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Case Based Question & Answer Session: Liver Diseases

Case Based Question & Answer Session on Liver Pathology, Abnormal LFTs and Jaundice, Hepatitis B, Hepatitis C, Autoimmune Hepatitis, Alcoholic and Nonalcoholic Liver Disease, Cholestatic Liver Disease, Liver Tumors, Cirrhosis, Portal Hypertension Bleeding, Ascites, Hepatorenal syndrome and Spontaneous Bacterial Peritonitis, Acute Liver Failure, Liver Transplant, Liver Disease in Pregnancy, Drug-induced Liver Injury, Vascular and Infectious Complications

October 13, 2020

Cirrhosis of the Liver Practice Questions with Answers and NCLEX ® Review

Cirrhosis of the liver is a chronic, progressive disease that leads to liver scarring (or fibrosis). Several factors can cause this scarring, including but not limited to alcoholism and hepatitis B and C.

Cirrhosis of the Liver Practice Questions with Answers

Learning outcomes, test taking tips, introduction to cirrhosis of the liver.

The liver has a wide range of functions, including filtering toxins from your blood, producing bile to help digest food, helping with blood clotting, and storing vitamins and minerals.

Cirrhosis of the liver is a chronic disease that is caused by long-term damage to the organ. Cirrhosis can cause major complications, including liver failure, bleeding in the esophagus or stomach, or death.

Clients with cirrhosis most likely experience liver damage from:

• Alcohol abuse (alcoholic hepatitis)
• Viral infections (hepatitis C)
• Autoimmune disorders (lupus or scleroderma)
• Genetic diseases (hemochromatosis or Wilson’s disease)
• Cystic fibrosis (severe mucus clogging the entire body & the liver)

Cirrhosis of the Liver Pathophysiology

The liver helps with digestion, detoxification, and blood filtering (about one liter of blood every minute). It also makes bile, which helps digest fat and stores red blood cells when they are not needed. Each time the liver is injured by excessive alcohol intake, medication overuse, or disease, it tries to repair itself.

In this process, scar tissue forms in place of healthy liver cells, which eventually hardens into nodes called fibrosis or cirrhosis. This scarring process can also cause nodules called hepatic adenomas. Severe cirrhosis complications could require a transplant, especially if the client experiences fluid build-up or bleeding in the stomach.

Memory Trick : Think liver cirrhosis – since normal healthy tissues get replaced with scar tissue, making the liver hard like a rock .

Cirrhosis of the Liver Signs & Symptoms

Clients with cirrhosis have an increased risk of developing liver cancer and intestinal bleeding, as well as experiencing other complications that can lead to death.

Symptoms of cirrhosis depend on how much scar tissue has formed in the liver. In the early stages, symptoms may be non-specific and hard to identify. Common symptoms include :

• Jaundice – Yellow skin and eyes from a build-up of bilirubin (dead RBCs).
• Portal hypertension – High pressure in the portal vein.
• Ascites – Huge fluid-filled abdomen as fluid backs up from the hard liver, and now spills into the third space.
• Esophageal Varices – The enlargement of veins in the esophagus. As blood backs up from the liver, it forces major pressure on the esophagus, causing the vessels to bulge.

Other symptoms include :

• Sudden weight loss
• Enlarged abdomen (from fluid buildup)
• Tenderness or pain in the area below your ribcage on the right side
• Itchiness or dry skin on your palms and soles
• Nausea and vomiting

Nursing Assessment & Interventions for Cirrhosis

Nursing interventions for cirrhosis are most effective when they are tailored to the individual client. Therefore, nursing interventions should focus on each client as an individual, considering the stage of disease, underlying risk factors, and problems.

As part of a nursing care plan for cirrhosis, nursing assessments and interventions could include:

Assessments

• Current liver function (and compare with baseline levels).
• Blood pressure, pulse rate, and respiratory rate.
• Skin integrity for jaundice or other signs of liver failure (e.g., spider angiomas).
• Nutritional status (the client may require an intravenous line or tube feeding).
• Cognition and understanding of their condition so they can adhere to treatment recommendations (e.g., what medications are being prescribed).
• The abdomen for abdominal palpation (when the liver is enlarged and firm on palpation).

