【摘要】 目的 分析合并免疫指標異常的視神經脊髓炎臨床特點。 方法 回顧性分析2009年5月-2010年11月收治的62例視神經脊髓炎患者中24例合并免疫指標異常患者的臨床資料。24例均為女性，發病年齡14~53歲。對其臨床表現、視覺誘發電位、影像學檢查結果、免疫檢查結果進行分析。 結果 所有患者均有脊髓和視神經同時或先后受累的表現。24例視覺誘發電位檢查23例異常。脊髓MRI顯示病變集中于頸段、上胸段脊髓。頸段和胸段脊髓同時受累17例，單純頸段脊髓受損6例，單純胸段脊髓受損1例。所有患者抗核抗體滴度均≥1∶100，合并抗SSA抗體陽性14例（55.5％），同時合并抗SSB抗體陽性11例（45.8％），合并抗Rib抗體陽性1例，合并抗SCL-70抗體陽性1例，合并抗dsDNA抗體1例。 結論 視神經脊髓炎合并免疫指標異常的患者以女性較為多見，易復發，青壯年患者發病率最高。脊髓MRI示病變集中于頸段、上胸段脊髓，表現為長節段脊髓損害。視神經脊髓炎患者合并結締組織病的病例較多。【Abstract】 Objective To analyze the clinical features of neuromyelitis optica (NMO) combined with abnormal immune parameters. Methods We retrospectively reviewed the clinical data of 24 patients with NMO and abnormal immune parameters among the 62 NMO patients who were admitted into our department between May 2009 and November 2010. All patients were female, aged from 14 to 53 years. We analyzed their clinical manifestations, visual evoked potentials, imaging results, and immunological examinations. Results All patients had simultaneous or successive spinal cord and optic nerve involvement. Twenty-three patients had abnormal visual evoked potential. MRI showed that the lesions focused on the cervical and upper thoracic spinal cord. Both cervical and thoracic spinal cord were involved in 17 cases; there were 6 cases of simple cervical spinal cord injury and 1 case of simple thoracic spinal cord damage. Antinuclear antibody titer of all the patients was ≥1∶100. Combined positive anti-SSA antibody occurred in 14 patients (55.5%); Concomitant positive anti-SSB antibodies occurred in 11 patients (45.8%); Combined positive anti-Rib antibodies occurred in 1 patient; Combined positive anti-SCL-70 antibody occurred in 1 patient; and combined positive anti-dsDNA antibodies occurred in 1 patient. Conclusions NMO combined with abnormal immune parameters mainly occurs in female patients, especially in young people. Recurrence rate is high. MRI shows that the lesions focus mainly on the cervical and upper thoracic spinal cord, manifesting the characteristic of long segment damage. And NMO is frequently combined with connective tissue disease.
Autoimmune pancreatitis (AIP) is a unique subtype of chronic pancreatitis, which shares many clinical presentations with pancreatic ductal adenocarcinoma (PDA). The misdiagnosis of AIP often leads to unnecessary pancreatic resection. 18F-FDG positron emission tomography/ computed tomography (PET/CT) could provide comprehensive information on the morphology, density, and functional metabolism of the pancreas at the same time. It has been proved to be a promising modality for noninvasive differentiation between AIP and PDA. However, there is a lack of clinical analysis of PET/CT image texture features. Difficulty still remains in differentiating AIP and PDA based on commonly used diagnostic methods. Therefore, this paper studied the differentiation of AIP and PDA based on multi-modality texture features. We utilized multiple feature extraction algorithms to extract the texture features from CT and PET images at first. Then, the Fisher criterion and sequence forward floating selection algorithm (SFFS) combined with support vector machine (SVM) was employed to select the optimal multi-modality feature subset. Finally, the SVM classifier was used to differentiate AIP from PDA. The results prove that texture analysis of lesions helps to achieve accurate differentiation of AIP and PDA.
Objective〓〖WTBZ〗To observe the clinical features of autoimmune optic neuropathy (AON). 〖WTHZ〗Methods〓 〖WTBZ〗The clinical data of 58 patients with AON from Jan. 2006 to Dec. 2007 were retrospectively analyzed. The patients had undergone routine ophthalmological, neurological examination, visual field test, all set of autoimmune antibody test, brain MRI. 〖WTHZ〗Results〓〖WTBZ〗In 93 eyes of 58 patients with AON, the lowest best corrected visual acuity (BCVA) was lt;01 in 68 eyes (731%), 10 patients (172%) had other symptoms of nervous system, 14 patients (241%) had lesions of nonneurological system. Positive antinuclear antibody was found in 43 patients (796%); other abnormal antibodies were also found, including antiSSA/SSB, antidsDNA, antihistonic, anticardiolipin, and antihuman leukocyte antigen B27 antibodies. Systematic connective tissue disease presented in 20 patients (345%), such as sicca syndrome, systemic lupus erythematosus, and Behcet disease. 32 patients (552%) had abnormal brain MRI, and the BCVA of 49 eyes (777%) improved significantly after hospitalization. 〖WTHZ〗Conclusion〓〖WTBZ〗Patients with AON always have poor visual function, some of whom associate with other systems, as well as damages to other parts of the nervous system. While some AON patients are secondary to systemic connective tissue disease involving the optic nerve, the majority of these patients are isolated autoimmune optic neuropathy.
