本文選題：原發(fā)性膽汁性肝硬化 + 干燥綜合征　； 參考：《山西醫(yī)科大學(xué)》2017年碩士論文

【摘要】：目的:比較原發(fā)性膽汁性肝硬化(primary biliary cirrhosis,PBC)合并干燥綜合征(Sj?gren syndrome,SS)和單純PBC、原發(fā)性干燥綜合征(Primary Sj?gren syndrome,pSS)之間的異同,進(jìn)一步分析PBC和SS之間的關(guān)系,提高對疾病的認(rèn)識。方法:收集山西醫(yī)科大學(xué)第二醫(yī)院2010年11月1日~2016年6月1日確診為PBC和pSS的病例,分別比較PBC合并SS與單純PBC、pSS患者在臨床特征、治療反應(yīng)及疾病進(jìn)展方面的異同,分析PBC和SS之間的關(guān)系。結(jié)果:1.PBC合并SS患者確診時(shí)基線資料中肝損害相關(guān)癥狀及生化指標(biāo)均較單純PBC患者輕;2.初診診斷為PBC而后期合并SS的患者,初診診斷時(shí)肝硬化及肝功能失代償發(fā)生率較單純PBC患者低,而口干癥狀較其明顯;3.抗SSB抗體陽性率及發(fā)熱癥狀在pSS患者較合并PBC患者多見;4.初診診斷為SS而后期合并PBC的患者與pSS患者基線資料中干燥綜合征相關(guān)癥狀及化驗(yàn)檢查客觀指標(biāo)無差異;5.應(yīng)用熊去氧膽酸+甲潑尼龍+嗎替麥考酚酯治療的PBC合并SS患者,其ALT、AST、ALP、GGT及ESR水平均較治療前下降,但TBIL水平治療前后變化不明顯,且ALT、AST、ALP總體平均水平均在治療3個(gè)月內(nèi)達(dá)到正常范圍;6.PBC合并SS患者應(yīng)用熊去氧膽酸+甲潑尼龍+嗎替麥考酚酯治療1年,生化未緩解組較生化緩解組TBIL、ALP、GGT基線水平高;7.PBC合并SS患者和單純PBC患者肝硬化及肝功能失代償進(jìn)展方面無差別。結(jié)論:1.PBC合并SS患者同時(shí)具有PBC和pSS的臨床特點(diǎn),但其確診時(shí)基線資料中肝損害相關(guān)表現(xiàn)比單純PBC患者輕,干燥綜合征相關(guān)癥狀及免疫指標(biāo)與pSS患者相似;2.伴口干癥狀的PBC患者易合并SS,需及早行干燥綜合征相關(guān)檢查進(jìn)行篩查;3.應(yīng)用熊去氧膽酸+甲潑尼龍+嗎替麥考酚酯治療PBC合并SS,可有效改善ALT、AST、ALP、GGT及ESR水平,但對TBIL改善不明顯;基線ALP、GGT、TBIL水平可能與此治療方案的生化緩解有關(guān);4.PBC合并SS未加速PBC的疾病進(jìn)展,甚至可能因兩種自身免疫性疾病同時(shí)存在影響了PBC的疾病特點(diǎn)及預(yù)后,使肝損害較單純PBC時(shí)輕。
[Abstract]:Objective: to compare the differences and similarities between primary biliary cirrhosis (primary biliary cirrhosis) and primary Sjgren syndrome with Sjgren syndrome (SS) and primary Sjgren syndrome (PSS), and to analyze the relationship between PBC and SS in order to improve the understanding of the disease. Methods: the cases of PBC and PSS diagnosed in the second Hospital of Shanxi Medical University from November 1, 2010 to June 1, 2016 were collected. The relationship between PBC and SS was analyzed. Results 1. The liver damage related symptoms and biochemical indexes in baseline data of PBC patients with SS were less than those of PBC patients. The incidence of liver cirrhosis and decompensation of liver function in the patients with PBC and SS in the first diagnosis was lower than that in the patients with PBC alone, but the symptoms of xerostomia were significantly lower than that of the patients with SS. The positive rate of anti SSB antibody and symptoms of fever were more common in PSS patients than in PBC patients. There was no significant difference in Sjogren's syndrome related symptoms and objective indexes of laboratory examination between the first diagnosis of SS and PBC in the baseline data of PSS patients. The levels of GGT and ESR in patients with PBC combined with SS treated with methylprednisolone ursodeoxycholate were lower than those before and after treatment, but the level of TBIL did not change significantly before and after treatment. The average level of ALP was within normal range within 3 months. Patients with PBC and SS were treated with methylprednisolone ursodeoxycholate for 1 year. The baseline level of TBILL ALP GGT in the non-remission group was higher than that in the biochemical remission group 7.There was no difference in liver cirrhosis and liver function decompensation between PBC patients with SS and PBC patients with simple PBC. Conclusion: 1. PBC patients with SS have the clinical characteristics of PBC and PSS simultaneously, but the liver damage related manifestations in baseline data of PBC patients are lighter than those in PBC patients alone. The symptoms and immune indexes of Sjogren's syndrome are similar to those of PSS patients. PBC patients with dry mouth symptoms are easy to be associated with SSS, and should be screened for Sjogren's syndrome (Sjogren syndrome) as early as possible. The application of methylprednisolone ursodeoxycholate in the treatment of PBC with SScan can effectively improve the levels of GGT and ESR in ALTAST, but the improvement of TBIL is not obvious. The level of TBIL may be related to the biochemical remission of the treatment regimen. 4. PBC combined with SS does not accelerate the progression of PBC, and may even affect the disease characteristics and prognosis of PBC because of the presence of two autoimmune diseases, which makes liver damage less severe than that of PBC alone.
【學(xué)位授予單位】：山西醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2017
【分類號】：R575.22;R593.2

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本文編號：2113193