Clinical features of primary biliary cirrhosis associated with systemic sclerosis
10.3760/cma.j.issn.1007-7480.2010.11.006
- VernacularTitle:系统性硬化病合并原发性胆汁性肝硬化的临床分析及文献复习
- Author:
Runrong LIU
;
Mengtao LI
;
Yunyun FEI
;
Dong XU
;
Yong HOU
;
Qian WANG
;
Zhaojun HU
;
Ning SONG
;
Xiaofeng ZENG
;
Fengchun ZHANG
- Publication Type:Journal Article
- Keywords:
Scleroderma,systemic;
Cirrhosis;
Autoantibody
- From:
Chinese Journal of Rheumatology
2010;14(11):746-749
- CountryChina
- Language:Chinese
-
Abstract:
Objective To assess the clinical features of primary biliary cirrhosis(PBC)associated with systemic sclerosis(SSc)in order to facilitate recognition of this overlap syndrome(SSc-PBC). Methods The clinical data of 9 patients with SSc-PBC in Peking Union Medical College Hospital were retrospectively studied with literature review. Results ① Nine patients including 8 female were at a mean age of(54±8)years. Sevene patients initially presented with SSc and developed PBC over(4.3±2.3)years. ② Eight patients were diagnosed limited cutaneous SSc(leSSc), which included 7 patients with CREST syndrome. The most frequent manifestations were Raynaud's phenomenon(8/9)and esophageal dysfunction(8/9). Four patients with PBC manifested subclinically, but laboratory results revealed increased ALP/GGT in most patients(8/9).Two patients received liver biopsies, which confirmed the diagnosis of PBC. ③ Antinuclear antibodies(ANA)and anti-mitochondrial antibody(AMA)were detected in all the patients, among them, 8 with positive anticentromere antibody(ACA)and 8 with positive AMA-M2. ④ UDCA and glucocorticosteroid might help early stage patients with SSc-PBC, but lack efficacy in patients complicated with interstitial lung disease, pulmonary hypertension or cirrhosis. Conclusion PBC might be overlapped with SSc, especially lcSSc(CREST syndrome). Screening of autoantibodies, such as ACA, AMA and AMA-M2, could help rheumatologists early recognition of SSc-PBC and improve the prognosis of this overlap syndrome by early intervention.