Interventions

• Educate clients about the causes of their illness and how to prevent complications.
• Encourage clients to eat a healthy diet and avoid alcohol and tobacco use.
• Provide emotional support and help them cope with changes in their lifestyle.

Lab Values for Cirrhosis of the Liver

The ABC s of the liver ( A lbumin, B ilirubin, C oagulation) will be low. This includes low calcium from the low albumin leading to the two classic signs: Trousseau’s and Chvostek’s .

• A lbumin Low (under 3.5), Low platelets → Calcium Low
• B bilirubin High
• C oagulation Panel (clotting time High ), High PT, PTT, INR
• Ammonia High → Hepatic Encephalopathy
• Elevated ALT & AST

Cirrhosis Pharmacology

Neomycin – Neomycin’s impact on the intestinal bacterial flora effectively lowers blood ammonia in cirrhotic clients – whether or not they have neurologic abnormalities. Lactulose – Lactulose is used to lower the ammonia levels in the blood. Ammonia is eliminated from the body by pulling it from circulation and into the colon.

During my exam, I could literally see and hear him going over different areas as I was answering my questions.

This past Friday I retook my Maternity Hesi and this time, I decided for my last week of Holiday break to just watch all of his OB videos. I am proud to say that with Mike’s help I received a score of 928 on my Maternity Hesi!

Cirrhosis of the Liver Conclusion

The liver aids in blood filtration, cleansing, and digestion (about one liter of blood every minute). It performs a variety of tasks, such as removing toxins from the blood, creating bile to aid in food digestion, promoting blood clotting, and storing vitamins and minerals.

When cirrhosis of the liver occurs, scar tissue replaces healthy liver cells. This tissue eventually hardens into nodules, known as cirrhosis or fibrosis. Because of the decreased blood flow brought on by the liver’s scarring, the nodules enlarge and eventually develop into tumors known as hepatic adenomas .

Clients with cirrhosis are more likely to have liver cancer, gastrointestinal bleeding, and other life-threatening consequences.

https://www.merckmanuals.com/professional/hepatic-and-biliary-disorders/fibrosis-and-cirrhosis/cirrhosis https://www.mayoclinic.org/diseases-conditions/cirrhosis/symptoms-causes/syc-20351487

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Practice Questions

Enhance your learning with these high-yield, original questions based on important topics in the general and transplant hepatology curricula. These questions are created by transplant hepatology fellows and reviewed by attending hepatologists but are not validated for ABIM boards.

Hepatology Practice Questions, Edition 2

The second edition of LFN’s original practice questions is here! These questions are created by transplant hepatology fellows with faculty oversight, and are intended to help solidify core concepts...

Jessica Davis

Matthew Odenwald

Hepatology Practice Questions, Edition 1

The first edition of LFN’s original practice questions is here! These questions are created by transplant hepatology fellows with faculty oversight, and are intended to help solidify core concepts...

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Clinical Cases in Hepatology

• © 2022
• Nora V. Bergasa 0

Department of Medicine, H+H/Metropolitan Physician Affiliate Group of New York, New York, USA

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• Features case presentations of how to appropriately approach treating patients with liver disease
• Describes the epidemiological characteristics of a variety of liver diseases
• Details techniques for interpreting experimental data and techniques for conducting clinical trials

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About this book

This book provides a comprehensive resource for clinical hepatology. It details the systematic approach to patients with liver disease in outpatient and inpatient medical settings. A variety of case studies in hepatology including chronic viral hepatitis, and metabolic, autoimmune, and alcohol related liver disease are presented. The book enables the reader to develop a thorough understanding of the clinical presentation, natural history, epidemiology, genetics, and therapeutic options for the liver diseases that clinicians must recognize and manage from the first encounter with the patient through the years of follow up.

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The Hepatological Curiosities

Diseases of the Liver

• Ischemic hepatitis
• Autoimmune hepatitis
• Fatty liver disease
• Biliary cirrhosis

Table of contents (15 chapters)

Front matter.

Nora V. Bergasa

Approach to the Patient with Liver Disease

Primary biliary cholangitis, autoimmune hepatitis, primary sclerosing cholangitis, chronic hepatitis c, chronic hepatitis b, alcohol induced liver disease, nonalcoholic fatty liver disease, alpha-1-antitrypsin deficiency, hemochromatosis, wilson’s disease, tumors of the liver, drug induced liver injury, complications of liver disease, back matter, editors and affiliations, about the editor.

