自身免疫性肝病患者的臨床特點(diǎn)分析及其血清IgG4檢測(cè)的臨床意義
發(fā)布時(shí)間：2018-08-20 13:36
【摘要】：目的:總結(jié)分析自身免疫性肝病(AILD)患者的臨床特征,為其早期診斷及治療提供數(shù)據(jù)參考。探討自身免疫性肝病患者血清Ig G4檢測(cè)對(duì)自身免疫性肝病患者的診斷、鑒別診斷、病情評(píng)估等方面的臨床意義,以期為臨床工作中關(guān)于血清Ig G4的檢測(cè)在自身免疫性肝病患者的價(jià)值提供一定數(shù)據(jù)參考。方法:回顧性分析我科近年來收治的522例自身免疫性肝病患者的一般資料、臨床表現(xiàn)、自身抗體、血清生化學(xué)、肝活檢病理等臨床指標(biāo)的差異,總結(jié)其各自的臨床特點(diǎn)。這其中包括211名自身免疫性肝炎(AIH)患者,148例原發(fā)性膽汁性肝硬化(PBC)患者,155例重疊綜合征(OS)例患者,8例原發(fā)性硬化性膽管炎(PSC)患者。繼之,從上述病例中選取在我院標(biāo)準(zhǔn)治療1年以上,且在實(shí)驗(yàn)室標(biāo)本庫中有治療前后兩次血清標(biāo)本的自身免疫性肝病患者65例,其中AIH 30例,PBC 25例,OS10例,收集這65例自身免疫性肝病患者的血清標(biāo)本,采用ELISA方法測(cè)定其治療前后血清Ig G、IgG4的值,分析比較不同類型自身免疫性肝病患者血清Ig G4、Ig G4/Ig G水平的差異,分析血清IgG4水平與各臨床指標(biāo)的相關(guān)性,比較治療前后Ig G4、Ig G4/Ig G的變化情況。結(jié)果:1.522例自身免疫性肝病患者中41~60歲年齡段人群所占比例最多,男女性別之比約為1:6。AIH患者臨床表現(xiàn)中乏力、納差、惡心癥狀較其余各組更為常見;PBC患者臨床表現(xiàn)中黃疸較為常見,PSC患者臨床表現(xiàn)中皮膚瘙癢較其余各組多見。部分患者在初診時(shí)已有肝硬化、脾大或腹水表現(xiàn),PBC患者在初診時(shí)已有肝硬化者較其余各組多見。2.血清生化指標(biāo)中反映肝細(xì)胞損傷的酶學(xué)指標(biāo)ALT、AST升高在AIH組更為明顯,而反映膽管損傷的酶學(xué)指標(biāo)ALP、GGT指標(biāo)升高在PBC及PSC組明顯,PBC組中ALB下降較其余各組明顯。免疫球蛋白IgG在AIH組升高明顯,Ig M在PBC組升高明顯,PSC組Ig G及Ig M升高無特異性。3.ANA陽性在AIH患者血清自身抗體檢測(cè)中最多見,其次依次為p ANCA、ssa、ssb;而ama-m2在pbc患者血清自身抗體陽性中最常見,部分患者僅有ama-m2陽性,而無ama陽性。上述幾種抗體陽性可以同時(shí)出現(xiàn)在os患者的血清檢測(cè)中。4.aih組患者肝組織活檢病理極少累及膽管,主要表現(xiàn)為肝組織中炎細(xì)胞浸潤、肝細(xì)胞變性壞死及界面性肝炎。pbc組患者主要表現(xiàn)為明顯淤膽及小膽管破壞和增生。psc患者組織病理主要表現(xiàn)為膽管周圍水腫、膽管周圍纖維化、匯管區(qū)膽管缺失及膽汁淤積。os組患者可兼有aih及pbc或psc患者的病理學(xué)特征。5.本地區(qū)自身免疫性肝病多合并其他自身免疫性系統(tǒng)疾病,主要包括風(fēng)濕關(guān)節(jié)炎、甲狀腺疾病、1型糖尿病、系統(tǒng)性紅斑狼瘡(sle)、干燥綜合征、潰瘍性結(jié)腸炎,而自身免疫性肝炎患者合并類風(fēng)濕關(guān)節(jié)炎最為常見,8例psc患者中2例合并潰瘍性結(jié)腸炎。6.65例自身免疫性肝病患者中有5例(7.7%)患者血清igg4水平≥1350mg/l,其中1例為pbc患者,其余4例均為aih患者,這4例aih患者中3例合并糖尿病,1例合并其他自身免疫性疾病。7.三種自身免疫性肝病患者血清igg4水平與所觀察血清指標(biāo)中alp及igg4/igg明顯相關(guān),且呈正相關(guān),而與其他血清學(xué)指標(biāo)沒有顯著相關(guān)性。8.血清igg4水平與aih、pbc、os三種自身免疫性肝病患者的不同診斷之間雖無顯著相關(guān)性,但aih組患者血清igg4及igg4/igg的中位數(shù)水平略高于pbc及os組患者。9.治療后血清酶學(xué)指標(biāo)明顯改善,而治療前后血清igg4、igg4/igg的值差異無統(tǒng)計(jì)學(xué)意義。結(jié)論:1.在我國西南地區(qū)自身免疫性肝病患者的高發(fā)人群為50歲左右的圍絕經(jīng)期女性,aih、pbc及os較為常見,psc少見。西南地區(qū)自身免疫性肝炎以i型為主。psc患者男性所占比例小于國內(nèi)其他報(bào)道。該地區(qū)相當(dāng)部分自身免疫性肝病患者在初診時(shí)病情多已進(jìn)展。2.我國西南地區(qū)自身免疫性肝炎主要合并類風(fēng)濕關(guān)節(jié)炎,而psc患者合并潰瘍性結(jié)腸炎比例小于國內(nèi)其他報(bào)道。ama陰性的pbc患者行ama-m2檢測(cè)尤為重要。多數(shù)psc患者有自身抗體panca陽性,但其無特異性血清生化學(xué)及免疫球蛋白改變,特異性影像學(xué)檢查及病理組織活檢對(duì)其診斷十分重要。綜合臨床表現(xiàn)、自身抗體、免疫球蛋白水平、血清生化學(xué)指標(biāo)、合并其他自身免疫系統(tǒng)疾病、影像學(xué)檢查、病理活檢對(duì)于自身免疫性肝病的診斷有重要意義。3.AIH患者血清Ig G4水平較PBC、OS患者稍高,合并其他自身免疫性疾病及代謝疾病的自身免疫性肝病患者血清Ig G4水平升高較為明顯。血清Ig G4水平與AIH、PBC、OS三種自身免疫性肝病患者的不同診斷之間相關(guān)性不顯著,這提示可能暫不能將Ig G4及Ig G4/Ig G作為三種自身免疫性肝病的鑒別診斷指標(biāo)。三種自身免疫性肝病患者血清Ig G4水平與所觀察血清指標(biāo)中ALP明顯相關(guān),提示Ig G4可能參與了膽管損傷。血清IgG4與自身免疫性肝病患者病情改善無明顯相關(guān)性。
