Hatzis Gregorios S, Fragoulis Georgios E, Karatzaferis Aggelos, Delladetsima Ioanna, Barbatis Calipso, Moutsopoulos Haralampos M
Department of Pathophysiology, National University of Athens Medical School, and Department of Pathology, Red Cross Hospital, Athens, Greece.
J Rheumatol. 2008 Oct;35(10):2012-6. Epub 2008 Aug 15.
To study the prevalence of primary biliary cirrhosis (PBC) and its progression in patients with primary Sjögren's syndrome (SS).
We investigated 410 patients with primary SS, without history of liver disease, for the presence of PBC based on a retrospective review of clinical, biochemical, immunologic, and histologic data.
Thirty-six (8.8%) patients had cholestatic liver biochemistry. Of them, 21 (5.1%) had positive antimitochondrial autoantibodies (AMA) detected by indirect immunofluorescence, while 15 were AMA-negative. Ten of the 21 AMA-positive patients and 7 of the 15 AMA-negative patients were further investigated with liver biopsy, the result of which was compatible with PBC in all but one (AMA-negative) patient. Overall, 27 (6.6%) patients had definite (n=10), probable (n=11), or AMA-negative (n=6) PBC. Pathologically, most PBC lesions were stage 1. Five patients had a second liver biopsy, with no significant histological deterioration.
PBC is a rather uncommon development in patients with primary SS. The disease appears to be pathologically mild, with a propensity for slow progression, as assessed clinically, biochemically, and histologically.
研究原发性干燥综合征（SS）患者中原发性胆汁性肝硬化（PBC）的患病率及其病情进展。
我们对410例无肝脏疾病史的原发性SS患者进行调查，通过回顾性分析临床、生化、免疫和组织学数据来确定是否存在PBC。
36例（8.8%）患者出现胆汁淤积性肝脏生化指标异常。其中，21例（5.1%）通过间接免疫荧光检测到抗线粒体自身抗体（AMA）阳性，15例AMA阴性。21例AMA阳性患者中的10例和15例AMA阴性患者中的7例进一步接受了肝活检，除1例（AMA阴性）患者外，其余患者的活检结果均符合PBC。总体而言，27例（6.6%）患者患有明确（n = 10）、可能（n = 11）或AMA阴性（n = 6）的PBC。病理上，大多数PBC病变处于1期。5例患者进行了第二次肝活检，组织学上无明显恶化。
PBC在原发性SS患者中是一种相当罕见的并发症。从临床、生化和组织学评估来看，该病在病理上似乎较轻，倾向于缓慢进展。
