Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100005, China.
World J Gastroenterol. 2013 Aug 21;19(31):5131-7. doi: 10.3748/wjg.v19.i31.5131.
To establish the frequency and clinical features of connective tissue diseases (CTDs) in a cohort of Chinese patients with primary biliary cirrhosis (PBC).
Three-hundred and twenty-two Chinese PBC patients were screened for the presence of CTD, and the systemic involvement was assessed. The differences in clinical features and laboratory findings between PBC patients with and without CTD were documented. The diversity of incidence of CTDs in PBC of different countries and areas was discussed. For the comparison of normally distributed data, Student's t test was used, while non-parametric test (Wilcoxon test) for the non-normally distributed data and 2 × 2 χ(2) or Fisher's exact tests for the ratio.
One-hundred and fifty (46.6%) PBC patients had one or more CTDs. The most common CTD was Sjögren's syndrome (SS, 121 cases, 36.2%). There were nine cases of systemic sclerosis (SSc, 2.8%), 12 of systemic lupus erythematosus (SLE, 3.7%), nine of rheumatoid arthritis (RA, 2.8%), and 10 of polymyositis (PM, 3.1%) in this cohort. Compared to patients with PBC only, the PBC + SS patients were more likely to have fever and elevated erythrocyte sedimentation rate (ESR), higher serum immunoglobulin G (IgG) levels and more frequent rheumatoid factor (RF) and interstitial lung disease (ILD) incidences; PBC + SSc patients had higher frequency of ILD; PBC + SLE patients had lower white blood cell (WBC) count, hemoglobin (Hb), platelet count, γ-glutamyl transpeptidase and immunoglobulin M levels, but higher frequency of renal involvement; PBC + RA patients had lower Hb, higher serum IgG, alkaline phosphatase, faster ESR and a higher ratio of RF positivity; PBC + PM patients had higher WBC count and a tendency towards myocardial involvement.
Besides the common liver manifestation of PBC, systemic involvement and overlaps with other CTDs are not infrequent in Chinese patients. When overlapping with other CTDs, PBC patients manifested some special clinical and laboratory features which may have effect on the prognosis.
在一组中国原发性胆汁性肝硬化（PBC）患者中，确定结缔组织病（CTD）的频率和临床特征。
筛选 322 名中国 PBC 患者，以确定是否存在 CTD，并评估其全身受累情况。记录 PBC 患者有或无 CTD 之间的临床特征和实验室检查结果的差异。讨论了不同国家和地区 PBC 中 CTD 发病率的差异。对于正态分布数据，使用 Student's t 检验，对于非正态分布数据使用非参数检验（Wilcoxon 检验），对于比例使用 2×2 χ(2)或 Fisher 确切检验。
150 例（46.6%）PBC 患者存在一种或多种 CTD。最常见的 CTD 是干燥综合征（SS，121 例，36.2%）。本队列中有 9 例系统性硬化症（SSc，2.8%）、12 例系统性红斑狼疮（SLE，3.7%）、9 例类风湿关节炎（RA，2.8%）和 10 例多发性肌炎（PM，3.1%）。与单纯 PBC 患者相比，PBC+SS 患者更易出现发热和红细胞沉降率（ESR）升高，血清免疫球蛋白 G（IgG）水平更高，类风湿因子（RF）和间质性肺病（ILD）的发生率更高；PBC+SSc 患者ILD 发生率更高；PBC+SLE 患者白细胞（WBC）计数、血红蛋白（Hb）、血小板计数、γ-谷氨酰转肽酶和免疫球蛋白 M 水平较低，但肾脏受累发生率较高；PBC+RA 患者 Hb 较低，血清 IgG、碱性磷酸酶、ESR 较快，RF 阳性率较高；PBC+PM 患者 WBC 计数较高，有心肌受累倾向。
除了 PBC 常见的肝脏表现外，中国患者的系统性受累和与其他 CTD 的重叠并不少见。当与其他 CTD 重叠时，PBC 患者表现出一些特殊的临床和实验室特征，可能会影响预后。
