2010-2017 tarihleri arasında otoimmün hepatit tanılı erişkin hastaların retrospektif olarak epidemiyolojik değerlendirilmesi
Date
2018
Authors
Ertem, Aytül Coşar
Journal Title
Journal ISSN
Volume Title
Publisher
Uludağ Üniversitesi
Abstract
Otoimmün hepatit (OİH), etiyolojisi bilinmeyen immün sistem aracılı karaciğer hastalığıdır. Dolaşımda otoantikorların varlığı ve yüksek serum globulin konsantrasyonlarının olduğu immünolojik özelliklerle karakterizedir. Çok ırklı toplumlarda OİH epidemiyolojisi ve klinik seyri hakkında az sayıda rapor bulunmaktadır. Retrospektif olarak gerçekleştirdiğimiz bu çalışmanın amacı 01.01.2010-30.06.2017 tarihleri arasında merkezimizde takip ettiğimiz 202 OİH'li hastanın demografik, etyolojik, klinik, laboratuvar ve radyolojik özelliklerini, histolojik bulgularını, tedavi sonrası hastalık prognozunu, hastalık komplikasyonlarını araştırmaktır. Ayrıca, tedavi kesilme süresi, tedavi kesildikten sonra nüks gelişimini ve uygulanan tedavi ile remisyon elde edilenlerin bulgularının literatür çalışmaları ile karşılaştırılması amaçlandı. Çalışmamızda hastalar; yaş, cinsiyet, serum immünglobülin G, serum protein elektroforezi, lipid profili, karaciğer ultrasonografisi, tedavi öncesi ve tedavi sonrası karaciğer fonksiyon testleri, eşlik eden varyant sendrom varlığı ve karaciğer biyopsilerinin detaylı analizi (lenfosit infiltrasyonu, interface hepatit, rozet formasyonu, eşlik eden kronik hepatit, siroz ve nonalkolik steatohepatit varlığı), hastalığı provoke eden ilaç kullanım öyküsü, karaciğere spesifik otoantikorların mevcudiyeti ve titreleri, tedavi ajanları (prednizolon, azatiopürin, ursodeoksikolik asit), tedavi süresi ve remisyon süresi, nüks varlığı, nüks ve takip süreleri açısından değerlendirildi. 202 hastanın tümüne, Uluslararası Otoimmün Hepatit Grubu kriterlerine göre teşhis kondu. Median tanı yaşı 46 ve hastaların çoğunluğu kadındı (%86). Tanı anı median karaciğer enzimleri; AST 100 İU/L, ALT 127 İU/L, GGT 146 İU/L, ALP 178 İU/L olarak bulundu. Hastaların otoantikorlarının dağılımı detaylı analiz edildi ve en yaygın otoantikor antinükleer antikor (ANA) bulundu (%86). Karaciğer biyopsi yapılma oranı (%80.1) ve biyopsi yapılanların detaylı biyopsi analizi sorgulandı; 145'inde (%71.8) lenfosit infiltrasyonu, 94'ünde (%46.5) arayüz hepatiti ve 19'unda rozet formasyonu (%9.4) bulundu. 31 hastada (%15.3) tanı öncesi hastalığı provoke ettiği düşünülen ilaç kullanım öyküsü mevcuttu. Çalışmamızda 76 hastada (%37.6), OİH'e eşlik eden başka bir otoimmün hastalık vardı ve 109 hastada (%53.9) biyopsi materyalinde tanımlanmış olan varyant sendrom saptandı. 186 hastada (%92.1) tedavi ile remisyona ulaşıldı. Remisyona girme median süresi 4 ay bulundu. Tedaviye rağmen remisyona ulaşılamayan 16 hastada (%7.9) teşhis anında siroz saptandı. 64 hastada (%34.4) hastalık nüks etti. Nüks süresinin medianı hastalık başlangıcından itibaren 48 ay olarak bulundu. Median takip süresi 60 ay olarak hesaplandı. Çalışmamızın epidemiyolojik sonuçları daha önce yayınlanan çalışmalarla karşılaştırıldı ve literatür verileri ile benzer veriler olduğu görüldü.
