Cases Of Primary Biliary Cirrhosis Research Articles

S1687 A Case of Primary Biliary Cirrhosis With New Onset Hyperthyroidism and Supratherapeutic INR

S1687 A Case of Primary Biliary Cirrhosis With New Onset Hyperthyroidism and Supratherapeutic INR

Serum Ghrelin Concentration in Patients With Primary Biliary Cirrhosis (PBC).

Introduction: The appetite-modulating hormone ghrelin may have a role in the etiology of anorexia which is a serious concern in patients with primary biliary cirrhosis (PBC). This study aims to assess the difference in ghrelin level between cases of PBC and healthy controls matched for age and gender, and to evaluate the level of ghrelin in relation to clinical and laboratory findings among cases.Methods: Twenty patients with primary biliary cirrhosis and 30 healthy controls matched by gender and age were recruited. The severity of liver disease was determined using the Child-Pugh grading system. Clinical comorbidities such as a history of ascites, gastrointestinal bleeding, and encephalopathy were evaluated. A commercial enzyme-linked immunosorbent assay was used to measure total ghrelin. Results: PBC cases had a significantly higher average level of ghrelin (2305.3 ± 639.4) pg/mL compared to controls (682 ± 197.3) pg/mL. Furthermore, the minimum reported level in cases was 1258 pg/mL compared to 326 pg/mL in controls, while the maximum level nearly tripled the control’s maximum level. In PBC patients, plasma levels of total ghrelin showed a weak positive correlation with age, an inverse correlation with body mass index, and were not associated with gender. The level was significantly higher than those in the controls. Ghrelin was associated with the severity of cirrhosis. Levels of serum ghrelin were higher in patients with associated comorbidities such as a history of ascites, gastrointestinal bleeding, and encephalopathy.Conclusions: Our study demonstrated elevated serum ghrelin levels in patients with primary biliary cirrhosis. Serum ghrelin was associated with the degree of severity and the presence of related comorbidities. Patients with primary biliary cirrhosis remain anorexic and catabolic despite elevated ghrelin levels, suggesting tissue resistance to this anabolic peptide which could be crucial to understanding anorexia and cachexia in primary biliary cirrhosis.

Primary biliary cirrhosis in early childhood - A rare case report.

Introduction and importancePrimary biliary cirrhosis (PBC) is a chronic and progressive autoimmune liver disease with no known etiology. This disease is mainly characterized by granulomatous destruction of intrahepatic biliary ducts, severe peri-portal inflammation, and ultimate progress to liver fibrosis and cirrhosis. Here, we report a five-year-old girl diagnosed with PBC, presented to us with end-stage liver disease for liver transplantation. Our patient successfully underwent liver transplantation with an uneventful recovery. This case highlights the need for awareness to report further PBC cases in the pediatric age group.Case presentationA five-year old female child presented with a 6 months history of progressive jaundice. She had multiple admissions for hepatic encephalopathy and this time she was admitted for hepatic transplantation. On examination, she was icteric and had hepatomegaly. After thorough workup, she underwent successful hepatic transplantation and was alright post-operatively. At 6 months follow up, she is doing well.ConclusionPBC is rare in childhood. The natural history and exact incidence of PBC in childhood are not known. Hence, there is a need for awareness to report further PBC cases in the pediatric age group.

中西医结合治疗原发性胆汁性肝硬化合并炎症性肠病1例

中西医结合治疗原发性胆汁性肝硬化合并炎症性肠病1例

Investigation and analysis of HEp 2 indirect immunofluorescence titers and patterns in various liver diseases.