Dr. Nora V. Bergasa is Professor of Medicine Emerita at New York Medical College and Chairman Emerita of the Department of Medicine at New York City (NYC) Health + Hospitals (H+H)/ Metropolitan and a retired member of the Physician Affiliate Group of New York. She serves as Hepatology Attending at NYC, H+H/Woodhull. She graduated from medical school from the Universidad Central del Este in the Dominican Republic, did her internal medicine residency and gastroenterology fellowship at the State University of New York (SUNY) at Downstate, and completed her clinical and research training in hepatology in the Liver Diseases Section of the National Institutes of Health. She has conducted basic and clinical investigations in several areas of hepatology, including cholestasis. Her major research field has concerned the pruritus of liver disease for which she is internationally recognized.

Bibliographic Information

Book Title : Clinical Cases in Hepatology

Editors : Nora V. Bergasa

DOI : https://doi.org/10.1007/978-1-4471-4715-2

Publisher : Springer London

eBook Packages : Medicine , Medicine (R0)

Copyright Information : Springer-Verlag London Ltd., part of Springer Nature 2022

Hardcover ISBN : 978-1-4471-4714-5 Published: 26 October 2021

eBook ISBN : 978-1-4471-4715-2 Published: 25 October 2021

Edition Number : 1

Number of Pages : XII, 497

Number of Illustrations : 3 b/w illustrations, 34 illustrations in colour

Topics : Hepatology

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Liver disease case studies: Case study level 1 – Alcoholic cirrhosis; alcohol withdrawal

In this chapter Case studies levels 1–3 explore the management of a patient with alcoholic liver disease. The patient has alcoholic liver cirrhosis and first presents with alcohol withdrawal (Case study level 1), then the patient’s risk of bleeding and treatment for the maintenance of alcohol abstinence are considered (Case study level 2). The patient then goes on to develop encephalopathy (Case study level 3). Case studies levels Ma and Mb consider two patients: one presents with TB and the other liver failure.

Case study level 1 – Alcoholic cirrhosis; alcohol withdrawal

Learning outcomes Level 1 case study: You will be able to:

• describe the risk factors
• describe the disease
• describe the pharmacology of the drug
• outline the formulation, including drug molecule, excipients, etc. for the medicines
• summarise basic social pharmacy issues (e.g. opening containers, large labels).

Mrs MW, 59 years old, is divorced and unemployed. She was admitted to an acute medical ward at the hospital presenting with general malaise, a grossly distended abdomen, swollen ankles, and jaundice. It was also noted that she smelt of alcohol and was showing signs of alcohol withdrawal.

1. What is cirrhosis of the liver?

2. List possible causes of cirrhosis.

3. What other clinical signs and symptoms may Mrs MW present with?

4. What drug treatment, including dose, would you recommend for Mrs MW’s alcohol withdrawal? What recommendations would you make if the patient was unable to take the medication orally?

1       What is cirrhosis of the liver?

Cirrhosis is defined as the histological development of regenerative nodules surrounded by fibrous bands in response to chronic liver injury. It is an advanced stage of liver fibrosis that is accompanied by distortion of the hepatic vasculature.

2        What are the risk factors for developing primary dysmenorrhoea?

Causes of cirrhosis can usually be identified by the patient’s history combined with serological and histological investigation. Alcoholic liver disease and hepatitis C and B are the most common causes of cirrhosis.

The association of excessive alcohol consumption with liver disease has been recognized for centuries. After the identification of the hepatitis C virus and of non-alcoholic steatohepatitis in obese patients with diabetes, the diagnosis of cirrhosis without an apparent cause (cryptogenic cirrhosis) is rarely made. Genetic causes of cirrhosis include hemochromatosis and Wilson’s disease.

Epidemiological studies have identified a number of factors that contribute to the risk of developing cirrhosis. Regular (moderate) alcohol consumption, age older than 50 years, and male gender are examples that increase cirrhosis risk in chronic hepatitis C infection, and older age, obesity, insulin resistance or type 2 diabetes, hypertension and hyperlipidemia in non-alcoholic steatohepatitis.

