Objective:To study the clinical and immunological features of two case of rare antisynthetase syndrome (ASS), so as to improve the level of diagnosis and treatment.Methods:Two cases with rare antisynthetase syndrome admitted to the First Affiliated Hospital of Zhengzhou University from July 2020 to August 2022 were collected.Results:The two rare ASS were anti-Zo antibody and anti-Ha antibody positive patients, both of which had interstitial lung disease (ILD) as the main clinical manifestation and positive anti-Ro52 antibody. Two rare antisynthetase autoantibodies manifested cytoplasmic ANA indirect immunofluorescence (IIF) staining pattern, but it is different from the cytoplasmic dense speckled pattern of several common ASS antibodies. After treatment with glucocorticoids and immunosuppressants, case 1 died of respiratory failure due to a long course of disease and late diagnosis, the lung lesions of case 2 improved significantly.Conclusion:When encountering the cytoplasmic ANA fluorescent pattern in ILD patients, especially with anti-Ro52 antibody, it is necessary to screen more myositis specific antibodies to rule out the possibility of rare ASS.

Antineoplastic drugs refer to the drugs that act at the cellular and molecular levels to inhibit tumor growth or eliminate tumors through pathways such as cell killing,immune regulation,and endocrine regulation.Antineoplastic drugs generally including chemotherapeutic drugs,molecular targeted therapeutic drugs,immunotherapeutic drugs,and endocrine therapeutic drugs.The management and rational application of antineoplastic drugs in medical institutions are related to the safety of patient treatment.The standard for management of antineoplastic drugs use in clinical is compiled by the Pharmaceutical Affairs Committee of China Hospital Association,which specification requirements 18 key elements in the organizational management and system,medication management,drug monitoring and evaluation of antineoplastic drug management in healthcare institutions.This standard is applicable to all levels and types of healthcare institutions carrying out oncology diagnosis and treatment.This paper describes the methodology and basic content of the standard,hoping to providing a reference for medical institutions to carry out relevant work.

OBJECTIVE To investigate the clinical efficacy and safety of rituximab （RTX） followed by belimumab （BLM） in patients with severe systemic lupus erythematosus（SSLE）. METHODS Nine SSLE patients， who were treated with RTX followed by BLM for more than 6 months in the Department of Rheumatology and Immunology of the First Affiliated Hospital of Zhengzhou University from October 2020 to June 2021， were enrolled. Baseline clinical data of patients， laboratory examination results and basic treatment status at weeks 0， 4， 12， and 24 of medication were collected retrospectively. The patients’ systemic lupus erythematosus disease activity index （SLEDAI） score， glucocorticoid dosage and serological indicators （complement C3， complement C4， serum albumin， and 24-hour urine protein quantification） level were analyzed. At the same time， the occurrence of adverse drug reaction was collected. RESULTS All 9 patients completed more than 24 weeks of RTX followed by BLM therapy. All patients suffered from renal impairment， of which 7 （77.8%） had renal pathology support， 3（33.3%） had blood system damage and 2 （22.2%） had nervous system damage. During treatment， with the prolongation of treatment time， the SLEDAI score， 24- hour urinary protein quantification， and glucocorticoid dosage of patients showed a significant downward trend， and ultimately decreased to the normal index level （P＜0.05）； serum albumin， complement C3 and complement C4 all showed a significant upward trend， eventually rose to the normal index level （P＜0.05）. During treatment and follow-up， 1 patient developed herpes zoster， 1 patient developed upper respiratory tract virus infection， and 1 patient developed urinary system bacterial infection. All patients recovered after symptomatic treatment. CONCLUSIONS In sequential use of RTX followed by BLM for SSLE， early administration of RTX can quickly stabilizethe condition， significantly alleviate clinical symptoms， and gradually normalize specific serological indicators； subsequent administration of BLM can reduce the type and dosage of basic treatment drugs； there is no increase in the incidence of adverse drug reactions.

