目的總結自身免疫性胰腺炎（AIP）的影像學特點。 方法回顧性分析13例經手術病理學檢查或糖皮質激素治療證實的AIP患者的臨床資料。 結果13例均行CT檢查，7例同時行MRI檢查、DWI掃描及磁共振胰膽管造影（MRCP）檢查，2例同時行超聲檢查。8例表現為胰腺彌漫性腫大，4例表現為胰腺局限性腫大，1例表現為胰腺混合型腫大。CT動態增強掃描結果提示，胰腺病變呈“雪花狀”漸進性強化，T1WI信號減低，T2WI信號略高，DWI信號增高；MRCP檢查可見膽總管胰內段呈“鳥嘴樣”狹窄；超聲檢查顯示胰腺病變部位腫大，回聲減低。 結論AIP具有典型的影像學特征，影像學檢查是發現和診斷AIP的重要手段。
Autoimmune hemolytic anemia (AIHA) is an autoimmune disease in which the life span of red blood cells is shortened by red blood cell autoantibodies. Due to immune intolerance and abnormal immune regulation, the hyperfunction of B lymphocytes produces too many red blood cell autoantibodies. Anti-CD20 monoclonal antibody is a second-line drug for warm antibody AIHA and first-line drug for cold antibody AIHA by reducing B lymphocytes. At present, the optimal dose of anti-CD20 monoclonal antibody in the treatment of AIHA has not been determined. There are no reports on the treatment of primary AIHA with second- or third-generation anti-CD20 monoclonal antibodies.
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.
Objective To investigate the postoperative biochemical changes and the strategy of steroid treatment for autoimmune pancreatitis （AIP）. Methods Six patients with AIP from January 2007 to October 2011 in this hospital were included in this study. Four patients received pancreaticoduodenectomy or distal pancreatectomy for being preoperatively misdiagnosed as malignant tumors. Two patients avoided the unnecessary resection and received the internal drainage for obstructive jaundice and postoperative steroid medication because of a confirmation of AIP by intraoperative frozen biopsy. All the patients underwent routine examinations， including liver function， CA19-9 level， and imaging data. Measurement of serum IgG or IgG4 was performed for fewer patients after operation. Results CA19-9 levels were normal or slightly elevated in six AIP patients. For five patients with AIP in the head of pancreas， preoperative serum γ-GT levels were elevated. For these patients， whether pancreaticoduodenectomy or the internal drainage were performed， TB/DB levels were transiently elevated on day 1 or 4 after operation but dropped to below preoperative levelsor normal levels on day 7 after operation， and serum γ-GT levels among them presented a downward. Serum γ-globulin levels exhibited a downward trend among four AIP patients after resection， while a upward trend was found in another two AIP patients receiving internal drainage. From the limited data， elevated serum level of IgG in the AIP patients were found. As to IgG4， the only case was tested on month 7 after operation， the serum IgG4 level was elevated. CT showed that the swollen status of pancreas obviously reduced on month 3 after operation as compared with before operation.Conclusions Postoperative steroid therapy of AIP should be based on the serum γ-globulin level and operation method. CT or MRI imaging is used to evaluate the response to steroid treatment for the AIP patients.
Serum anti-retinal autoantibodies (ARA) are a group of autoantibodies that bind to retinal auto-antigens with significant biological importance in pathological processes such as retinal degeneration, inflammatory microenvironment formation, and tissue destruction. In recent years, the expression of serum anti-retinal antibodies has been found to be upregulated in patients with various blinding retinal diseases such as age-related macular degeneration, autoimmune retinopathy, and retinitis pigmentosa, closely correlated with the progression of diseases. However, current researches on ARA are incomplete, lacking animal experiments and large randomized controlled clinical trials. As a result, the exact mechanism of ARA is not well understood. Although several studies have demonstrated that serum ARA has an important diagnostic value in hereditary, autoimmune, and degenerative retinal diseases, there still lacks recognized laboratory tests and laboratory indicators with high specificity and sensitivity. Clinical symptoms should be considered when making definitive diagnosis of the diseases. Therefore, clarifying the mechanisms of ARA in retinal dystrophies provides new ideas in early diagnosis and treatments of retinal diseases, which is clinically and scientifically important for the maintenance of visual functions.