3        What other clinical signs and symptoms may Mrs MW present with?

Cirrhosis is often asymptomatic until complications of liver disease are present. Mrs MW may present with itching, jaundice, dark urine, pale fatty stools, abdominal pain, nausea, fatigue, bleeding – such as nosebleeds, hepatic encephalopathy, hepatomegaly, ascites, distended abdominal veins, spider angiomata, palmar erythema and asterixis. She may also present with the signs and symptoms of alcohol withdrawal, which include irritability, anxiety, tachycardia, tremor, sweating, confusion, and hallucinations.

4        What drug treatment, including dose, would you recommend for Mrs MW’s alcohol withdrawal? What recommendations would you make if the patient was unable to take the medication orally?

Long-acting benzodiazepines (e.g. diazepam and chlordiazepoxide) are used to attenuate alcohol withdrawal symptoms but they also have a dependence potential. To minimize the risk of dependence, administration should be for a limited period only (e.g. chlordiazepoxide 20 mg 4 times daily, gradually reducing to zero over 7–14 days). Mild alcohol withdrawal symptoms may be treated with a lower starting dose, such as 15 mg four times a day. In all cases, the patient should be counseled about the proposed length of the treatment course . Benzodiazepines should not be prescribed if the patient is likely to continue drinking alcohol.

In patients unable to take medication by the oral route, diazepam may be administered by intramuscular or slow intravenous injection (into a large vein, at a rate of not more than 5 mg/min), at a dose of 10 mg, repeated if necessary after not less than 4 hours. Alternatively, diazepam may be administered via the rectal route as a rectal solution or suppository. The intramuscular route should only be used when both the oral and intravenous routes are not possible.

General references

• Schuppan D and Afdhal NH (2008) Liver cirrhosis. Lancet 371: 838–851.
• Heidelbaugh JJ and Sherbondy M (2006) Cirrhosis and chronic liver failure: Part II. Complications and treatment. American Family Physician 74: 767–776.
• Joint Formulary Committee (2008) British National Formulary 55. London: British Medical Association and Royal Pharmaceutical Society of Great Britain, March.
• Vincent WR, Smith KM, Winstead PS and Lewis DA (2007) Review of alcohol withdrawal in the hospitalized patient: management . Orthopedics 30: 446–449.

Author: Caron Weeks [BPharm (Hons), MRPharmS, DipPharmPrac. Lead pharmacist – Medicine, Southampton University Hospitals NHS Trust] and Mark Tomlin [BPharm, MSc, MRPharmS (IPresc) Consultant Pharmacist, Critical Care, Southampton General Hospital]

• Alcohol withdrawal
• Case study for pharmacist
• liver cirrhosis
• liver disease
• Pharmacy case study

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Cardiovascular Case Studies: Case study level Mb – Myocardial infarction

Noakhali Science and Technology University (NSTU)

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Cirrhosis of the Liver: A Case Report and Literature Review of a Rare Case Presentation of Autoimmune Hepatitis With Systemic Sclerosis

Shashank banait.

1 Department of Ophthalmology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, (Deemed to be University), Wardha, IND

Chetan Burriwar

2 Department of Medicine, Mahatma Gandhi Institute of Medical Sciences, Wardha, IND

Priti G Verma

3 Department of Obstetrics and Gynaecology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, (Deemed to be University), Wardha, IND

Tanvi Banait

4 Department of Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, (Deemed to be University), Wardha, IND

Madhura Joshi

Systemic sclerosis (SSc) is a chronic systemic disease that affects the skin, heart, lungs, kidneys, gastrointestinal tract, and musculoskeletal system. Although gastrointestinal involvement has been reported in approximately 90% of scleroderma patients, liver involvement is uncommon. A 51-year-old female was admitted to the hospital due to abdominal distension and pedal edema. She had a history of Raynaud's syndrome and multiple hypopigmented and hyperpigmented patches over her body for the last year. Her ascetic fluid analysis was transudative with a serum ascites albumin gradient >1.1, and the abdomen and pelvis ultrasonography reported liver cirrhosis with splenomegaly with perisplenic varices. Her antinuclear antibody and anti-centromere antibody were positive. Skin thickening was visible. Her alanine aminotransferase (ALT), aspartate aminotransferase (AST), and serum globulin were raised. Viral serology was negative. We managed her with diuretics, beta-blockers, prednisolone (30 mg/day administered orally), angiotensin-converting enzyme inhibitors, and calcium channel blockers. Edema and abdominal distension decreased with this management, and no Raynaud's phenomenon was observed during the hospital stay.