Objective:To investigate the application value of spiral CT combined with a disintegrin and metalloproteinase 8 (ADAM8), neuron-specific enolase (NSE) and cytokeratin 19 fragment (CYFRA21-1) measurements in the clinical diagnosis of lung cancer.Methods:Fifty patients with lung cancer who received treatment in Shanxi Rongjun Hospital from February 2018 to December 2019 were included in the lung cancer group. Fifty patients with benign lung disease who concurrently received treatment in Shanxi Rongjun Hospital were included in the benign lung disease group. Fifty 50 healthy controls who concurrently received treatment in Shanxi Rongjun Hospital were included in the control group. Serum levels of ADAM8, NSE and CYFRA211 were compared among the three groups. Three groups received spiral CT scans. ADAM8-, NSE- and CYFRA211-positive detection rates were analyzed. The receiver operating characteristic (ROC) curve was used to analyze the efficacy of different methods in the diagnosis of lung cancer. Serum ADAM8, NSE and CYFRA211 levels in patients with lung cancer at different TNM stages were compared.Results:Serum levels of ADAM8, NSE and CYFRA211 in the lung cancer and benign lung disease groups were (381.69 ± 34.82) ng/L, (255.28 ± 30.48) ng/L, (16.87 ± 3.11) μg/L, (9.27 ± 2.11) μg/L,(13.54 ± 8.10) μg/L, (3.01 ± 1.34) μg/L, respectively, which were significantly higher than those in the control group [(225.83 ± 24.19) ng/L, (7.42 ± 2.35) μg/L, (1.78 ± 1.02) μg/L, t = 5.352, 25.994, 4.142, 17.142, 5.165, 10.186, all P < 0.05]. Serum levels of ADAM8, NSE and CYFRA211 in the lung cancer group were significantly higher than those in the benign lung disease group ( t = 19.316, 14.299, 9.069, all P < 0.05). The positive detection rate of lung cancer by spiral CT combined with ADAM8, NSE and CYFRA211 measurements was 92.00%, which was significantly higher than that by spiral CT combined with a single detection ( χ2 = 7.862, 9.000, 11.422, 9.000, all P < 0.05). The ROC curve analysis revealed that the area under the ROC curve, sensitivity and specificity of the combined method were 0.916, 0.920 and 0.900, respectively, which were significantly higher than those of spiral CT combined with a single detection. Serum ADAM8, NSE and CYFRA211 levels at TNM stages III-IV were (430.18 ± 36.43) ng/L, (20.05 ± 3.49) μg/L, (15.93 ± 8.22) μg/L, respectively, which were significantly higher than those at TNM stages I-II [(314.72 ± 30.85) ng/L, (12.49 ± 2.67) μg/L, (10.25 ± 6.35) μg/L, t = 11.777, 8.312, 2.644, all P < 0.05). Conclusion:Spiral CT combined with serum ADAM8, NSE and CYFRA211 levels can greatly increase the diagnostic sensitivity and specificity of lung cancer, which is worthy of clinical application.

