Santos Gonçalo A, Brandão Mariana, Farinha Fátima
Department of Internal Medicine, Hospital de Braga, Braga, PRT.
Clinical Immunology Unit, Centro Hospitalar Universitário do Porto, Porto, PRT.
Cureus. 2022 Apr 29;14(4):e24590. doi: 10.7759/cureus.24590. eCollection 2022 Apr.
Objective To analyze the prevalence and clinical progression of primary biliary cholangitis (PBC) in patients with primary Sjögren's syndrome (pSS) and possible associations between biochemical and immunological features and the development of PBC. Methods We retrospectively reviewed a cohort of 115 pSS patients followed up in an outpatient clinic from 1987 to 2020, without a history of liver disease, and looked for the presence of PBC through analysis of several biochemical, immunological, and histologic characteristics. Results Twenty patients (17.4%) had chronic cholestatic liver biochemistry. After exclusion of extrahepatic liver disease by abdominal ultrasound, 13 of them were tested for antimitochondrial autoantibodies (AMA) detected by indirect immunofluorescence (IF), of which five tested positive, fulfilling the diagnostic criteria for PBC. Three of the five PBC patients and three of the eight chronic cholestasis AMA-negative patients were further investigated with liver biopsy, which showed features of PBC in all three PBC patients and in one of the chronic cholestasis AMA-negative patients, allowing for the diagnosis of AMA-negative PBC in the latter. The remaining two AMA-negative patients had liver histology compatible with autoimmune hepatitis and unspecific findings, respectively. Overall, six (5.2%) patients with pSS had AMA-positive PBC (n=5) or AMA-negative PBC (n=1). Comparing immunological characteristics between PBC and non-PBC patients, we found that PBC patients had a higher mean maximum erythrocyte sedimentation rate (ESR) during follow-up than patients without PBC. All PBC patients were treated with ursodeoxycholic acid (UDCA) and after treatment with UDCA, only one patient showed biochemical and clinical progression of PBC, with increasing alkaline phosphatase and total bilirubin levels, eventually progressing to cirrhosis. Conclusions Among patients with pSS, PBC had an overall prevalence of six of 115 (5.2%). Higher ESR was a feature associated with PBC patients. In our cohort, after initiation of UDCA treatment, PBC showed predominantly slow progress, with only one patient progressing to cirrhosis during follow-up.
目的 分析原发性干燥综合征（pSS）患者中原发性胆汁性胆管炎（PBC）的患病率及临床进展情况，以及生化和免疫特征与PBC发生之间的可能关联。方法 我们回顾性分析了1987年至2020年在门诊随访的115例无肝脏疾病史的pSS患者队列，通过分析多项生化、免疫和组织学特征来查找PBC的存在情况。结果 20例患者（17.4%）存在慢性胆汁淤积性肝生化指标异常。经腹部超声排除肝外肝脏疾病后，其中13例检测间接免疫荧光（IF）法检测的抗线粒体自身抗体（AMA），5例检测结果为阳性，符合PBC诊断标准。5例PBC患者中的3例以及8例慢性胆汁淤积AMA阴性患者中的3例进一步接受了肝活检，所有3例PBC患者以及1例慢性胆汁淤积AMA阴性患者的肝活检显示出PBC特征，后者被诊断为AMA阴性PBC。其余2例AMA阴性患者的肝脏组织学分别与自身免疫性肝炎相符和为非特异性表现。总体而言，6例（5.2%）pSS患者患有AMA阳性PBC（n = 5）或AMA阴性PBC（n = 1）。比较PBC患者与非PBC患者的免疫特征，我们发现PBC患者在随访期间的平均最高红细胞沉降率（ESR）高于无PBC的患者。所有PBC患者均接受了熊去氧胆酸（UDCA）治疗，UDCA治疗后，仅1例患者出现PBC的生化和临床进展，碱性磷酸酶和总胆红素水平升高，最终进展为肝硬化。结论 在pSS患者中PBC的总体患病率为115例中的6例（5.2%）。较高的ESR是PBC患者的一个特征。在我们的队列中，开始UDCA治疗后，PBC主要表现为进展缓慢，随访期间仅1例患者进展为肝硬化。