Introduction

Systemic sclerosis (SSc) is a rare chronic disorder of unknown etiology, characterized by diffuse fibrosis and generalized vascular abnormalities in the skin, joints, and internal organs, leading to the failure of these organs. The etiology of SSc is multifactorial, with multiple genetic, endogenous, and exogenous factors appearing to interact with disease development. The pathogenesis of fibrosis is complex and is due to the interaction between immunological events and vascular changes, which activate fibrogenic fibroblasts [ 1 ]. Autoimmune hepatitis is a chronic inflammation of the liver characterized by the presence of autoantibodies and raised serum globulin levels. It is predominantly a disease that affects women and can present at any age. The disease may begin as acute hepatitis, which may progress to cirrhosis [ 2 ]. It is commonly associated with various autoimmune diseases, like autoimmune thyroiditis, type 1 diabetes mellitus, glomerulonephritis, ulcerative colitis, autoimmune hemolytic anemia, and autoimmune thrombocytopenia. However, its association with systemic connective tissue disorders like SSc, systemic lupus erythematosus, and mixed connective tissue disorders is infrequent [ 3 , 4 ]. Here, we report the case of a patient presenting with cirrhosis of the liver due to autoimmune hepatitis along with systemic sclerosis.

Case presentation

A 51-year-old non-obese female with no history of alcoholism was admitted with complaints of gradually progressive abdominal distension and swelling over both lower limbs for the last month. She had difficulty swallowing 15 days before the presentation, which was gradual and worse with solid food. She had a history of multiple small hypopigmented and hyperpigmented patches around the neck, over the back, forearms, and legs for one year. She also presented with Raynaud's phenomenon, which was characterized by bluish discoloration followed by diffuse pain in her fingers since last year, especially during contact with water. However, there was no history of joint pain or deformity, morning stiffness, fever, pain in the abdomen, diarrhea, constipation, chest pain, or paroxysmal nocturnal dyspnoea or orthopnoea. There was no past history of any neurological deficit like dysarthria, gait abnormalities, dystonia, tremors, diabetes, hypertension, dyslipidemia, metabolic syndrome, rheumatic heart disease, or ischemic heart disease. A family history of liver cirrhosis or any chronic liver disease was obtained to rule out hereditary hemochromatosis, as well as red skin lesions and epistaxis for hereditary hemorrhagic telangiectasia, which were all negative.

On general examination, she was conscious, afebrile, and pale, with a pulse rate of 100/min and blood pressure of 110/80 mmHg. She was anicteric, and bilateral pitting pedal edema up to the knee was present. Salt and pepper pigmentation around the neck, over the back, forearms, and legs was present (Figure ​ (Figure1 1 ).

Skin distal to the metacarpophalangeal joint in the hands and at the elbow was tight, but there were no ulcers, swollen fingertips, calcinosis, sclerodactyly, or telangiectasia (Figure ​ (Figure2 2 ).

On systemic examination, the abdomen was distended with full flanks, the umbilicus was everted, fluid thrill and shifting dullness were present, and the liver was not palpable, but splenomegaly was present. The rest of the systemic (cardiovascular, respiratory, and central nervous system) examination did not reveal any abnormalities. On slit-lamp examination of the anterior chamber of the eyes, the Kayser-Fleischer (KF) ring was absent. Her hemogram revealed a hemoglobin of 9.6 g/dl, anisopoikilocytosis, microcytic hypochromic red blood corpuscles with a mean corpuscular volume of 71, a white blood corpuscle count of 3.88 with 77.1% granulocytes, and reduced platelets of 0.68 lac/ul. Her liver function tests showed hypoalbuminemia (2.44 gm/dl), hypergammaglobulinemia (4.08 gm/dl), and raised alanine aminotransferase (ALT) of 127 IU/L and aspartate aminotransferase (AST) of 276 IU/L with normal bilirubin and alkaline phosphatase (73 IU/L). Other parameters like blood sugar, serum electrolytes, lipid profile, kidney function tests, and electrocardiogram were normal. Her ascitic fluid was transudative in nature with a serum ascites albumin gradient of 1.35. Her blood, urine, and ascitic fluid cultures did not show the growth of any bacteria. The presence of bilateral pitting pedal edema, ascites, splenomegaly, hypoalbuminemia, and a serum ascites albumin gradient of 1.35 led to the diagnosis of liver cirrhosis with portal hypertension [ 5 , 6 ].

