Systemic lupus erythematosus with primary sclerosing cholangitis
10.3760/cma.j.c141217-20200218-00160
- VernacularTitle:系统性红斑狼疮合并原发性硬化性胆管炎三例并文献复习
- Author:
Cui LU
;
Jialin TENG
;
Zhuochao ZHOU
;
Fan WANG
;
Naying HE
;
Chengde YANG
;
Junna YE
- From:
Chinese Journal of Rheumatology
2021;25(3):184-188,F3
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical manifestations, laboratory and imaging features, treatment and prognosis of systemic lupus erythematosus (SLE) with primary sclerosing cholangitis (PSC).Methods:This is a retrospective case series study describing the clinical, laboratory and imaging manife-stations, treatment and prognosis of 3 SLE patients with PSC. The related literatures were reviewed.Results:In total, 10 patients were included. SLE with PSC, with an average age of (43±17) years old, was more common with hematological and renal involvement, jaundice and arthralgia, positive anti-double-stranded DNA (anti-dsDNA) antibody, hypocomplementemia, elevated erythrocyte sedimentation rate (ESR) and abnormal liver function with predominately elevated alkaline phosphatase (ALP). The classic magnetic resonance cholangio-pancreatography (MRCP) features of PSC include multifocal strictures, beading and areas of dilatation involving the intra- and/or extrahepatic bile ducts. The treatment included glucocorticosteroids, immunosup-pressants and ursodeoxycholic acid (UDCA), and some patients required biliary drainage. Most patients had a good prognosis.Conclusion:Although PSC is rare, attention should be paid to SLE patients with abnormal liver function, especially with elevated ALP, in order to differentiate from PSC.
