Serological markers of rheumatoid arthritis in patients with primary biliary cholangitis and the vice versa: A Tunisian study

BackgroundPrimary biliary cholangitis (PBC) is an autoimmune disease of the liver characterized by destructive lymphocytic cholangitis and anti-mitochondrial antibodies (AMA). Anti-gp210 and anti-Sp100, are used for the diagnosis of PBC in AMA-negative PBC patients. Patients with PBC have a propensity to have an extrahepatic manifestation which is especially autoimmune.ObjectiveWe aimed to determine the frequency of serological markers of rheumatoid arthritis (RA) (CCP-Ab or RF) in PBC patients and to do the vice versa.MethodsOur PBC study included 70 patients with PBC and 80 healthy blood donors (HBD) and our RA study included 75 patients with RA and 75 HBD. Anti-cyclic citrullinated peptide antibodies (CCP-Ab) and rheumatoid factor (RF) were performed by indirect ELISA. AMA, anti-Sp100 and anti-gp210 were determined by indirect immunofluorescence.ResultsRA autoantibodies (CCP-Ab or RF) were more frequent in PBC patients than in HBD (65.7% vs. 8.7% p 〈1 0 ⁻⁶). CCP-Ab were significantly more frequent in patients than in controls (15.7% vs. 2.5%; p = 0.004). Nine patients had both CCP-Ab and RF vs. none of controls (12.8% vs. 0%; p = 0.001). RF were detected in 45 patients with PBC and in 5 HBD (64.3% vs. 6.2%; p 〈1 0 ⁻⁶). In PBC patients, RF were more frequent than CCP-Ab (64.3% vs. 15.7%; p 〈1 0 ⁻⁶). RF-IgG were present in 18.5% of patients; RF-immunoglobulin (Ig) A in 34.3% and RF-IgM in 54.3%. These frequencies were significantly higher than those found in control group (1.2% for RF-IgG (p 〈1 0 ⁻³); 0% for RF-IgA (p 〈1 0 ⁻⁶); and 6.2% for RF-IgM (p 〈1 0 ⁻⁶)). In our PBC patients, RF-IgA were more frequent than RF-IgG (34.3% vs. 18.5%; p = 0.03) and than CCP-Ab (34.3% vs. 15.7%; p = 0.01). Six patients had only RF-IgA versus none of the control group (8.6% vs. 0%; p = 0.01). AMA, anti-Sp100 and anti-gp 210 were absent in all RA patients.ConclusionsSerological markers of RA were more frequent in PBC patients than in HBD and the vice versa was not true.     

Definition and pathology of primary sclerosing cholangitis

Although primary sclerosing cholangitis (PSC) is not a common disease, it is important in the differential diagnosis of hepatobiliary tract diseases in clinical practice. A diagnosis of PSC should be made only after the exclusion of similar diseases with well known etiologies or pathogeneses. In this review, the pathology of classical PSC and its variants or related diseases is highlighted. PSC is histologically characterized by progressive periductal fibrosis with luminal stenosis or obliteration, along with the formation of a fibrous core, as well as dilatation (cholangiectasis). Its etiology is unknown. Bacterial ascending cholangitis is superimposed on its long clinical course. Such a heterogeneous distribution of biliary lesions with biliary obliteration and cholangiectasis is responsible for the radiological demonstration of biliary abnormalities, particularly the beaded appearance. Sampling variability is common in needle or wedge biopsied specimens. As a result of biliary damage, the liver shows progressive cholestatic change followed by biliary fibrosis and cirrhosis, and this hepatic progression is divisible into four stages. There are several variants of PSC or related diseases, such as localized biliary sclerosis and stenosis, sclerosing cholangitis associated with inflammatory pseudotumor, and PSC-autoimmune hepatitis overlapping syndrome. Cholelithiasis, including secondary hepatolithiasis and, to a lesser degree, biliary carcinoma and dysplasia, are also known to develop at the perihilar bile ducts as a late complication of PSC. Received for publication on March 8, 1999; accepted on April 30, 1999     

Medical treatment of primary sclerosing cholangitis

Until 1970, primary sclerosing cholangitis (PSC) was considered to be a medical curiosity. With the development of endoscopic cholangiography, PSC is now recognized more frequently and is a common indication for liver transplantation. PSC is usually progressive, leading to cirrhosis, portal hypertension, and liver failure. The manifestations of disease may be clinically similar to those of other causes of bile duct obstruction and must be distinguished from gallstone disease, bile duct carcinoma, primary biliary cirrhosis, and secondary biliary cirrhosis due to bile duct stricture. Medical management of PSC must take into account the likelihood that destroyed bile ducts do not regenerate as hepatocytes do. Hence, PSC should be treated early in its course. The goal of therapy is to prevent further damage and destruction of bile ducts. In this article, we will present relevant data concerning the medical management of primary sclerosing cholangitis. Received for publication on March 8, 1999; accepted on April 5, 1999     

