and human GM-CSF took the first three place in the list of DDC during 2009-2013,and Leucogen tablets were the top one in 2014. CONCLUSIONS:The application of leucocyte increasing drugs in lung cancer patients is decreasing year by year in 11 Zhejiang hospitals,and those with definite therapeutic efficacy and moder-ate price predominate clinical application predominate in the clinical practive.

Objective To evaluate the current status and outcomes of pelvic exenteration (PE) for recurrent cervical cancer.Methods The following electronic databases has been searched on recurrent cervical cancer management and treatment:Chinese Biological Medicine Disk (CBM),PubMed and Cochrane library.All retrieved studies had to fulfill the following inclusion criteria:cohort studies of recurrent cervical cancer,containing information of detailed patient and operation characteristics as well as the survival rate.Only publications in the English literature were included.All eligible literatures between Jan.1990 and Aug.2013 were assessed for quality.Relevant basic characteristics,complications,survival rate and prognostic factors were reviewed.Results There were eight trials involving 607 patients with cervical cancer received PE,including 515 cases with recurrent disease and 92 cases with primary disease.Four hundred and ninety patients had received total pelvic exenteration (TPE) operation,103 underwent anterior pelvic exenteration(APE) and 14 received posterior pelvic exenteration(PPE).The 5-year overall survival rate for recurrent cervical cancer fluctuate from 26.7％ to 56.0％.Complication rates were from 34.3％ to 83.3％ and the mortality rate was 1.2％ (7/607).Among the relevant factors affecting survival time,resection margin status seemed to be the most important.Conclusion Based on this systematic review,PE does help improve the survival of recurrent cervical cancer patients on the basis of strict selection of candidates.

ObjectiveTo retrospectively analyze the clinical characteristics and outcomes of endometrial carcinoma patients aged 45 years and younger MethodsFifty-two cases of endometrial carcinoma aged 45 years and younger were treated in Peking Union Medical College Hospital They were further divided into group A (35 years of age and younger) and group B (older than 35 years) Clinical data of these patients were reviewed and the two groups were compared ResultsPatients aged 45 years and younger accounted for 12 7% of all the endometrial carcinoma cases About 50% of the patients were nulliparous, infertile or had irregular menstruation and endometrial hyperplasia, 29% were obese, 23% had polycystic ovaries Eighty-three percent of the patients were stage [ Int ernational Federation of Gynecology and Obstetris (FIGO),1988] Group A had mo re polysystic ovaries and atypical endometrial hyperplasia than group B (53% v s 9%, 59% vs 26% respectively, P