[Abstract]:Objective: To summarize and analyze the clinical features of autoimmune liver disease (AILD) and provide data for its early diagnosis and treatment. Methods: Retrospective analysis of 522 patients with autoimmune liver disease treated in our department in recent years, the general data, clinical manifestations, autoantibodies, serum biochemistry, liver biopsy pathology and other clinical indicators of the differences, summed up their respective clinical characteristics. Patients with autoimmune hepatitis (AIH), 148 patients with primary biliary cirrhosis (PBC), 155 patients with overlapping syndrome (OS) and 8 patients with primary sclerosing cholangitis (PSC) were selected from the above-mentioned cases for more than one year, and the laboratory specimen bank had autoimmunity of serum samples before and after treatment for two times. Serum IgG and IgG4 were measured by ELISA in 65 patients with autoimmune liver disease, including 30 patients with AIH, 25 patients with PBC and 10 patients with OS. Results: Among the 1.522 patients with autoimmune liver disease, 41-60 years old accounted for the largest proportion, the ratio of male to female was about 1:6. The clinical manifestations of AIH patients were fatigue, poor appetite, nausea symptoms more common than the other groups; PBC patients with clinical manifestations of jaundice more common. Some of the patients had cirrhosis, splenomegaly or ascites at the time of initial diagnosis, and the patients with liver cirrhosis at the time of initial diagnosis were more common than the other groups. 2. Serum biochemical indicators of hepatocyte injury in ALT, AST increased more significantly in AIH group, but reflected bile duct injury. ALP and GGT increased significantly in PBC and PSC groups, and ALB decreased significantly in PBC group. IgG increased significantly in AIH group, Ig M increased significantly in PBC group, and IgG and Ig M increased nonspecific in PSC group. Serum autoantibodies were the most common in patients with chronic hepatitis C. only AMA-M2 was positive in some patients, but no AMA was positive. these antibodies could be detected simultaneously in serum of patients with chronic hepatitis C. 4. liver biopsy pathology in AIH group rarely involved bile ducts, mainly manifested