1.A Case of Multidisciplinary Treatment for Inflammatory Myofibroblastic Tumor Complicated by ANCA-Associated Vasculitis
Shaoying WANG ; Linyi PENG ; Ke ZHENG ; Zhiwei WANG ; Dachun ZHAO ; Xia ZHANG ; Lin ZHAO ; Wenhui WANG ; Weiqing WANG ; Zhenzhen ZHU ; Jin XU ; Min SHEN
JOURNAL OF RARE DISEASES 2026;5(1):43-51
A 51-year-old male presented with nasal obstruction, followed by progressive hearing loss and blurred vision. Imaging identified space-occupying lesions in the paranasal sinuses, orbits, and paraspinal regions, while laboratory tests confirmed positive anti-proteinase 3 anti-neutrophil cytoplasmic antibody(PR3- ANCA) immunoglobulin G (IgG)and markedly elevated serum IgG4. Despite treatment with corticosteroids, immunosuppressants, and radiotherapy, the patient exhibited steroid dependency with relentless disease progression. Following multidisciplinary consultation, a diagnosis of inflammatory myofibroblastic tumor (IMT) coexisting with ANCA- associated vasculitis (AAV) was favored, though IgG4-related disease remained a critical differential. Ultimately, profound immunosuppression precipitated a severe herpesvirus infection, leading to disseminated intravascular coagulation and multiple organ dysfunction syndrome. This case underscores the rarity and diagnostic complexity of concurrent IMT and AAV, highlights the therapeutic dilemma of balancing primary disease control against fatal opportunistic infections, and emphasizes the critical role of multidisciplinary collaboration in the diagnosis and treatment of complex diseases.
2.Longitudinal cohort study on the relationship between cystatin C and the risk of Parkinson's disease in middle-aged and elderly people in China
Xiao LIANG ; Dan WAN ; Ke DU ; Ni GUO ; Shaoying ZHANG ; Gaixia HE ; Lan YANG ; Zongfang LI
Journal of Xi'an Jiaotong University(Medical Sciences) 2025;46(4):656-662
Objective To investigate the relationship between serum cystatin C(CysC)level and the risk of Parkinson's disease(PD)in middle-aged and elderly people in China.Methods Based on the baseline survey data from the China Health and Retirement Longitudinal Study(CHARLS)in 2011,participants who were not diagnosed with PD at the time of the baseline survey were recruited.The onset of PD was tracked and followed up until 2020,and the participants were divided into PD group and non-PD group according to whether they were newly diagnosed with PD in 2020.Multivariable Logistic regression analysis was performed to assess the association between serum CysC level and the risk of PD.Subgroup and interaction analyses were performed to assess effect modifications by age,gender and depression.Additionally,restricted cubic spline(RCS)was used to explore the linear or non-linear relationship between serum CysC level and the risk of PD in different subgroups.Results We included a total of 3 339 subjects in this study,who consisted of 1 495 males(44.77%)and 1 844 females(55.23%).While baseline participants were followed until 2020,32 subjects had a new PD,and the incidence of PD was 0.96%.The median age of PD group was 63.00 years.Multivariable Logistic regression analysis found that CysC was an independent risk factor for the risk of PD,and CysC was positive significantly associated with the risk of PD(OR=2.34,95% CI:1.14-4.82,P=0.021).Subgroup analysis showed that CysC was positively associated with PD in females(OR=2.70,95% CI:1.30-5.58,P=0.007)and subjects aged 60 years or older(OR=5.29,95% CI:1.69-16.53,P=0.004).RCS model indicated a linear relationship between serum CysC level and the risk of PD in females(Ptotal=0.018,Pnon-linear=0.062)and subjects aged 60 years or older(Ptotal=0.024,Pnon-linear=0.379).Conclusion High level of CysC may increase the risk of PD in middle-aged and elderly people,especially in females and those aged 60 years or older.
