West Margaret, Jasin Hugo E, Medhekar Swati
Division of Rheumatology and Clinical Immunology, Department of Internal Medicine, University of Arkansas for Medical Sciences and Central Arkansas Veterans Hospital System, Little Rock, AR 72205, USA.
Semin Arthritis Rheum. 2006 Jun;35(6):344-8. doi: 10.1016/j.semarthrit.2005.12.001.
To study the prevalence of connective tissue diseases (CTD) in patients with autoimmune hepatitis (AIH).
We identified 11 cases of AIH over the past 7 years at our institution, through a systematic chart review of patients with this diagnosis. Their charts were reviewed for the development of systemic CTD.
Three of the 11 patients with a definitive diagnosis of AIH developed systemic CTD. All were white: 2 women and 1 man, with an age range of 33 to 62 years, and with disease duration of 1 to 7 years. One patient developed systemic lupus erythematosus (SLE) with vasculitis and peripheral neuropathy. The second developed limited scleroderma and the third developed undifferentiated connective tissue disease (UCTD) and interstitial lung disease. There appear to be shared susceptibility alleles for AIH and CTD in addition to the shared positive autoantibodies.
Patients with AIH may be at increased risk for developing systemic CTD. Conversely, a review of the literature reveals that patients with systemic CTD may be at increased risk of developing AIH. Patients with either AIH or CTD should be monitored for further development of concurrent autoimmune diseases.
研究自身免疫性肝炎（AIH）患者中结缔组织病（CTD）的患病率。
通过对我院过去7年确诊为AIH的患者病历进行系统回顾，我们确定了11例AIH患者。对他们的病历进行了系统性结缔组织病发病情况的审查。
11例确诊为AIH的患者中有3例发生了系统性结缔组织病。所有患者均为白人：2名女性和1名男性，年龄在33至62岁之间，病程为1至7年。1例患者发生了伴有血管炎和周围神经病变的系统性红斑狼疮（SLE）。第2例发生了局限性硬皮病，第3例发生了未分化结缔组织病（UCTD）和间质性肺病。除了共同的阳性自身抗体外，AIH和CTD似乎还有共同的易感等位基因。
AIH患者发生系统性结缔组织病的风险可能增加。相反，文献回顾显示，系统性结缔组织病患者发生AIH的风险可能增加。AIH或CTD患者均应监测是否并发其他自身免疫性疾病。