Objective:To investigate the correlations of SOX17 gene polymorphisms at the rs1072737, rs9298506 and rs10958409 loci with sporadic intracranial aneurysm (IA) in patients from Anhui province. Methods:A case-control study was performed on 162 patients with sporadic ruptured IA admitted to our hospital from January 2017 to December 2020 and 182 age-matched controls from Inpatient or physical examination center at the same time-period. The genotype and allele frequencies of SOX17 gene at the rs1072737, rs9298506 and rs10958409 loci between the 2 groups were analyzed and compared to determine the influence of different genotypes and alleles in IA. According to gender, the subjects were divided into male and female subgroups; according to rupture of IA, the subjects were divided into ruptured and un-ruptured subgroups; the risk factors and protective factors for IA in these subgroups were verified. Results:There was no significant difference in genotype distributions at rs10958409 locus between the two groups ( P>0.05). The results of multivariate Logistic regression analysis indicated that GG+GT genotype ( OR=0.913, 95%CI: 0.845-0.990, P=0.031) and allele G ( OR=0.805, 95%CI: 0.718-0.932, P=0.029) at rs1072737 locus were independent protective factors for IA, and GG+AG genotype ( OR=1.043, 95%CI: 1.008-1.084, P=0.011) and allele G ( OR=1.003, 95%CI: 1.001-1.007, P=0.023) at rs9298506 locus were independent risk factors for IA. GG+GT genotype and G allele at rs1072737 locus were still risk factors for IA in both males and females ( P<0.05), while GG+AG genotype and G allele at rs9298506 locus were still protective factors for IA in both males and females ( P<0.05). There was no significant difference in in genotype distributions at rs1072737 and rs9298506 loci between ruptured subgroup and un-ruptured subgroup ( P>0.05). Conclusions:In Anhui province, GG+GT genotype (allele G) carriers in SOX17 gene at rs1072737 locus have a relatively low risk of IA, while GG+AG genotype (allele G) carriers in SOX17 gene at rs9298506 locus have a relatively high risk of IA. There is no correlation of SOX17 gene polymorphisms at rs1072737 and RS9298506 loci with rupture of IA.

OBJECTIVE:To study the relationship of GSTP 1(rs1695)(simply as GSTP 1)gene polymorphism with the hematological toxicity in autologous hematopoietic stem cell transplantation(AHSCT)patients who used CBV regimen (cyclophosphamide,carmustine,etoposide). METHODS:A total of 83 AHSCT patients receiving CBV regimen were retrospective analyzed in our hospital during Apr. 2015-Jun. 2017. The gene polymorphism of GSTP 1 A313G was detected by fluorescence staining in situ hybridization. The hematological toxicity and the incidence of agranulocytosis fever,the implantation time of leukocyte,neutrophils and platelet were analyzed statistically. The relationship of GSTP 1 with above indexes were analyzed. RESULTS:Among 83 patients,gene variation was observed in one gene loci at least of 28 patients(33.73%).The gene frequency of A allele was 81.3%,while that of G allele was 18.7%. The reduce time of Ⅳ grade leukopenia,Ⅳ grade neutropenia and Ⅳgrade thrombocytopenia in GSTP 1 AA genotype patients were(8.91 ± 1.25),(9.02 ± 1.19),(11.56 ± 1.58)d after chemotherapy;those of patients with GSTP 1 313 allele G(AG/GG genotype) were(8.61 ± 1.17),(8.68 ± 1.19),(11.44 ± 1.34)d after chemotherapy. The implantation time of leukocyte,neutrophils and platelet in patients with GSTP 1 AA genotype were(11.98±1.99),(10.44±1.35),(15.55±2.18)d after autologus peripheral blood stem cell reinfusion;those of patients with GSTP1 313 allele G(AG/GG genotype)were(12.41±2.44),(10.36±1.62),(16.29±3.15)d after autologus peripheral blood stem cell reinfusion. The case number of grade Ⅲ-Ⅳ anemia were 24 and 11,accounting for 43.64% and 39.29% of corresponding genotype patients. The case number of agranulocytosis fever in patients with GSTP 1 AA genotype or GSTP 1 313 allele G(AG/GG genotype)were 21 and 11 during transplantation,accounting for 38.18% and 39.29% of corresponding genotype patients, respectively,without statistical significantly(P>0.05). CONCLUSIONS:There is no relationship between GSTP 1 gene polymorphism and hematological toxicity of AHSCT patients receiving CBV regimen.