3.Longitudinal cohort study on the relationship between cystatin C and the risk of Parkinson's disease in middle-aged and elderly people in China
Xiao LIANG ; Dan WAN ; Ke DU ; Ni GUO ; Shaoying ZHANG ; Gaixia HE ; Lan YANG ; Zongfang LI
Journal of Xi'an Jiaotong University(Medical Sciences) 2025;46(4):656-662
Objective To investigate the relationship between serum cystatin C(CysC)level and the risk of Parkinson's disease(PD)in middle-aged and elderly people in China.Methods Based on the baseline survey data from the China Health and Retirement Longitudinal Study(CHARLS)in 2011,participants who were not diagnosed with PD at the time of the baseline survey were recruited.The onset of PD was tracked and followed up until 2020,and the participants were divided into PD group and non-PD group according to whether they were newly diagnosed with PD in 2020.Multivariable Logistic regression analysis was performed to assess the association between serum CysC level and the risk of PD.Subgroup and interaction analyses were performed to assess effect modifications by age,gender and depression.Additionally,restricted cubic spline(RCS)was used to explore the linear or non-linear relationship between serum CysC level and the risk of PD in different subgroups.Results We included a total of 3 339 subjects in this study,who consisted of 1 495 males(44.77%)and 1 844 females(55.23%).While baseline participants were followed until 2020,32 subjects had a new PD,and the incidence of PD was 0.96%.The median age of PD group was 63.00 years.Multivariable Logistic regression analysis found that CysC was an independent risk factor for the risk of PD,and CysC was positive significantly associated with the risk of PD(OR=2.34,95% CI:1.14-4.82,P=0.021).Subgroup analysis showed that CysC was positively associated with PD in females(OR=2.70,95% CI:1.30-5.58,P=0.007)and subjects aged 60 years or older(OR=5.29,95% CI:1.69-16.53,P=0.004).RCS model indicated a linear relationship between serum CysC level and the risk of PD in females(Ptotal=0.018,Pnon-linear=0.062)and subjects aged 60 years or older(Ptotal=0.024,Pnon-linear=0.379).Conclusion High level of CysC may increase the risk of PD in middle-aged and elderly people,especially in females and those aged 60 years or older.
4.Prognostic value of inflammation-based scores and TNM stage for pancreatic cancer patients after radical resection
Zijian SU ; Qunxiong PAN ; Chongren WANG ; Jianhua ZHANG ; Shaoying KE ; Shengli ZHANG
Chinese Journal of Pancreatology 2016;16(5):298-304
Objective To compare the prognostic value of different inflammation-based prognostic scores and Tumor Node Metastasis ( TNM) stage for patients undergoing radical resection of pancreatic cancer with the routine TNM stage in clinical practice.Methods Clinical data of 185 patients with pancreatic cancer who underwent radical surgery were retrospectively analyzed.Based on the inflammation-based prognostic scores ( Glasgow prognostic score ( GPS ) , neutrophil lymphocyte ratio ( NLR ) , platelet lymphocyte ratio ( PLR) , prognostic nutritional index ( PNI ) and prognostic index ( PI ) ) before surgery, univariate and multivariate analyses were used for identifying influential factors on patients′survival.Homogeneity of different scoring systems was compared using likelihood ratio chi-quare test, and linear trend chi-square test, and receiver operating characteristic ( ROC) curve were performed to compare the differentiating ability and single trend of the selected scores with those of routine TNM stage.Results In univariate analysis, preoperative weight loss, serum C-reactive protein, serum albumin, CA19-9, radical surgery, NLR, PLR, GPS, PI, PNI and TNM stage were all significantly associated with patients′overall survival after surgery (P<0.001).In multivariate cox risk model analysis, TNM stage, radical surgery, GPS, NLR, PLR, PI and PNI were independent risk factors for patients′survival after surgery.ROC curve showed that GPS had higher AUC than other scoring systems, but TNM stage had the highest AUC.The homogeneity, differentiating ability and single trend of GPS were higher compared to other inflammation-based prognostic scores, but those of TNM stage were the highest.Conclusions The inflammation-based prognostic scores like GPS, NLR, PLR, PI and PNI were independent prognostic factors for pancreatic caner patients′survival after surgery, and the prognostic value of GPS was superior to that of NLR, PLR, PI and PNI.

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