Antinuclear antibody (ANA) testing using indirect immunofluorescence assay (IIFA) is a common and economical method which contributes to detect systemic autoimmune diseases (SARD) and autoimmune liver diseases (AILD). The primary aim of our study was to investigate ANA positivity and their patterns in multiple liver diseases, including primary biliary cirrhosis (PBC), autoimmune hepatitis (AIH), hepatitis B virus infection (HBV), hepatitis C virus infection (HCV), and hepatic carcinoma (HCC). Besides, we also compared the ANA titers and patterns in patients with liver disease, SARD, and healthy controls (HC). A total of 2537 patients with SARD, 137 PBC cases, 57 AIH cases, 3420 HBV cases, 769 HCV cases, 268 HCC cases, and 1073 HC were retrospectively assessed. The titers and patterns of ANA were detected with the IIFA method. ANA positivity rate was considerably discernible between these diseases, which is 90.1% in SARD, 93.4% in PBC, 49.1% in AIH, 19.1% in HBV, 13.9% in HCV, and 23.5% in HCC. Moreover, only 4.9% of HCC cases, 2.5% of HBV patients, and 1.6% of HCV patients had an ANA titer ≥ 1:320. The mixed pattern which composed of at least two patterns majorly lied in PBC. AC-15 and AC-21 was frequently related to liver diseases; the former pattern was more frequently found in AIH (84.2%) and PBC (8.8%), and the latter pattern was easily seen in PBC (62.2%) and HCC (22.6%). The positive rate of ANA in HC was 12.2%, and its major pattern was AC-2. There are differences in ANA positivity among patients with SARD and various liver diseases. Some mixed patterns may provide important evidence for the diagnosis of PBC. Clinicians should pay attention to ANA patterns and titer during the interpretation of this test. Key Points • Defining the clinical relevance of antinuclear antibody (ANA) using indirect immunofluorescence assay in the context of diseases can be an important tool for the clinician in the diagnostic work-up of patients with liver diseases. • The mixed pattern of ANA is majorly found in primary biliary cirrhosis (PBC). ANA patterns including AC-15 and AC-21 are frequently related to liver diseases. AC-15 is more often found in autoimmune hepatitis (AIH) (84.2%) and PBC (8.8%), and AC-21 is easily found in PBC (62.2%, and hepatic carcinoma (HCC) (22.6%). • ANA positivity can be seen in 19.1% of hepatitis B virus infection (HBV) cases, 13.9% of hepatitis C virus infection (HCV) cases, and 23.5% of HCC cases. Only 2.5% of HBV patients, 1.6% of HCV patients, and 4.9% of HCC cases have an ANA titer ≥ 1:320.

A62 CANADIAN POPULATION-BASED EVALUATION OF THE NATURAL HISTORY OF PRIMARY BILIARY CHOLANGITIS OVER THE LAST DECADE

Abstract Background Few studies have evaluated the epidemiology of primary biliary cirrhosis (PBC) in North America. Ursodeoxycholic acid (URSO) improves outcomes amongst PBC patients. Aims Therefore, we undertook this study to determine the epidemiology of PBC in Canada, and impact of URSO on clinical outcomes, two decades after URSO approval in North America. Methods We used our previously validated coding algorithm to identify PBC patients in population based administrative databases in the Calgary Health Zone (population ~1.5 million) from 2005–2015. Multiple sources of data including inpatient, ambulatory, physician billing, laboratory, and pharmaceutical were linked. Annual prevalence and incidence were estimated using Poisson regression. Age/sex adjusted rate ratios were calculated. We used Cox regression models to estimate: all-cause mortality, liver transplant and decompensated cirrhosis free survival. In our models, we adjusted for demographic and clinical variables including response to URSO. Results During the study period over 11 years, the overall annual age/sex adjusted PBC incidence was 29.5 cases per million (48.3 and 9.9 per million for women and men, respectively). The highest incidence rate was observed among women aged 60–79 (90.9 per million) with an incidence rate ratio of 10.4 (95%: 6.83–15.9) compared to those aged 20–39. While incidence rate remained stable, prevalence rate increased significantly from 242 to 343 cases/ million, between 2005 and 2015 (P<0.01). Prevalence rate was highest at 965 cases/ million amongst women aged 60–79. We identified 299 incident PBC cases with a median follow up of 5.1 years (IQR 2.9–8.3). 87 (29.1%) of these incident cases developed decompensated cirrhosis or HCC, 10 patients (3.4%) underwent liver transplantation, and 44 (14.7%) patients died. The annual mortality rate was 2.9% (95%CI: 2.1- 3.8) compared to 3.4% (2.3–4.9%) a decade ago (P=0.04). The estimated 5-year survival rate was 87.6% (82.6- 91.3). Standardized mortality rate was 3.2 (95%CI: 2.2–4.1). Overall, 25% of our incident cohort were not prescribed URSO. Patients not prescribed URSO were more likely to be male, older age, have normal ALP and had decompensated cirrhosis at diagnosis (P<0.01 for all). Among those who used URSO, response rate was 86%. Response to URSO was an independent predictor of reduced risk of decompensated cirrhosis and liver transplant, and higher survival (aHR: 0.46, 0.29–0.74). Conclusions PBC prevalence is increasing in this North American population. While mortality rate decreases in our PBC cohort, survival was suboptimal compared to the general population, mainly due to late presentation at diagnosis. Therefore, better surveillance and early detection of possible PBC patients in the primary care setting are essential to improve PBC outcomes. Funding Agencies None