The pathogenesis of Vogt-Koyanagi Harada disease (VKH) has not yet been fully defined. Current studies mainly suggest that VKH is actually an autoimmune disease, especially related to the immune response mediated by various signal transduction pathways involved in the function of T cells. In recent years, the influence of the balance imbalance of various T cell subsets in cellular immunity on the pathogenesis of VKH has been a hot research direction. Currently, T helper cell 17/T regulatory cells, balance is the focus of clinical research, meanwhile, new discoveries and potential clinical treatment schemes have been made for related cellular pathways, particularly the Janus kinase/signal transducers and activators of transcription pathway and NF-kappa B pathway. The exploration of B cells in the pathogenesis of VKH has also achieved initial results through the successful application of various targeted drugs. In the future, further screening and localization of genes or proteins that are abnormally regulated or expressed in VKH, for which early comprehensive and in-depth exploration will be helpful, thus improve the efficacy of clinical treatment programs and develop new therapeutic targets.
ObjectiveTo evaluate the diagnostic value of monitoring 1,3-beta-D-glucan (G test) in patients with autoimmune disease complicated with invasive fungal disease (IFD). MethodsA retrospective study was performed in hospitalized patients in the First Affiliated Hospital of Zhengzhou Universisty who were diagnosed as autoimmune disease with lung infection during the immunosuppressive therapy between January 2014 and January 2016. A total of 372 patients were enrolled in this study. All subjects were classified according to the 2006 diagnostic criteria and treatment of invasive pulmonaary fungal infection, with serum 1,3-β-D-glucan results not included in the diagnosis. There were 18 cases with proven IFD, 35 cases with probable IFD, and 70 ceses with possible IFD. Fifty-three patients with proven IFD or probable IFD were as a case group, and another 249 patients with no evidence for IFD were as a control group. The value of the G test for diagnosis of automimmune disease with IFD was analyzed by ROC curve. ResultsThe serum 1,3-β-D-glucan level was significantly higher in the case group when compared with the control group [median (interquartile range): 135.0 (63.1 to 319.0) pg/ml vs. 75.9 (41.2 to 88.1) pg/ml, P<0.05]. When the cut-off value of serum 1,3-β-D-glucan level was set at 93.8 pg/ml, the sensitivity, specificity, positive predictive value, and negative predictive value for diagnosis of autoimmune disease with IFD were 0.65 (95% CI 0.56 to 0.73), 0.87 (95% CI 0.83 to 0.92), 0.70 (95% CI 0.64 to 0.81), and 0.83 (95% CI 0.79 to 0.88), respectively. ConclusionThe 1,3-beta-D-glucan test is a valuable method for diagnosis of IFD in patients with autoimmune disease.
ObjectiveTo investigate the clinical heterogeneity and auto-immunologic state in patients with diabetic ketosis (DK) or diabetic ketoacidosis (DKA).
MethodsDiabetic patients who presented with DK/DKA were recruited from West China Hospital of Sichuan University from January 1, 2000, to December 31, 2008. We analyzed the clinical classification, biochemical profiles, and auto-immunologic state of the patients.
ResultsAmong them, 257 (15.0%) patients had type 1 diabetes, while 883 (51.6%) with type 2 diabetes, and 515 (30.1%) could be typed as "atypical diabetes" or "untying diabetes". The average age of hospitalized patients with type 1 diabetes was (31.1±13.5) years, which was significantly lower than that of the type 2 diabetes patients [(58.1±13.2) years] (P＜0.001). The most common contributing factor for DK/DKA was infection, followed by noncompliance with therapy. Glutamic acid decarboxylase antibody positive rate was 4.37% in patients with "atypical diabetes", which was similar with type 2 diabetes group (2.69%, P=0.79), but lower than that of the patients with type 1 diabetes (13.79%, P＜0.001).
ConclusionsWe conclude that DK or DKA can occur not only in type 1 diabetic patients but also in patients with type 2 diabetes under infection or stress condition. DK/DKA patients have a high clinical heterogeneity. The auto-immunologic state and β-cell function have significant implications for the diagnosis and classification of diabetes.
Neuromyelitis optica spectrum disorders (NMOSDs) are a class of immune-mediated inflammatory demyelinating diseases of the central nervous system that mainly involve the optic nerve and spinal cord. As an important environmental factor, the gut microbiota may play an important role in the occurrence and development of NMOSDs. Previous studies have shown that the structure and number of intestinal flora in NMOSDs patients are different from those of normal healthy people. The altered intestinal flora may cross-react with central nervous system autoantigens, induce T cell differentiation, and affect short-chain fatty acids, etc. The metabolite secretion pathway triggers the occurrence of NMOSDs. The summary of the changes of gut microbiota in patients with NMOSDs and the possible underlying mechanisms by summarizing the literature, aim to provide more effective treatments for the prevention and treatment of NMOSDs in the future.