Ultrasonography of the abdomen showed a small-sized (9.7 cm) shrunken liver with coarsened echotexture of parenchyma with irregular margins, suggestive of liver cirrhosis with gross ascites, periportal fibrosis, and splenomegaly (14.1 cm), a dilated portal vein of 12 mm with the normal color flow on Doppler imaging, a dilated splenic vein of 15 mm, and a partially distended gall bladder. The common bile duct and intra-hepatic biliary radicals were normal (Figure ​ (Figure3 3 ).

Her fibroscan of the liver could not be done due to the unavailability of this facility at our rural hospital. Her viral serology, hepatitis B surface antigen (HBsAg) for hepatitis B, antibody to hepatitis C virus (anti-HCV), and quantitative hepatitis C virus ribonucleic acid (HCV RNA) by polymerase chain reaction (PCR) and human immunodeficiency virus (HIV) infection test by enzyme-linked immunosorbent assay (ELISA) method were negative. Her serum ferritin of 69 ng/ml, serum ceruloplasmin of 30 mg/dL, and 24-hour urinary copper of 20 µg/day were normal. Her anti-mitochondrial antibodies were negative. Antinuclear antibody 17 profile blot (ANA17B) and anti-centromere antibody with a class index of four were positive (Figure ​ (Figure4 4 ).

On the American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) criteria for the classification of SSc (2013), her total score was 15. (nine for skin thickening and three each for Raynaud's phenomenon and positive anti-centromere antibody [ 7 ]. A liver biopsy could not be performed as the patient did not give consent.

The provisional diagnosis of SSc with autoimmune hepatitis leading to cirrhosis of the liver was considered as per the international autoimmune hepatitis group diagnostic criteria [ 2 ] (Table  1 ).

ALT: alanine aminotransferase; ANA: antinuclear antibody; Anti - LKM - 1: Anti – Liver kidney microsomal – 1 antibody; ASMA: anti-smooth muscle antibody; AST: aspartate aminotransferase.

She was managed with diuretics, beta-blockers, angiotensin-converting enzyme inhibitors, calcium channel blockers, and steroids. She showed significant improvement in her symptoms; her edema and abdominal distension decreased, and no Raynaud's phenomenon was observed during the hospital stay.

Systemic sclerosis is a multisystem disorder that primarily affects the skin, heart, lungs, gastrointestinal tract, musculoskeletal system, and kidneys. The disease is characterized by tissue fibrosis, vasculopathy, and an autoimmune response associated with specific autoantibodies. Gastrointestinal involvement is common, up to 95% [ 8 ]. However, the liver is rarely involved in this disease. As shown in the review, eight (1.1%) of 727 patients with scleroderma had involvement of the liver [ 9 ]. Primary biliary cirrhosis is the most commonly found hepatic disease in patients with SSc, while autoimmune hepatitis is rare [ 10 , 11 , 12 ].

Although the pathogenesis of the development of autoimmune hepatitis in patients with SSc is still unclear, it may be due to a dysregulated response of the cellular and humoral immune systems. According to the literature, anti-centromere antibodies are found in 3-13% of patients with autoimmune hepatitis [ 1 , 9 ]. Our patient was diagnosed with autoimmune hepatitis according to the criteria proposed by the international autoimmune hepatitis group, and she fulfilled all the diagnostic criteria as mentioned in Table ​ Table1 1 [ 2 , 13 ].

A few cases of autoimmune hepatitis without primary biliary cirrhosis in SSc patients have been reported. In three cases, SSc was diagnosed before the development of autoimmune hepatitis, while in one case, both conditions were diagnosed simultaneously [ 14 - 17 ]. The clinical characteristics of five patients and their laboratory profiles, along with the patients described in the current report, are summarized in Table ​ Table2 2 .