as infiltration of inflammatory cells in liver tissue, degeneration and necrosis of hepatocytes and hepatitis interface. The main histopathological features of PSC patients were peribiliary edema, peribiliary fibrosis, absence of bile ducts in the portal area and cholestasis. Epidemic systemic diseases, including rheumatoid arthritis, thyroid disease, type 1 diabetes mellitus, systemic lupus erythematosus (sle), Sjogren's syndrome, and ulcerative colitis, were most common in autoimmune hepatitis patients with rheumatoid arthritis, 2 of 8 PSC patients with ulcerative colitis, 5 of 6.65 patients with autoimmune liver disease (7.7) Serum IgG4 levels were higher than 1 350 mg / L in 3 of the 4 AIH patients with diabetes mellitus and 1 with other autoimmune diseases. There was no significant correlation between serum IgG4 levels and the diagnosis of aih, pbc, OS three kinds of autoimmune liver disease, but the median serum IgG4 and IgG4 / IgG levels in AIH group were slightly higher than those in PBC and OS group. Conclusion: 1. In perimenopausal women aged 50 or so, aih, PBC and OS are more common and PSC is less common. In the southwest of China, autoimmune hepatitis is predominant in type I. The proportion of male patients with PSC is less than other reports in China. Most of the patients with autoimmune hepatitis in southwest China were accompanied by rheumatoid arthritis, while the proportion of PSC patients with ulcerative colitis was lower than other reports in China. Am-m2 test was especially important in patients with PBC negative for ama. Biochemical and immunoglobulin changes in heterosexual serum, specific imaging and pathological biopsy are important for the diagnosis of autoimmune liver disease. Significance. 3. The levels of serum Ig G4 in AIH patients were slightly higher than those in PBC and OS patients. The levels of serum Ig G4 in patients with autoimmune liver diseases complicated with other autoimmune diseases and metabolic diseases were significantly higher than those in patients with AIH, PBC and OS. Ig G4 and Ig G4/Ig G can be used as the differential diagnostic indexes of three kinds of autoimmune liver diseases.The levels of serum Ig G4 in patients with three kinds of autoimmune liver diseases are significantly correlated with ALP in the serum indexes observed, suggesting that Ig G4 may be involved in bile duct injury.There is no significant correlation between serum IgG4 and the improvement of the condition of patients with autoimmune liver diseases.
