Vaiphei Kim, Bhatia Alka, Sinha Saroj Kant
Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Indian J Pathol Microbiol. 2011 Jan-Mar;54(1):25-31. doi: 10.4103/0377-4929.77319.
Collagen vascular disorders (CVDs) are autoimmune disorders with multisystem involvement. Clinical liver involvement is not a characteristic feature though histological involvement could be frequent. Liver disease in CVDs could be the consequence of various factors.
The aim was to analyze the histological spectrum of liver in collagen vascular disorders (CVDs) at autopsy.
Thirty-six autopsy livers negative for hepatitis B or C virus were studied in CVD cases with no known association with chronic liver disease or vascular thrombosis or hematological disorder. Cirrhotic and normal livers were used as controls. The paired t-test, one-way ANOVA, and two-sided Dunnett t-test were used for comparison (< 0.05). None of the control cases showed any abnormal vessels.
There were 21 systemic lupus erythematosus (SLE), 7 rheumatoid arthritis (RA), 5 systemic sclerosis (SSc), and 3 polyarteritis nodosa (PAN) cases (M:F = 11:25, age range 23-60 years).
Diffuse nodular regenerative hyperplasia of liver (NRHL) was seen in 10 cases, and 6 (5 SLE and 1 RA) had numerous abnormal thin-walled vessels in intermediate- and small-sized portal tracts with no vascular occlusion or inflammation. Moderate sized portal tracts showed more interface and lobular inflammation. The main portal vein and its major branches were normal. None of these six cases had increased transmainases (P>0.05). Most SLE cases had increased transaminases (P<0.05). No evidence of portal hypertension was seen in all except in one RA. Septicemia is known to be associated with raised transaminases.
A rare pathology of conglomerate of abnormal vessels in intermediate- and small-sized portal system was observed co-existing with NRHL in CVDs. Raised liver enzyme with interface hepatitis in CVD may not necessarily warrant an overlap, as a similar feature could be observed in septicemia.
胶原血管病（CVDs）是累及多系统的自身免疫性疾病。临床肝脏受累并非其特征性表现，不过组织学受累可能较为常见。CVDs中的肝脏疾病可能是多种因素导致的结果。
本研究旨在分析胶原血管病（CVDs）尸检肝脏的组织学特征。
选取36例尸检肝脏，这些肝脏乙肝或丙肝病毒检测均为阴性，来自无慢性肝病、血管血栓形成或血液系统疾病相关病史的CVDs患者。以肝硬化和正常肝脏作为对照。采用配对t检验、单因素方差分析和双侧Dunnett t检验进行比较（P<0.05）。所有对照病例均未发现任何异常血管。
其中包括21例系统性红斑狼疮（SLE）、7例类风湿关节炎（RA）、5例系统性硬化症（SSc）和3例结节性多动脉炎（PAN）患者（男:女 = 11:25，年龄范围23 - 60岁）。
10例可见肝脏弥漫性结节状再生性增生（NRHL），6例（5例SLE和1例RA）在中小门静脉分支中有大量异常薄壁血管，无血管闭塞或炎症。中等大小门静脉分支处界面和小叶炎症更明显。主要门静脉及其主要分支正常。这6例患者转氨酶均未升高（P>0.05）。大多数SLE患者转氨酶升高（P<0.05）。除1例RA患者外，其余患者均未发现门静脉高压证据。已知败血症与转氨酶升高有关。
在CVDs中观察到中小门静脉系统中异常血管聚集这一罕见病理表现与NRHL共存。CVDs中出现转氨酶升高伴界面性肝炎不一定意味着存在重叠综合征，因为败血症也可能有类似表现。
