Angiomyolipoma ; pathology ; Humans ; Kidney Neoplasms ; pathology ; Lung Neoplasms ; pathology ; Lymphangioleiomyomatosis ; pathology ; Neoplasms, Multiple Primary ; pathology

Objective To explore the clinical manifestations, imaging and pathology features, treatment, and prognosis of endocrine glands involved patients with IgG4-related disease ( IgG4-RD) . Methods Ten patients admitted in Peking Union Medical College Hospital from 1 January 2014 to 30 June 2018 diagnosed as IgG4-RD with endocrine glands involved were enrolled in this study. All the clinical data were collected and analyzed. Results Ten patients, 4 males and 6 females, median 55 years old at the onset were enrolled, five patients with single organ involvement ( 1 case involved in pituitary, and 4 cases involved in thyroid) , while another 5 patients with 2 and more organs involved. C-reactive protein or erythrocyte sedimentation rate was elevated in 8 patients, antinuclear antibodies were positive in 7, and serum total IgE was elevated in 6 patients. Nine patients were treated with oral glucocorticoids, among whom 3 patients were treated in combination with immunosuppressive agents or rituximab. After treatment, the clinical symptoms were alleviated and imaging was improved in all patients. Serum IgG levels were significantly decreased in all patients and normalized in 5 patients. Conclusion For the patients with multiple endocrine gland diseases and elevated IgG4 level, the possibility of IgG4-RD should be carefully considered and should be confirmed by tissue biopsy and histopathology.

ObjectiveTo observe the therapeutic effect of Shugan Huazheng prescription on hepatic fibrosis model rats induced by carbon tetrachloride （CCl₄） and explore whether it plays its role through hypoxia-induced factor-1α/vascular endothelial growth factor/transforming growth factor-β₁ （HIF-1α/VEGF/TGF-β₁） pathway. MethodA total of 54 male SPF SD rats were randomly divided into six groups： blank group， model group， colchicine group （0.2 mg·kg^-1）， and high-， medium-， and low-dose groups （29.52， 14.76， and 7.38 g·kg^-1） of Shugan Huazheng prescription， with nine rats in each group. The molding was conducted three times a week for eight weeks. Administration began the day after the first injection， and the drug intervention was once a day for eight weeks. On the day after the last administration， the rats were deprived of food and water， and they were killed the next day， during which the physiological status of each group of rats was dynamically monitored. The pathological changes in the liver were observed by hematoxylin-eosin （HE） staining， and the content of hydroxyproline （HYP） and angiotensin Ⅱ （AngⅡ） in liver tissue were detected by enzyme-related immunosorbent assay （ELISA）. Real-time fluorescent quantitative PCR （Real-time PCR） was used to determine the mRNA expression levels of HIF-1α， VEGF， and TGF-β₁ in liver tissue， and immunohistochemical method （IHC） and Western blot were used to detect the protein expression levels of HIF-1α， VEGF， and TGF-β₁ in liver tissue. ResultCompared with the blank group， the overall condition of rats in the model group decreased significantly. The proliferation of connective tissue and the increase in adipose cells between hepatocytes were obvious. The content of HYP and Ang was increased. The mRNA and protein expressions of HIF-1α， VEGF， and TGF-β₁ were increased to varying degrees （P<0.05）. Compared with the model group， the proliferation of connective tissue and inflammatory cell infiltration in the liver tissue of colchicine and Shugan Huazheng prescription groups were reduced. The content of HYP and Ang was decreased. The mRNA and protein expression levels of HIF-1α， VEGF， and TGF-β₁ were decreased， and the colchicine group and high-dose group of Shugan Huazheng prescription were the most significant （P<0.05）. ConclusionShugan Huazheng prescription has an obvious therapeutic effect on CCl₄-induced hepatic fibrosis model rats. Its therapeutic mechanism may be related to the regulation of the HIF-1α/VEGF/TGF-β₁ signaling pathway and the improvement of hepatic hypoxia， vascular remodeling， and the syndrome of Qi deficiency and blood stasis in hepatic fibrosis.