Objective To investigate the serum levels of dickkopf-related protein 1 (DKK1) and sclerostin (SOST) in patients with axial spondyloarthritis treated with selective cyclo-oxygenase 2 inhibitor and its relation to clinical efficacy.Methods A randomized double-blind controlled trial with axial spondyloarthritis (ax-SpA) was carried out in our hospital.The data from patients in a single center was collected and analyzed.Serum DKK1 and SOST levels were measured by enzyme-linked immuno sorbent assay (ELISA)method before and after 12 weeks treatment,then correlation analysis were conducted for DKK1 and SOST levels with erythrocyte sedimentation rate (ESR),C reactive protein (CRP),Bath ankylosing spondylitis disease activity index (BASDAI),Bath ankylosing spondylitis functional index (BASFI) and SPARCC of the sacroiliac joint inflammation score.Chi-square tests were used for analyzing of categorical data.Fisher exact tests were performed when the expected frequencies were less than 5.Two independent samples t-test was used to compare the difference between groups.Single sample t-test was used to ompare the differences between data before and after treatment.Pearson or Spearman correlation was used for correlation analysis.Results After 12 weeks of treatment,a total of 116 patients completed the follow-up,including 57 cases of imrecoxib group and 59 cases of the celecoxib group.There were no statistically significant difference between the two groups (P＞0.05).The level of serum DKK1 was significantly increased after treatment [(393±137) pg/ml,vs (542±274)pg/ml,P＜0.05].The serum level of SOST increased significantly [(39±19) pg/ml vs (57±36) pg/ml,t=5.814,P＞0.05],too.The difference between the two groups was not statistically significant (P＞0.05).Spearman correlation analysis showed that serum DKK1 was positively correlated with serum SOST (r=0.226,P=0.015).A significantcorrelation was found between SOST level and ESR,CRP,finger to floor distance,left and fight lumbar side flexion and Schober's test (ESR:r=-0.379,P＜0.01;r=-0.309,P=0.001;r=-0.225,P=0.015;r=0.185,P=0.047;r=0.247,P=0.008;r=0.214,P=0.021).Conclusion Imrecoxib and celecoxib have similar efficacy on relieving the signs and symptoms of patients with ax-SpA.Short-term application of selective COX-2 inhibitors can increase DKK1 and SOST and possibly delay radiographic progression.

Objective To investigate the sleep quality of patients with systemic lupus erythematosus (SLE) and to analyze its influencing factors. Methods A total of 156 patients with SLE who were treated in the Rheumatology Department of the First Affiliated Hospital of Zhengzhou University from October 2016 to January 2017 were selected as the research objects by convenience sampling method. All the participants were investigated by general information questionnaire, Pittsburgh sleep quality index (PSQI) scale and systemic lupus erythematosus disease activity index (SLEDAI). The influencing factors of patients' sleep quality was analyzed by single factor analysis and multiple linear regression analysis. Results The score of PSQI scale of SLE patients was (10.04±5.83). It was reported that 62.23% of patients had poor sleep quality. The main reason for night sleep disturbance were easy to wake up at night, night frequent urination and sensory heat. Single factor analysis showed that there was significant difference in total sleep quality among SLE patients with different age, education level, number of hospitalization, course of the disease, daily average dose of glucocorticoid and disease activity index (P< 0.05). Multiple linear regression analysis showed that age, course of the disease, daily average dose of glucocorticoid and disease activity index were the influencing factors of SLE patients' sleep quality (P<0.01). Conclusions The results indicate that sleep quality of SLE patients is influenced by age, course of disease, daily average dose of glucocorticoids and disease activity index. Medical staff should pay attention to patients' sleep problems and give sleep nursing interventions on the basis of treatment in order to improve the sleep quality and life quality of SLE patients.