Autoimmune hepatitis (AIH) is an immunemediated liver disease of unknown etiology. It is characterized by immunologic features, generally including the presence of circulating autoantibodies and high serum globulin concentrations. There has been a paucity of reports on the epidemiology and clinical course of AIH in multiethnic populations. The purpose of this retrospective study is to investigate the demographic, etiological, clinical, laboratory and radiological characteristics, histological findings, prognosis after treatment, disease complications of 202 patients with AIH treated in our center between 01.01.2010-30.06.2017. In addition, it was aimed to compare the duration of treatment discontinuation, relapse development after discontinuation of treatment, and the findings obtained in the remission with the applied therapy with the literature studies. Patients were evaluated in terms of age, sex, serum immunoglobulin G, serum protein electrophoresis, lipid profile, liver ultrasonography, liver function tests before treatment initiation and after treatment initiation, presence of accompanying variant syndrome and detailed analysis of liver biopsy (lymphocyte infiltration, interface hepatitis, rosette formation, concomitant chronic hepatitis, cirrhosis or NASH presence), presence of provoking drug use narrative at diagnosis, presence and titers of liver specific antibodies, treatment agents (prednisolone, azathiopurine, ursodeoxycholic acid), duration of therapy and remission, presence of recurrence and recurrence time, duration of recurrence and follow-up. All 202 patients were diagnosed according to International Autoimmune Hepatitis Group criteria. The median age of diagnosis was 46 years and the majority of the patients were female (86%). Median values of liver enzymes at diagnosis; AST 100 İU/L, ALT 127 İU/L, GGT 146 İU/L, ALP 178 İU/L. The autoantibody distribution in the patients was analyzed in detail and the most common autoantibody (86%) was found to be antinüclear anticor (ANA). The rate of liver biopsy and the detailed biopsy analysis of the constructs were questioned; lymphocyte infiltration in 145 (71.8%), interface hepatitis in 94 (46.5%) and rosette formation was found in 19 (9.4%). 31 (15.3%) patients who were diagnosed with AIH had a drug use history that was thought to provoke the patient before diagnosis. In our study, we found 76 (37.6%) patients with another autoimmune disease accompanying AIH and 109 patients (53.9%) had the variant syndrome identified in the biopsy material. The remission was achieved with treatment in 186 patients (92.1%). Entering the median was 4 months. Despite treatment, liver cirrhosis was detected at the time of diagnosis in 16 patients (7.9%) who could not be remissioned. The number of relapsed patients was 64 (34.4%). The median recurrence time was found to be 48 months from the onset of the disease. The duration of median follow-up was calculated as 60 months by the patients who were diagnosed with AIH. The epidemiologic results of our study were compared with the previously published studies and it was seen that the data were similar to the literature data.
Autoimmune hepatitis (AIH) is an immunemediated liver disease of unknown etiology. It is characterized by immunologic features, generally including the presence of circulating autoantibodies and high serum globulin concentrations. There has been a paucity of reports on the epidemiology and clinical course of AIH in multiethnic populations. The purpose of this retrospective study is to investigate the demographic, etiological, clinical, laboratory and radiological characteristics, histological findings, prognosis after treatment, disease complications of 202 patients with AIH treated in our center between 01.01.2010-30.06.2017. In addition, it was aimed to compare the duration of treatment discontinuation, relapse development after discontinuation of treatment, and the findings obtained in the remission with the applied therapy with the literature studies. Patients were evaluated in terms of age, sex, serum immunoglobulin G, serum protein electrophoresis, lipid profile, liver ultrasonography, liver function tests before treatment initiation and after treatment initiation, presence of accompanying variant syndrome and detailed analysis of liver biopsy (lymphocyte infiltration, interface hepatitis, rosette formation, concomitant chronic hepatitis, cirrhosis or NASH presence), presence of provoking drug use narrative at diagnosis, presence and titers of liver specific antibodies, treatment agents (prednisolone, azathiopurine, ursodeoxycholic acid), duration of therapy and remission, presence of recurrence and recurrence time, duration of recurrence and follow-up. All 202 patients were diagnosed according to International Autoimmune Hepatitis Group criteria. The median age of diagnosis was 46 years and the majority of the patients were female (86%). Median values of liver enzymes at diagnosis; AST 100 İU/L, ALT 127 İU/L, GGT 146 İU/L, ALP 178 İU/L. The autoantibody distribution in the patients was analyzed in detail and the most common autoantibody (86%) was found to be antinüclear anticor (ANA). The rate of liver biopsy and the detailed biopsy analysis of the constructs were questioned; lymphocyte infiltration in 145 (71.8%), interface hepatitis in 94 (46.5%) and rosette formation was found in 19 (9.4%). 31 (15.3%) patients who were diagnosed with AIH had a drug use history that was thought to provoke the patient before diagnosis. In our study, we found 76 (37.6%) patients with another autoimmune disease accompanying AIH and 109 patients (53.9%) had the variant syndrome identified in the biopsy material. The remission was achieved with treatment in 186 patients (92.1%). Entering the median was 4 months. Despite treatment, liver cirrhosis was detected at the time of diagnosis in 16 patients (7.9%) who could not be remissioned. The number of relapsed patients was 64 (34.4%). The median recurrence time was found to be 48 months from the onset of the disease. The duration of median follow-up was calculated as 60 months by the patients who were diagnosed with AIH. The epidemiologic results of our study were compared with the previously published studies and it was seen that the data were similar to the literature data.
Description
Keywords
Otoimmün hepatit, Epidemiyoloji, OİH tedavisi, Autoimmune hepatitis, Epidemiology, AIH treatment
Citation
Ertem, A. C. (2018). 2010-2017 tarihleri arasında otoimmün hepatit tanılı erişkin hastaların retrospektif olarak epidemiyolojik değerlendirilmesi. Yayınlanmamış uzmanlık tezi. Uludağ Üniversitesi Tıp Fakültesi.