3016 Can Primary Biliary Cirrhosis Cause Bradycardia?

INTRODUCTION: Obstructive jaundice is a known cause of sinus bradycardia. It is usually attributed to the effect of bile salts on the electrophysiology of the heart and mainly on the SA node. But can Primary biliary cirrhosis cause bradycardia? Here, we are presenting a case of sinus bradycardia with a junctional rhythm that could be secondary to primary biliary cirrhosis. CASE DESCRIPTION/METHODS: A 65-year-old female with a history of primary biliary cirrhosis complicated by esophageal varices and recurrent ascites. The patient presented with a syncopal episode. The patient had a history of episodes of lightheadedness for several days before admission. Upon arrival at the hospital, the patient was bradycardiac with heart rate around 30 and she was hypotensive. She was given several doses of atropine and then she was started on dopamine drip. Initial EKG showed that the patient has a junctional rhythm. After reviewing the patient cardiac history, we found that the patient didn't have any heart issues before and she had a coronary angiogram done one year ago and showed normal coronaries. Medications were reviewed and showed that the patient was on Metoprolol 25 mg twice daily for anxiety and she was on it for more than 10 years and there were no recent changes in the dose. Labs showed that the patient had mild elevation is AST 41 IU/L and ALT 62 IU/L. Total serum bilirubin was 1.7 mg/dl (mildly elevated) and direct bilirubin was 0.7 mg/dl (mildly elevated). After beta-blocker was held, the patient heart rate improved, and dopamine drip was discontinued. DISCUSSION: This is a case of primary biliary cirrhosis who presented with symptomatic bradycardia. This patient was on beta-blockers for a long period of time and she didn't have any heart issues given her last coronary angiogram and echocardiogram were normal. We believe that one of the contributors of the patient bradycardia is the primary biliary cirrhosis itself as it can cause bradycardia which is believed to be secondary to bile salts. In conclusion, primary biliary cirrhosis should be considered as a cause of bradycardia and the use of metoprolol in those patients should be closely monitored as Metoprolol is primarily metabolized by the liver and can intensify the bradycardia.

Is extra-glandular organ damage in primary Sjögren's syndrome related to the presence of systemic auto-antibodies and/or hypergammaglobulinemia? A long-term cohort study with 110 patients from the Netherlands.

To test the hypothesis that systemic auto-antibodies or hypergammaglobulinemia are related to the prevalence of extra-glandular tissue organ damage (EGOD) in primary Sjögren's syndrome (SS). A real practice-based investigation of a relatively large (n = 110) Dutch cohort of primary SS patients systematically followed up in a large non-academic hospital. After a follow up of mean 8.2 years a significant correlation was found between disease duration and the prevalence of EGOD. We did not observe a relationship between the total number or type of systemic auto-antibodies or hypergammaglobulinemia and the total number of EGOD. However, there was a correlation between the prevalence of polyneuropathy (PNP) and antinuclear antibodies (ANA) as well as anti-Ro/SS-A positivity and there was an inverse relationship between the presence of anti-Ro/SS-A antibodies and primary biliary cirrhosis (PBC). All PBC cases were anti-Ro/SS-A and anti-La/SS-B negative but ANA positive. There was a trend for a higher occurrence of pleuro-pulmonary disease in the ANA negative cases. Although we did not find a relationship between the total number or type of systemic auto-antibodies and the total number of EGOD, there were correlations between specific systemic auto-antibodies and specific types of EGOD. The presence of ANA and anti-Ro/SS-A was associated with the occurrence of PNP, as well as was the absence of anti-Ro/SS-A with PBC.