ALT: alanine aminotransferase; anti-centromere: anti-centromere antibody; AST: aspartate aminotransferase; CREST: calcinosis, Raynaud’s phenomenon, oesophageal dysmotility, sclerodactyly, and telangiectasia syndrome; CRST: calcinosis cutis, Raynaud’s phenomenon, sclerodactyly, telangiectasia syndrome; FANA: fluorescent anti-nuclear antibody; g/dL: grams/deciliter; g/L: Grams/liter; IgG: immunoglobulin G; IU/L: international units /liter; lacs/ul: lacs/microliter; mg/day: milligrams/day; mg/dl: milligrams/deciliter; SSc: systemic sclerosis.

In our case, cirrhosis of the liver due to autoimmune hepatitis and SSc was diagnosed during the current admission. However, her fibroscan of the liver could not be done due to the unavailability of this facility at our rural hospital, and a liver biopsy could not be performed as the patient did not give consent. In the present case, liver involvement was advanced compared to the previous four cases. These previous case reports indicate that patients with SSc are at an increased risk of developing autoimmune hepatitis (Table ​ (Table2 2 ).

This case was diffuse SSc in which the patient had Raynaud's phenomenon, skin thickening in the hands, feet, and elbows, and salt and pepper pigmentation skin over the back and trunk also, but there were no calcinosis cutis, sclerodactyly, or telangiectasia. High-dose oral corticosteroids and azathioprine are the drugs of choice for treatment as they effectively alleviate symptoms and the outcome of autoimmune hepatitis [ 18 ].

Managing autoimmune hepatitis is difficult with diffuse cutaneous SSc patients, as these patients may develop a renal crisis with high-dose steroid therapy [ 19 , 20 ]. Future studies are required to determine the treatment outcome and complications of high-dose steroid therapy for combined autoimmune hepatitis and SSc.

Conclusions

Patients with SSc and autoimmune hepatitis should be observed for the development of other autoimmune diseases. A high degree of observation is necessary to diagnose autoimmune hepatitis in patients with SSc as symptoms are non-specific and may appear gradually over years. Patients with SSc should be monitored for liver function tests regularly as early diagnosis and treatment of autoimmune hepatitis will help achieve a reasonable remission rate.

The content published in Cureus is the result of clinical experience and/or research by independent individuals or organizations. Cureus is not responsible for the scientific accuracy or reliability of data or conclusions published herein. All content published within Cureus is intended only for educational, research and reference purposes. Additionally, articles published within Cureus should not be deemed a suitable substitute for the advice of a qualified health care professional. Do not disregard or avoid professional medical advice due to content published within Cureus.

The authors have declared that no competing interests exist.

Human Ethics

Consent was obtained or waived by all participants in this study. Institutional Ethics Committee, Mahatma Gandhi Institute of Medical Sciences, Sevagram. Reg. No. ECR/47/Inst/MH/2013/RR-19 issued approval MGIMS/IEC/MED/336/2022. Approved.

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Patients with chronic liver disease face emotional conflicts during carcinoma surveillance

by Toho University

Assistant Professor Keiko Hatanaka of Toho University, a Ph.D. student of Tokyo Medical and Dental University (TMDU), in collaboration with Professors Yoshiko Sasaki and Makoto Tanaka of Tokyo Medical and Dental University (TMDU), revealed the process of adjusting to living with chronic liver disease (CLD) among patients who continued regular health care visits for hepatocellular carcinoma (HCC) surveillance.

This process is characterized by a cycle with three-phase transitions centered around the core concepts of "inferring my liver condition" and "desiring the status quo."

The transitions are described as follows: Phase 1 involves seeking ways to live with CLD, Phase 2 encompasses being overwhelmed by living with CLD, and Phase 3 focuses on reconstructing one's life to accommodate living with CLD.

Over time, the relative importance of the cycle's core gradually shifts from "inferring my liver condition" to "desiring the status quo."

These findings were published in the Japan Journal of Nursing Science .

The researchers found that patients with CLD who continue regular HCC surveillance visits experience emotional conflicts "caused by continuing" their regular visits; in other words, surveillance adds to their difficulty of living with CLD.