【Abstract】Objective To study the relation between iodine and experimental autoimmune thyroiditis(EAT). Methods Establishment of animal model was performed with iodine and thyroglobulin(TG).The rats were randomly divided into 5 groups: normal control group(NC), low iodine group (LI,500 μg/L), high iodine group(HI,500 mg/L), TG+Freund adjuvant group(TG) and TG+Freund adjuvant+HI group(TG+HI). The rats in TG group and TG+HI group were rejected hypodermically with TG emulsified by complete Freund adjuvant, and strengthen immunity was conducted with TG emulsified by incomplete Freund adjuvant on 15 days. After that, strengthen immunity was done weekly till the end of the experiment. Serum TGAb and TPOAb were measured by radioimmunoassay. Observation of the pathological changes of thyroid gland was also done. Results Thyroid follicular destruction and lymphocytic infiltration in the TG+HI group (3.83±1.72) and HI group (3.00±0.89) were significantly higher than that of the NC group(0.67±0.82)，P＜0.05. The results of the TG group were higher compared with the NC group, but there were no significant differences between them（Pgt;0.05）. The levels of TGAb in the TG+HI (4.990±1.505)，HI (3.589±1.240) and TG group (4.883±1.198) were significant higher than those of the NC group (0.642±0.454) and the LI group (0.707±0.240)，P＜0.01. The levels of TPOAb in TG+HI group (1.475±0.523) and TG group (1.316±0.606) were significantly higher than those of the NC group (0.365±0.196) and the LI group（P＜0.01）. Serum TGAb and TPOAb levels were positively correlated with the histological grades of lymphocytic thyroiditis(r=0.9,P＜0.05）. Conclusion Excessive iodine intake may induce the occurrence of EAT. The induction of EAT with excessive iodine and TG may be more efficient.
Objective To summarize the clinical characteristics of interstitial pneumonia with autoimmune features (IPAF). Methods The interstitial lung disease (ILD) patients diagnosed in our department between January 2010 and August 2013 were retrospectively analyzed to screen out the patients with IPAF.The clinical manifestations, laboratory examination, imaging, pulmonary function and treatment were summarized. Results In 254 ILD patients, 25 patients met the diagnosis criteria of IPAF, and 26 patients were diagnosed with definite connective tissue diseases associated ILD (DCTD-ILD). There were differences in arthralgia, sicca symptoms, mechanic’s hand, positive antinuclear antibodies, anti-CCP antibodies and residual lung volume between the IPAF patients and the DCTD-ILD patients (all P < 0. 05). Five IPAF patients were revealed hug or “pancake” the diaphragm in their chest high resolution CT radiographs. The microscopic performance showed that diffuse thickened with collagen fiber, alveolar wall thickening with marked interstitial lymphocyte inflammatory cells infiltration, and granulation tissue that filled bronchiolar lumina. The patients were pathologically diagnosed with nonspecific interstitial pneumonia (NSIP) overlap organized pneumonia (OP). During following-up, the progression-free survival time of the IPAF patients was significant longer and that of the DCTD-ILD patients [(14.32±5.74)months vs. (10. 31± 3. 70) months, P < 0. 05]. Conclusions If an ILD patient has mechanic’s hand, positive antinuclear antibodies or NISP overlap OP in image, the diagnosis of IPAF should be considered. IPAF have slower disease progression and better prognosis than DCTD-ILD.
Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myopathy characterized by relatively severe proximal weakness with high serum muscle enzyme levels, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy, and infrequent extra-muscular involvement. The mechanism of necrotizing myopathy remains unclear. The new European Neuromuscular Centre criteria divides IMNM into three distinct subtypes according to different autoantibodies, which reminds us antibodies may be involved in the pathogenesis of IMNM and different subtypes may have different pathogenesis. This review summarizes the current understanding of the pathogenesis of IMNM.