Concomitant Case of Primary Biliary Cirrhosis and Autoimmune Hemolityc Anemia Responding to Corticosteroid and Ursodeoxycholic Acid in Young Woman

Primary biliary cirrhosis (PBC) is an autoimmune liver disease of unknown etiology and is characterized by chronic progressive cholestasis with destruction of the small intrahepatic bile ducts and associated most commonly with antimitochondrial antibodies. PBC is most common in women and is often associated with other autoimmune disease such as autoimmune hemolityc anemia (AIHA), rheumatoid arthritis, thyroiditis, and systemic lupus eritomatosus. We report one case, a 20 years old woman with AIHA have been treated by corticosteroid since last year and she came to the outpatient department (OPD) with fatique and jaundice. The result of laboratory were haemoglobin 8.7 mg/dL, white blood cell 8700 mg/dL, coomb test +2, total bilirubin 33.2 mg/dL, direct bilirubin 29.3 mg/dL, γGT: 297 mg/dL and alkalyphospatase: 158 mg/dL. The result of Abdominal CT scan showed the size of liver and spleen increased and normal common bile duct (CBD). The result of ANA test, anti-nuclear (ANA) and antimitochondrial M2 (AMA M2) antibodies were positive. From the physical examination, laboratory and CT scan Abdomen; the diagnose of this patient was AIHA with PBC. After treatment with corticosteroid (prednison 1mg/kg/day) and ursodeoxicholic acid (UDCA) for several weeks, the clinical manifestation of PBC such as jaundice getting better (the laboratory result: total bilirubin 2.7 mg/dL, direct bilirubin 1.5 mg/dL, gamma GT 80 mg/dL).

Otoimmün karaciğer hastalıklarında Helicobacter pylori ve üst gastrointestinal endoskopi bulgularının sıklığı

Background and Aims: Helicobacter pylori is the main cause of gastric lesions in chronic gastritis, autoimmune gastritis, peptic ulcer disease, and gastric cancer. Autoimmune liver disease is associated with various upper gastrointestinal mucosal lesions, which are not linked to the severity of the disease. The aim of this study was to retrospectively investigate upper gastrointestinal mucosal lesions in relation to the prevalence of Helicobacter pylori infection in autoimmune liver disease. Materials and Methods: This study included 99 patients with autoimmune liver disease and 110 dyspeptic patients as the control group. Endoscopy was performed in all patients, with biopsy specimens taken from the antrum and gastric body for histological examination and Helicobacter pylori detection. Patients were excluded if they had a history of acid suppression therapy, antibiotic or non-steroidal anti-inflammatory drug treatment, or if antrum and gastric body biopsy specimens could not be taken. Results: Helicobacter pylori was detected in 60% of autoimmune hepatitis cases, 57% of primary biliary cirrhosis cases, and 63% of controls. There was no statistically significant difference between the three groups. Abnormal findings during upper gastrointestinal endoscopy were found in 45% of autoimmune hepatitis cases, 52% primary biliary cirrhosis cases, and 43% of dyspeptic controls. Conclusion: Helicobacter pylori was detected in autoimmune liver disease cases at a similar frequency as in the control group. Endoscopic antral gastritis was more prevalent in autoimmune hepatitis than in primary biliary cirrhosis, but autoimmune liver disease was not significantly characterized by more upper gastrointestinal mucosal lesions than in dyspeptic controls.

A Case of Primary Biliary Cirrhosis Mimicking Acute Hepatitis B in the Clinic, Republic of Korea.

Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic autoimmune liver disease characterized by progressive bile duct injury. The most common symptoms of this disease include fatigue and pruritus. The diagnosis of PBC is based on cholestatic biochemical liver tests, presence of antimitochondrial antibodies, and characteristic histological biopsy findings. We report a case of a patient with PBS, who was initially suspected to be in the window period of hepatitis B by a private doctor in a local clinic based on the detection of isolated immunoglobulin M antibody against hepatitis B core antigen. The presence of this antibody is the most useful index in diagnosing acute hepatitis B (+) by immunoserological test. The final diagnosis of the patient in Good Gang-An Hospital was PBC through additional tests. The patient is receiving outpatient treatment.

Fungal infection involvement in primary biliary cirrhosis: A review of 2 cases.

The present study aimed to analyze the imaging, clinical and pathological features of fungal infection involvement in primary biliary cirrhosis (PBC) by retrospectively analyzing and reviewing the features of two patients with fungal infection involvement in PBC. Both patients were female. One patient had a confirmed diagnosis of PBC. The other patient had confirmed Sjogren syndrome and PBC. The two cases of PBC were infected with fungal infection after treatment with hormonal and immunosuppressive agents. RCR of sputum confirmed Pneumocystis spp. infection in the patient with PBC alone. The mucormycosis infection was confirmed in the other patient after pathological examination of a renal biopsy. The state of the illnesses progressed quickly and both patients ultimately succumbed to their conditions. The patient prognosis of fungal infection involvement PBC is poor. Patients treated with long-term hormone and immunosuppressive agents should be monitored.

A rare nonsynonymous variant in the lipid metabolic gene HELZ2 related to primary biliary cirrhosis in Chinese Han.

BackgroundSeveral genome-wide association studies of primary biliary cirrhosis (PBC) in European and Japanese origins have shown significant association of dozens of genetic loci contributive to the susceptibility of PBC. Most of the loci were related to immune response pathway. In this study, we tested whether the lipid metabolic gene HELZ2 was associated with the pathogenesis of PBC.MethodsIn 586 PBC cases (358 in case 1 group and 201 in case 2 group) and 726 healthy controls of Chinese Han, six nonsynonymous SNPs were genotyped by MassArray iPLEX. The same control were used for the two groups of PBC cases. Allele frequencies were calculated by χ2 test based on 2 × 2 contingency tables. All data were analyzed using the PLINK tool set. The odds ratio (OR) and 95 % confidence interval (95 % CI) were calculated, and p values (corrected for multiple testing by Bonferroni adjustment) less than 0.05 were considered statistically significant.ResultsThe A allele of rs79267778 was significantly associated with PBC (ORcombined = 4.204 [1.670–10.582], pcombined = 1.87E−04). It changed the amino acid at position 1904 (NM_001037335) from Threonine (ACG) to Methionine (ATG). This site was highly conserved in mammals and predicted to be POSSIBLY DAMAGING with a score of 0.469 by PolyPhen-2. It’s further predicted that T1904 M could INCREASE the protein stability with a confidence at 25.18 % under the condition of pH 7.0 and 37 °C.ConclusionThe result was the first time to show evidence of the lipid metabolic gene HELZ2 related to autoimmune disease, at least in PBC of Chinese Han.

One case of primary biliary cirrhosis with pulmonary interstitial lesions

One case of primary biliary cirrhosis with pulmonary interstitial lesions

経過中に自己免疫性肝炎の病像を合併した超高齢原発性胆汁性肝硬変の2例

症例1は84歳時に原発性胆汁性肝硬変（primary biliary cirrhosis；PBC）と診断されursodeoxycholic acid（UDCA）投与されたが治療を自己中断．86歳時に発熱と黄疸が出現，肝胆道系酵素，IgG，IgM著明高値，抗核抗体（ANA），抗ミトコンドリア抗体（AMA）陽性より，自己免疫性肝炎（AIH）を合併したと診断．一時肝不全徴候を呈したがpredonisolone（PSL）とazathioprineが奏功し64病日に退院．肝組織は，第30病日に亜広汎壊死，2年後にPBC stage 2を呈した．症例2は76歳時AMA陰性PBCと診断されUDCA内服開始．80歳時に全身倦怠感が出現，肝胆道系酵素，IgG著増，肝組織はPBC stage 2＋interface hepatitisを呈しAIHの病像を合併したと診断，PSL投与され著効した．両症例はPSL投与が必須で，示唆に富むと考え報告した．