Objective:To evaluate the changes of quality of life in patients with axial spondyloarthritis (ax-SpA) after treatment with non-steroidal anti-inflammatory drugs (NSAIDs) by the 36-item Short Form Health Survey (SF-36).Methods: 120 patients diagnosed with ax-SpA were collected in the first Affiliated Hospital of Zhengzhou University from October 2014 to September 2015.They all agreed to be treated with the special drugs and assessed by special scale.Then they all signed the agreement.In the 3 months,double-blind,parallel controlled trial patients were randomized to 200 mg twice daily (bid) imrecoxib,or 200 mg twice daily (bid) celecoxib.They were assessed for the changes of quality of life at enrollment and after three months of NSAIDs therapy by the SF-36 of Chinese edition.The correlation between quality of life and erythrocyte sedimentation rate (ESR),C-reactive protein (CRP),Bath Ankylosing Spondylitis Disease Activity Index (BASDAI),Bath Ankylosing Spondylitis Functional Index (BASFI),Spondylo Arthritis Research Consortium of Canada (SPARCC) was analyzed.Results: A total of 116 ax-SpA patients completed the study and 4 patients were lost to follow-up.We used the SF-36 scale to assess the quality of life in patients with ax-SpA before and after 3 months,NSAIDs treatment.The treatment effects were not statistically significant difference between the two drugs (P>0.05).After all the patients were treated with NSAIDs for 3 months,there was statistically significant difference (P<0.05) of the physical functioning,role-physical,bodily pain,general health,social functioning,role-emotional;and there was no statistically significant difference (P>0.05) of vitality and mental health.The positively significant correlations had been identified between BASDAI and PF,RP,BP,GH,VT,SF,RE (P<0.05),while no significant correlation was found between BASDAI and MH (P>0.05).A positively significant correlation had been identified between BASFI and PF,RP,BP,GH,SF,RE,MH (P<0.05),while no significant correlation was found between BASFI and VT (P>0.05).The ESR was positively correlated with SF,RE (P<0.05);and CRP was positively correlated with SF,MH (P<0.05);and SPARCC was positively correlated with PF (P<0.05).BASDAI and BASFI were the important influence factors of PF (P<0.05);and BASDAI was the important influence factor of BP,GH,VT,RE(P<0.05);BASFI was the important influence factor of RP,SF,MH(P<0.05).Conclusion: Non-steroidal anti-inflammatory drugs can improve the quality of life of the ax-SpA patients.Imrecoxib and celecoxib have the equivalent curative effect.SF36 scale is suitable for the assessment of the quality of life in patients with ax-SpA.

Objective To analyze the clinical characteristics of respiratory involvement in relapsing polychondritis(RPC). Methods The clinical data of 38 patients with respiratory (larynx, trachea and bronchus) involvement in RPC were retrospectively analyzed. Results The incidence of respiratory involvement in patients with RPC was 51.35%(38/74), and the most common symptoms were cough, wheezing, chest tightness and dyspnea. The incidences of erythrocyte sedimentation rate (ESR) increasing, C- reactive protein (CRP) increasing, fibrinogen increasing, D- dimer increased and rheumatoid factor (RF) positive in patients with respiratory involvement were significantly higher than those in patients without respiratory involvement: 47.37% (18/38) vs. 30.56% (11/36), 52.63% (20/38) vs. 33.33% (12/36), 31.58% (12/38) vs. 25.00% (9/36), 21.05% (8/38) vs. 13.89% (5/36) and 36.84%(14/38) vs. 5.56% (2/36), and there were statistical differences (P<0.05). CT was the main method to discover the respiratory involvement, and MRI could detect early cartilage inflammation lesions. Laryngoscope and bronchoscope could early detect mucosa and cartilage damage. Pathology was given priority to lymphocytes and neutrophils infiltration. Some patients had epithelium metaplasia and even canceration. Primary treatment methods were glucocorticoids combined with immunosuppressant. Airway stenosis and infection was the main factors influencing the prognosis of patients. Conclusions The respiratory involvement is not uncommon in RPC, and early CT, MRI, laryngoscope and bronchoscope examination is an important means of early diagnosis.Early glucocorticoid combined immunosuppressive therapy is the key to achieve good prognosis.