The ability to shift focus from "inferring my liver condition" to "desiring the status quo" is key to overcoming the challenges of continuing HCC surveillance visits. However, placing too much emphasis on "desiring the status quo" can lower the priority of HCC surveillance.

A balanced focus on "desiring the status quo" while still considering "inferring my liver condition" appears to be crucial for sustaining regular HCC surveillance.

In addition, the psychological impact of uncertainty during surveillance visits, stemming from the discrepancy between inferred liver conditions and actual test results, can lead to patients neglecting their liver condition.

Conversely, this uncertainty can also motivate health-conscious behaviors following a surveillance visit, potentially influencing health practices leading up to the next visit.

This study offers valuable insights into ways to support patients with CLD in continuing regular health care visits for HCC surveillance.

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Study reveals key role of A20 protein in controlling inflammation in liver disease

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Many individuals worldwide suffer from chronic liver disease (CLD), which poses significant concerns for its tendency to lead to hepatocellular carcinoma or liver failure. CLD is characterized by inflammation and fibrosis. Certain liver cells, called hepatic stellate cells (HSCs), contribute to both these characteristics, but how they are specifically involved in the inflammatory response is not fully clear. In a recent article published in The FASEB Journal , a team led by researchers at Tokyo Medical and Dental University (TMDU) uncovered the role of tumor necrosis factor-α-related protein A20, shortened to A20, in this inflammatory signaling.

Previous studies have indicated that A20 has an anti-inflammatory role, as mice lacking this protein develop severe systemic inflammation. Additionally, certain genetic variants in the gene encoding A20 result in autoimmune hepatitis with cirrhosis. This and other published work made the TMDU team become interested in how A20 functions in HSCs to potentially affect chronic hepatitis.

We developed an experimental line of mice called a conditional knockout, in which about 80% to 90% of the HSCs lacked A20 expression. We also simultaneously explored these mechanisms in a human HSC cell line called LX-2 to help corroborate our findings in the mice." Dr. Sei Kakinuma, author of the study

When examining the livers of these mice, the team observed inflammation and mild fibrosis without treating them with any inducing agent. This indicated that the observed inflammatory response was spontaneous, suggesting that HSCs require A20 expression to suppress chronic hepatitis. 

"Using a technique called RNA sequencing to determine which genes were expressed, we found that the mouse HSCs lacking A20 displayed expression patterns consistent with inflammation," describes Dr Yasuhiro Asahina, one of the study's senior authors. "These cells also showed atypical expression levels of chemokines, which are important inflammation signaling molecules." 

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When working with the LX-2 human cells, the researchers made similar observations to those for the mouse HSCs. They then used molecular techniques to express high amounts of A20 in the LX-2 cells, which resulted in decreased chemokine expression levels. Through further investigation, the team identified the specific mechanism regulating this phenomenon.

"Our data suggest that a protein called DCLK1 can be inhibited by A20. DCLK1 is known to activate an important pro-inflammatory pathway, known as JNK signaling, that increases chemokine levels," explains Dr Kakinuma. 

Inhibiting DCLK1 in cells with A20 expression knocked down resulted in much lower chemokine expression, further supporting that A20 is involved in inflammation in HSCs through the DCLK1-JNK pathway. 

Overall, this study provides impactful findings that emphasize the potential of A20 and DCLK1 in novel therapeutic development for chronic hepatitis.

Tokyo Medical and Dental University

Watakabe, K.,  et al. (2024) A20 in hepatic stellate cells suppresses chronic hepatitis by inhibiting DCLK1–JNK pathway‐dependent chemokines.  The FASEB Journal . doi.org/10.1096/fj.202400109r .

Posted in: Medical Science News | Medical Research News | Medical Condition News

Tags: Anti-Inflammatory , Autoimmune Hepatitis , Carcinoma , Cell , Cell Line , Chemokine , Chemokines , Chronic , Cirrhosis , Fibrosis , Gastroenterology , Gene , Genes , Genetic , Hepatitis , Hepatocellular Carcinoma , Hepatology , Inflammation , Knockout , Liver , Liver Disease , Necrosis , Phosphorylation , Protein , RNA , RNA Sequencing , Tumor , Tumor Necrosis Factor

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