Primary Biliary Cirrhosis in a Male: A Case Report and Review of Literature

A middle aged woman presenting with fatigue, pruritus, xanthelesma and hepatomegaly remains the typical presentation of primary biliary cirrhosis. Here, we present a case of primary biliary cirrhosis occurring in a male patient, who presented with fatigue and pruritus, had hepato-splenomegaly, raised serum alkaline phosphatase, positive anti-mitrochondrial antibody and negative antinuclear antibody.J MEDICINE July 2015; 16 (2) : 128-131

Clinical Characteristics of Concomitant Systemic Lupus Erythematosus and Primary Biliary Cirrhosis: A Literature Review.

Although autoimmune diseases often coexist, concomitant cases of systemic lupus erythematosus (SLE) and primary biliary cirrhosis (PBC) are uncommon. In this review paper, 34 cases of SLE with concomitant PBC found in English and Japanese scientific literature and Japanese proceedings were reviewed and summarized, including cases with liver dysfunction complicated by SLE. Of the 34 reported concomitant cases of SLE and PBC, 97.1% (33/34) were females, and PBC was diagnosed initially in 69.0% (20/29), except for five cases in which both SLE and PBC were simultaneously diagnosed. Sjögren's syndrome was the most common autoimmune disease complicating concomitant SLE and PBC (23.5%, 8/34). Five deaths have been reported: two elderly patients died of liver failure because of the worsening of PBC, and another two patients died from pulmonary infection associated with SLE pharmacotherapy. It is uncertain whether concomitant cases occur by chance or share a common immunological or genetic basis.

A Literature Review of Concomitant Primary Biliary Cirrhosis and GravesÂ’ Disease

Although Hashimoto’s thyroiditis (HT) is commonly characterized by extrahepatic manifestations of primary biliary cirrhosis (PBC), coexistence of PBC and Graves’ disease (GD) is uncommon. This is a review of the English and Japanese scientific literature, comprising 7 cases of PBC and GD coexistence. All patients were female. In 4 cases, both diseases were almost simultaneously diagnosed, whereas PBC preceded GD in the remaining 3 cases. One fatality was observed on account of sepsis and liver failure caused by progressing PBC.

肝切除を施行した原発性胆汁性肝硬変に発生した肝細胞癌の1例

症例は74歳，男性．68歳より原発性胆汁性肝硬変（primary biliary cirrhosis，以下PBC）の診断で経過観察中であった．定期フォローの腹部造影CTでsegment 7に右肝静脈に接する34mm大の腫瘤を認めた．精査にて肝細胞癌と診断，右肝静脈を含む拡大後区域切除術を施行した．術中PBCの硬変肝に対する肝切離は他の肝硬変症への肝切離と大きく異なる印象は無く，また術後も特異な合併症なく軽快した．切除標本の病理組織検査所見では中分化型を主体とする肝細胞癌を認め，非腫瘍部ではScheuer分類IV期のPBC像を呈していた．PBCに対する肝切除の報告では，PBCに特有の周術期合併症は認められず，PBCに発生した肝細胞癌に対する肝切除術を施行する際には，他の原因の肝硬変の際と同様に，癌の進行度診断と肝予備能評価に基づいて切除術式を決定することが重要と考えられた．

Concomitant Cases of Primary Biliary Cirrhosis and Autoimmune Hemolytic Anemia: Literature Review

Although autoimmune diseases are often concomitant, the coexistence of primary biliary cirrhosis (PBC) and autoimmune hemolytic anemia (AIHA) is not common. This is a review of the English and Japanese literature, regarding concomitant cases of PBC and AIHA. Among the 23 concomitant cases, PBC was diagnosed first in 10 cases and both diseases were almost simultaneously diagnosed in the remaining 13 cases. This suggests that there have been few concomitant cases in which AIHA developed first before the development of PBC. Moreover, there may be no correlation between the occurrence of AIHA and the staging or progression of PBC.