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.
Objective To investigate the MRI features of the autoimmune pancreatitis （AlP）. Methods MRI data of 8 patients with AIP were retrospectively analyzed. Results MRI showed that diffuse swelling of the pancreas in 8 cases. T1WI signal intensity homogeneous or inhomogeneous decreased， and T2WI signals intensity homogeneous or inhomogeneous increased. In arterial phase the enhancement of the lesion was not obviously，in portal venous phase there was gradual increase of enhancement. There was coated sample annular enhancement around pancreas， and the degree of enhancement was slightly lower than the pancreatic parenchyma. Pancreatic duct was irregular narrow. Conclusion AIP is a special kind of chronic pancreatitis，MRI features of AIP are helpful for the diagnosis and treatment of AIP．
ObjectiveTo summarize the clinical characteristic of IgG4 related lung disease. MethodsThe clinical manifestation,laboratory examination,imaging,diagnosis and treatment data of 2 patients with IgG4 related lung disease admitted in the PLA General Hospital from January 2000 to January 2014 were collected and analyzed retrospectively.Related literatures were also reviewed. ResultsThe serum IgG level of IgG4 related lung disease might be normal and the levels of IgG1,2,3 and 4 might increase.Some autoantibodies were positive.IgG4 related lung disease could be easily misdiagnosed as Sjogren's syndrome associated with interstitial pneumonia when the lacrimal gland and salivary gland were involved,and the chest CT occasionally showed multiple vesicles.The immunochemical staining of lung tissues revealed the increase of IgG4/IgG and the number of IgG4 positive lymphatic plasma cells >10/HP.The combination of clinical manifestation,laboratory examination,imaging and pathology results was more conducive for the diagnosis of IgG4 related lung disease.After the treatment of glucocorticoid,the clinical symptoms were relieved and the serum IgG4 level obviously decreased. ConclusionIgG4 related lung disease is rare in clinic,which can involve the lung alone or multiple organs.The increase of IgG4/IgG indicated by the immunochemical staining of lung tissues is the gold standard for diagnosis.Glucocorticoid has good treatment effect in IgG4 related lung disease.
ObjectiveTo explore the epidemiological and clinical features of hepatic hemangioma. MethodsThe clinical data of patients with hepatic hemangioma who were diagnosed in the First Affiliated Hospital of Guangxi Medical University from 2003 to 2011 were retrospectively analyzed, and then analyzed the epidemiological and clinical characteristics of hepatic hemangioma. ResultsEight hundreds and eighteen patients with hepatic hemangioma were included in the study.Among them, 398 cases (48.7%) were male, 420 cases (51.3%) were female, and there were no significant differences in constitute of gender for each year (χ2=9.912, P=0.271), but there were significant differences in constitute of gender between different age groups (χ2=18.791, P=0.000 1), male patients were more than female in the group of aged over 60 years old.There was no special clinical manifestations of hepatic hemangioma.There were 75 patients (9.2%) combined autoimmune diseases in this group.The size of hepatic hemangioma ranged from 0.5-39.0 cm, the median diameter was 3.0 cm.There were significant differences between different gender, the tumor size of female patients were larger than male's (P < 0.05).The tumor happened mostly in the right hepatic lobe (57.2%), and there were no significant differences of the tumor location in different gender (P > 0.05).Hepatic hemangioma with a single lesion was most common (70.0%), and the right lobe lesions were more common than the left lobe lesions in single lesion group, multiple lesions in double lobes were most common in multiple lesion group. ConclusionsHepatic hemangioma has a certain distribution pattern in gender, age, size, position, and so on, further research in prevention and controlled strategy need to be carried out in the future.At the same time, more in-depth research in the related factors participated in occurrence and development of hepatic hemangioma also need to be carried out, especially for the relationship between gender, age, autoimmune diseases, and hepatic hemangioma, which is worthy to be discussed.
Alternative splicing plays an important role in the pathogenesis, diagnosis, treatment and prognosis of autoimmune diseases. Alternative splicing is universal and non-preferred in autoimmune diseases, and exon skipping is the most common type in alternative splicing types. The occurrence and development of autoimmune diseases can be influenced by the 5′ splicing, 3′ splicing, number change of exons, splicing affected by the single nucleotide polymorphism and the variance of gene expression levels. Moreover, different single nucleotide polymorphisms of the same gene can affect the development of various autoimmune diseases. This review summarizes the role of different forms of alternative splicing in various autoimmune diseases, and aims to provide a basis for further study of the conditions in different development stages of autoimmune diseases and the regulatory mechanism of different levels of splicing isoforms.