【學(xué)位授予單位】：第三軍醫(yī)大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2015
【分類號(hào)】：R575
本文編號(hào)：2193805
[Abstract]:Objective: To summarize and analyze the clinical features of autoimmune liver disease (AILD) and provide data for its early diagnosis and treatment. Methods: Retrospective analysis of 522 patients with autoimmune liver disease treated in our department in recent years, the general data, clinical manifestations, autoantibodies, serum biochemistry, liver biopsy pathology and other clinical indicators of the differences, summed up their respective clinical characteristics. Patients with autoimmune hepatitis (AIH), 148 patients with primary biliary cirrhosis (PBC), 155 patients with overlapping syndrome (OS) and 8 patients with primary sclerosing cholangitis (PSC) were selected from the above-mentioned cases for more than one year, and the laboratory specimen bank had autoimmunity of serum samples before and after treatment for two times. Serum IgG and IgG4 were measured by ELISA in 65 patients with autoimmune liver disease, including 30 patients with AIH, 25 patients with PBC and 10 patients with OS. Results: Among the 1.522 patients with autoimmune liver disease, 41-60 years old accounted for the largest proportion, the ratio of male to female was about 1:6. The clinical manifestations of AIH patients were fatigue, poor appetite, nausea symptoms more common than the other groups; PBC patients with clinical manifestations of jaundice more common. Some of the patients had cirrhosis, splenomegaly or ascites at the time of initial diagnosis, and the patients with liver cirrhosis at the time of initial diagnosis were more common than the other groups. 2. Serum biochemical indicators of hepatocyte injury in ALT, AST increased more significantly in AIH group, but reflected bile duct injury. ALP and GGT increased significantly in PBC and PSC groups, and ALB decreased significantly in PBC group. IgG increased significantly in AIH group, Ig M increased significantly in PBC group, and IgG and Ig M increased nonspecific in PSC group. Serum autoantibodies were the most common in patients with chronic hepatitis C. only AMA-M2 was positive in some patients, but no AMA was positive. these antibodies could be detected simultaneously in serum of patients with chronic hepatitis C. 4. liver biopsy pathology in AIH group rarely involved bile ducts, mainly manifested as infiltration of inflammatory cells in liver tissue, degeneration and necrosis of hepatocytes and hepatitis interface. The main histopathological features of PSC patients were peribiliary edema, peribiliary fibrosis, absence of bile ducts in the portal area and cholestasis. Epidemic systemic diseases, including rheumatoid arthritis, thyroid disease, type 1 diabetes mellitus, systemic lupus erythematosus (sle), Sjogren's syndrome, and ulcerative colitis, were most common in autoimmune hepatitis patients with rheumatoid arthritis, 2 of 8 PSC patients with ulcerative colitis, 5 of 6.65 patients with autoimmune liver disease (7.7) Serum IgG4 levels were higher than 1 350 mg / L in 3 of the 4 AIH patients with diabetes mellitus and 1 with other autoimmune diseases. There was no significant correlation between serum IgG4 levels and the diagnosis of aih, pbc, OS three kinds of autoimmune liver disease, but the median serum IgG4 and IgG4 / IgG levels in AIH group were slightly higher than those in PBC and OS group. Conclusion: 1. In perimenopausal women aged 50 or so, aih, PBC and OS are more common and PSC is less common. In the southwest of China, autoimmune hepatitis is predominant in type I. The proportion of male patients with PSC is less than other reports in China. Most of the patients with autoimmune hepatitis in southwest China were accompanied by rheumatoid arthritis, while the proportion of PSC patients with ulcerative colitis was lower than other reports in China. Am-m2 test was especially important in patients with PBC negative for ama. Biochemical and immunoglobulin changes in heterosexual serum, specific imaging and pathological biopsy are important for the diagnosis of autoimmune liver disease. Significance. 3. The levels of serum Ig G4 in AIH patients were slightly higher than those in PBC and OS patients. The levels of serum Ig G4 in patients with autoimmune liver diseases complicated with other autoimmune diseases and metabolic diseases were significantly higher than those in patients with AIH, PBC and OS. Ig G4 and Ig G4/Ig G can be used as the differential diagnostic indexes of three kinds of autoimmune liver diseases.The levels of serum Ig G4 in patients with three kinds of autoimmune liver diseases are significantly correlated with ALP in the serum indexes observed, suggesting that Ig G4 may be involved in bile duct injury.There is no significant correlation between serum IgG4 and the improvement of the condition of patients with autoimmune liver diseases.
【學(xué)位授予單位】：第三軍醫(yī)大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2015
【分類號(hào)】：R575
【參考文獻(xiàn)】
相關(guān)期刊論文 前2條
1 張敏;韓濤;聶彩云;王偉;杜衛(wèi)杰;周淑芬;王棕覃;孫揚(yáng);丁賢;朱爭(zhēng)艷;;自身免疫性肝病患者血清IgG4水平分析[J];實(shí)用肝臟病雜志;2014年04期
2 施健,謝渭芬;自身免疫性肝病的發(fā)病機(jī)制[J];中華肝臟病雜志;2005年01期
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