Recent status of primary sclerosing cholangitis in Japan

Patients with primary sclerosing cholangitis (PSC) in Japan have two peaks in age distribution, one in their twenties and the other in their fifties and sixties. PSC patients in Japan have different characteristics from those in other countries: there is a higher incidence of eosinophilia (27%) and positivity for anti-nuclear antibody (30%), less frequent complication with inflammatory bowel diseases (IBD; 21%), and more frequent complication with chronic pancreatitis (15%). In younger patients in Japan (those aged less than 40 years), the incidence of positivity for anti-nuclear antibody was lower (20% vs 38% P < 0.05), complication with IBD was more frequent (39% vs 9% P < 0.01), complication with chronic pancreatitis was less frequent (4% vs 22% P < 0.01), and damage to both the intra- and extrahepatic bile ducts was more frequent (89% vs 56% P < 0.01) than in older patients (those aged 40 years or more). These findings suggest that younger PSC patients in Japan have characteristics similar to those of patients in other countries, and that in Japan older PSC patients have a different pathogenesis from that of younger patients. Received for publication on Feb. 16, 1999; accepted on April 5, 1999     

Clinicopathologic findings of recurrent primary sclerosing cholangitis after orthotopic liver transplantation

Whether primary sclerosing cholangitis (PSC) occurs after orthotopic liver transplantation is controversial, largely because the pre-transplant diagnosis of PSC is based on nonspecific radiological and histological findings. We reviewed clinical, radiological, and histological records of 53 patients who underwent liver transplantation for PSC between 1985 and 1998. Three patients with patent hepatic arteries and no evidence of chronic rejection had radiological and histological findings that may have been due to recurrent PSC. Bile duct stricturing in these patients proved permanent and progressive and affected both the quality of life and graft survival. The first patient, who is 110 months after transplantation, has had repeated episodes of cholangitis for the last year. The second patient underwent excision of a strictured hepatic duct 45 months after transplantation and was ultimately retransplanted 95 months after initial transplantation. The third patient underwent left hemihepatectomy of an atrophied lobe 50 months after transplantation. Although the patient population assessed in this study is limited, putative recurrent PSC in the allografts has led either to graft loss or to clinically significant hepatobiliary complications of the graft. Received for publication on March 8, 1999; accepted on April 30, 1999     

前列地尔治疗老年急性重症胆管炎50例

目的 :观察前列地尔治疗老年急性重症胆管炎 (ACST)的疗效。方法 :ACST患者 96例 ,均给予抗休克、抗感染和手术治疗 ,其中治疗组 5 0例 ,同时加用前列地尔注射液 10 μg于 0 .9%氯化钠注射液 10mL中 ,iv ,qd ,疗程 1周 ;对照组 46例 ,给予全肠外营养。结果 :治疗组疗效优于对照组 ,血浆蛋白升高明显 ,严重并发症发生率和病死率低 ,两组均差异有显著性 (P <0 .0 5 )。结论 :前列地尔联合全肠外营养是治疗老年ACST重要的、有效的支持方法。     

LANGERHANS' CELL GRANULOMA CONFINED TO THE BILE DUCT

Langerhans' cell histiocytosis (LCH) of the liver is uncommon. When seen, it is part of multifocal disease and can present as biliary obstruction. We present a case of sclerosing biliary disease with a solitary LCH lesion and no evidence of systemic disease. We postulate that the LCH is a secondary phenomenon, arising against a background of a complex, familial liver disease. This case also raises the possibility that some instances of idiopathic sclerosing cholangitis may follow cryptic LCH of the bile ducts.     

自身免疫性肝病的病理学新进展

自身免疫性肝病是指由于机体免疫系统攻击自体肝组织引起的肝组织损伤和肝功能异常的一组免疫性疾病,包括有自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(PBC)和原发性硬化性胆管炎(PSC).通常情况下这三种肝脏疾病独立存在,约有6％～9％的患者可以在同一时段或病程中出现两种疾病的临床表现、血清学和组织学特征,称为重叠综合征.近年来随着对自身免疫性肝病认识水平的提高以及自身抗体检测方法的不断改进,一些学者在自身免疫性肝病的经典肝组织学病变特点的基础上,提出了一些见解,介绍如下.