Inside autoimmune liver disease 40 Nursing made Incredibly Easy! January/February 2019 www.NursingMadeIncrediblyEasy.com Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved. 1.5 ANCC CONTACT HOURS An overactive immune system can target any body tissue and cause damage. In AILD, the liver and bile ducts are under attack. By Richard L. Pullen, Jr., EdD, MSN, RN, CMSRN, and Patricia Francis-Johnson, DNP, RN Autoimmune liver disease (AILD)—primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and autoimmune hepatitis (AIH)—is comprised of three distinct pathologic processes in which a person’s immune system doesn’t recognize certain hepatic and biliary structures as belonging to the body. The im- mune system becomes overactive and targets healthy tissue, leading to inflammation, cirrho- sis, and end-stage liver disease (ESLD). Some patients may require liver transplantation as a lifesaving measure. This article presents the pathophysiology, epidemiology, risk factors, signs and symptoms, treatment strategies, and nursing care of patients with AILD. Primary biliary cholangitis PBC is a progressive inflammatory autoimmune disease that’s manifested by a destruction of the small intrahepatic bile ducts. Pathophysiology Destruction of the bile ducts leads to cholestasis, which causes an impairment in the flow and retention of bile—a substance produced by the liver and stored in the gallbladder that flows through the bile ducts and into the small intes- tine to digest lipids. Bile is comprised of bile salts, cholesterol, and bilirubin—a waste product from damaged red blood cells that accumulates in the blood because of cholestasis in PBC. Approximately 95% of patients with PBC pos- sess the antimitochondrial antibody (AMA) and a PBC-specific antinuclear antibody (ANA) that has a MEGIJA / CANSTOCK rim-like staining pattern. These antibodies mediate www.NursingMadeIncrediblyEasy.com January/February 2019 Nursing made Incredibly Easy! 41 Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved. an immune response by causing a prolif- liver makes a futile attempt to transform eration of T lymphocytes that attack and hepatocytes and progenitor cells into destroy the cholangiocytes of the interlobu- new bile ducts. Progenitor cells normally lar and septal bile ducts. The T lympho- replace damaged or dead cells in the liver. cytes are both CD4 and CD8 cells and may Because the ducts are no longer present, be reactive to PDC-E2—another antibody bile gets trapped in the liver and causes in PBC. T-lymphocytes may contain granu- intense inflammation and destruction of lomas that are identified as a “florid duct hepatic structures, leading to cirrhosis lesion” through liver biopsy. A florid duct (see A brief cirrhosis primer). lesion is an intense area of inflammation. Over time, the damaged bile ducts Epidemiology and risk factors disappear (ductopenia) even though the PBC affects approximately 35 out of 100,000 individuals. The typical patient is female between the ages of 40 and 60; A brief cirrhosis primer 90% of patients are female and 10% are Point #1 male. Research indicates that individu- PBC, PSC, and AIH may lead to cirrhosis, which results from a progres- als at greater risk for PBC are those who sion of intense inflammation to a fibrotic or scarring process that causes have a family history of the disease and ischemia in the liver. Ischemia leads to the liver being unable to carry out a coexisting autoimmune disease, such its vital functions, including, but not limited to, bile production, absorbing as Sjögren syndrome, thyroid disease, or and metabolizing bilirubin, metabolizing carbohydrates, storing vitamins, rheumatoid arthritis. protein synthesis, blood clotting, and metabolizing drugs and toxins. Researchers postulate that environmen- Point #2 tal factors may trigger an immune response The portal tracts are usually involved in the cirrhotic process. Portal tracts in the development of PBC, including, but are connective tissues that join the portal vein and hepatic artery to supply not limited to, cigarette smoke; hair dye; the liver with blood and nutrients. Bridging necrosis may also be present in hormonal therapy; toxic superfund sites; the portal tracts. Bridging necrosis is areas of confluent necrosis from the and infections, such as Escherichia coli infec- hepatic lobes to the portal tracts. tion, Epstein-Barr virus, herpes virus, retro- Point #3 viruses, and HIV-1. Some medications have The scarred or fibrotic liver causes a blockage of blood flow through the portal been implicated in triggering an immune venous system. This blockage causes pressure in the blood vessels through- response in PBC, including interferon and out the body, particularly those that line the esophagus and stomach, which chlorpromazine. places the patient at high risk for hemorrhage. This is called portal hyperten- sion. Ascites—a leakage of fluids and proteins into the peritoneal space—may also occur. Third-space fluid shift may cause edema in the legs. Signs and symptoms Approximately 60% of patients are as- Point #4 ymptomatic at the time of diagnosis. A Elevated serum ammonia may occur from a breakdown in protein and diagnosis of PBC is suspected during a cause hepatic encephalopathy, which leads to changes in the patient’s mental status from altered brain function. routine checkup with an analysis of lab tests. Liver enzymes show a cholestatic Point #5 pattern, which means that the alkaline Fibrosis and bridging necrosis cause a collapse of the hepatic parenchyma, phosphatase (ALP) is disproportionately leading to ESLD. elevated to alanine aminotransferase Point #6 (ALT), aspartate aminotransferase (AST), Airway management, bleeding, anemia, fluid volume excess, infection, and gamma-glutamyl transferase (GGT). fatigue, nutrition, mobility, and safety are major problems for the ESLD Patients also have elevated bilirubin and patient that need to be addressed by the interprofessional team. prolonged prothrombin time. These lab Point #7 tests correlate with the presence of anti- Priority goal: Implement early and aggressive therapy in PBC, PSC, and bodies, specifically AMA. Most patients AIH to prevent the development of cirrhosis and progression to ESLD. will become symptomatic within a few 42 Nursing made Incredibly Easy! January/February 2019 www.NursingMadeIncrediblyEasy.com Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved. Picturing xanthelasma and xanthoma Xanthelasma Xanthoma Sources: Ayala C, Spellberg B. Boards and Wards for USLME Step 2. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2017. DeLong L, Burkhart NW. General and Oral Pathology for the Dental Hygienist. 3rd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2018. years of diagnosis; however, some patients legs (see Picturing xanthelasma and xantho- with PBC may initially present with severe ma). Patients may also have hepatomegaly, disease such as cirrhosis. splenomegaly, joint pain, and dry eyes and The most common symptoms of PBC are mouth (Sicca syndrome). fatigue, jaundice, and pruritus. Research- A diagnosis of PBC may be made by cor- ers postulate that fatigue is caused by an relating the patient’s physical symptoms increase in mitochondrial activity. Patients with lab data. However, a liver biopsy is must also cope with chronic pruritus, usually necessary to confirm the diagnosis, which interrupts rest and sleep, leading to determine the extent of liver damage, and fatigue. Cholestasis causes an accumulation develop a treatment plan. The Ludwig of bile acids in the systemic circulation and Histological Staging System for PBC is com- on the skin. The bile acid receptor cell TGR5 monly used to classify the disease: may affect neurotransmission from the cho- • Stage 1: florid duct lesion lestatic process to the skin, causing intense • Stage 2: loss of normal bile duct struc- pruritus. Scratching from pruritus may ture, an increase in the number of biliary cause the skin to bleed. Jaundice from an epithelial cells, and inflammation of the increase in bilirubin and hyperpigmenta- hepatic parenchyma tion of the skin from an increase in melanin • Stage 3: progressive loss of bile ducts deposits on the skin may accompany PBC. and bridging necrosis Jaundice may indicate hepatic decompen- • Stage 4: cirrhosis with ESLD. sation, leading to ascites, peripheral edema, and gastrointestinal bleeding. Changes in Treatment strategies mental status may indicate the onset of The first-line treatment in PBC is admin- hepatic encephalopathy. istering ursodeoxycholic acid (UDCA)—a Two cutaneous features of PBC are bile acid normally found in the body that xanthelasma and xanthoma. These yel- isn’t toxic to hepatocytes, unlike other bile low, fatty lesions are caused by a markedly acids. Increasing the amount of UDCA elevated cholesterol level in PBC, although in the body reduces biliary epithelial and they may also occur in other disease states. hepatic cell damage from cholestasis and Xanthelasma appears around the eyes and slows the progression of PBC by improving xanthoma appears on the hands, arms, and bile flow through the ducts. UDCA may www.NursingMadeIncrediblyEasy.com January/February 2019 Nursing made Incredibly Easy! 43 Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved. also reduce the immune-mediated response sufficient fluid and fiber in their diet to did you know? underpinning disease development, reduce avoid constipation. The antituberculosis Primary biliary cholesterol, and prevent gallstones.