Inside Liver Disease

Inside autoimmune liver disease

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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 immune system becomes overactive and targets healthy tissue, leading to inflammation, cirrhosis, 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 intestine 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 hypertension. 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

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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. antibiotic rifampin is a second-line medica- cholangitis was UDCA is most effective in Stages 1 and tion to reduce pruritus in PBC because it previously called 2 when the bile ducts are still present. increases the rate of bile acid excretion in primary biliary cir- Patients in Stages 3 and 4 aren’t likely to the body. Liver enzymes, bilirubin level, rhosis. The name have a therapeutic outcome because of platelets, and WBC count should be moni- change more accu- ductopenia. UDCA is administered orally tored closely during therapy, although the rately describes and generally well tolerated. Diarrhea, medication has a low incidence of toxicity. what’s happening in a decrease in white blood cells (WBCs), Opioid receptor blockers, serotonin reup- the disease, which and an increase in blood glucose and take inhibitors, antihistamines, and corti- is an inflammation of the intrahepatic creatinine are the most common adverse costeroids may also be used to control pru- bile ducts. reactions. UDCA is effective in normaliz- ritus. Plasmapheresis may be considered ing liver enzymes and reducing antibody with intractable cases of pruritus. titers in at least 70% of patients with PBC. Patients with PBC have a poor prognosis Additional medications may be used when they have elevated bilirubin or signs to treat the underlying pathology of and symptoms of ESLD, such as ascites, PBC. Methotrexate (MTX) is an immuno- bleeding esophageal varices, and hepatic suppression agent that reduces inflam- encephalopathy. Some research findings mation in the bile ducts and improves indicate that one-third of patients die from hepatic function in combination with ESLD within 1 to 2 years. These patients UDCA. Patients should take folic acid to must have immediate liver transplantation decrease the chances of anemia second- to save their life. There’s a rapid improve- ary to MTX treatment. Corticosteroids, ment in quality of life after transplantation. such as budesonide, may also be used in The survival rate is approximately 90% at 5 PBC because of their immunosuppressive years and 70% at 10 years after transplan- properties. Patients should have good tation. PBC may recur after transplanta- body hygiene; avoid people who are sick; tion because the AMA is still a part of the and report symptoms of infection, such as patient’s immune system. Recurrence is a fever or a sore throat. Colchicine is an between 20% and 40% after transplantation. antigout agent that may be used in combination with UDCA for its anti-inflammatory Primary sclerosing cholangitis properties. The most common adverse PSC is an autoimmune disease that reaction is diarrhea that resolves once causes progressive inflammation, stric- the dosage is reduced. Major research tures, fibrosis, and obliteration of the in- studies indicate that colchicine also trahepatic and extrahepatic bile ducts. reduces fibrotic processes in the liver and delays the need for liver transplantation. Pathophysiology Improvement in liver enzymes is the PSC is classified as an autoimmune desired outcome of these agents. disease because it’s associated with the Symptom management is important. human leukocyte antigen (HLA) genes The cholesterol-lowering agent cholestyr- B8 and DR3. HLA is a cell surface protein amine is the first-line therapy for pruritus responsible for immune system regula- because it’s a bile acid resin, which means tion. Unlike PBC, PSC isn’t associated it metabolizes and removes excessive bile with specific antibodies. However, patients from the blood. It reduces pruritus in 90% with PSC may have nonspecific antibod- of patients with PBC. Patients shouldn’t ies, including ANA, anti-neutrophil cyto- take this medication with other agents to plasmic antibodies (ANCA), and smooth avoid problems with absorption and have muscle antibody (SMA). Ductopenia

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Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved. occurs secondary to obliterative cholangi- there are bile duct strictures or damage. tis and cholestasis, leading to portal tract Another approach to determining bile fibrosis, cirrhosis, portal hypertension, duct strictures or damage is through a ESLD, and a risk of liver cancer. Patients magnetic resonance cholangiopancreatog- with PSC are also prone to developing raphy (MRCP)—a noninvasive imaging pancreatitis, colorectal cancer, and cancer procedure of the intrahepatic and extra- of the bile ducts. hepatic bile ducts. A contrast medium is injected into the patient’s vein and images Epidemiology and risk factors of the bile ducts are obtained. PSC affects approximately 1 to 6 individu- A liver biopsy often complements a als out of 100,000. The disease predomi- diagnosis of PSC by ERCP, lab analysis, nately affects males of all ages and eth- and the patient’s signs and symptoms. A The nurse nicities. PSC may also be associated with characteristic histologic finding in PSC other autoimmune diseases, such as rheu- is a fibrous obliteration of the bile ducts and patient matoid arthritis, thyroiditis, celiac disease, with connective tissue in an “onion skin” become diabetes mellitus, myasthenia gravis, and pattern. This connective tissue is concen- partners in inflammatory bowel disease (IBD). Ap- tric and resembles the layers of an onion. proximately two-thirds of patients have PSC may be classified in the following the care plan IBD (ulcerative colitis and Crohn disease). manner: to achieve People with a Northern European heri- • Stage 1: chronic inflammation of the optimal tage are at increased risk for developing portal tracts and bile duct proliferation the disease. Research studies indicate • Stage 2: chronic inflammation of the outcomes. that the incidence of PSC is lower in non- portal tracts and interface hepatitis (in- smokers and those who drink coffee. flammation of the liver from destruction of hepatocytes between the parenchyma Signs and symptoms and the portal tracts) Most patients with PSC are asymptomatic • Stage 3: fibrosis of the portal tracts with at the time of diagnosis. Lab values fol- bridging necrosis low a similar pattern to PBC. For example, • Stage 4: cirrhosis. ALP is disproportionately elevated to ALT, AST, and GGT, and the bilirubin level is Treatment strategies elevated. Like PBC, patients with PSC may Some research studies support the use of present with fatigue, jaundice, pruritus, UDCA, whereas others don’t. Additional hepatomegaly, and splenomegaly. large studies are needed to further de- There’s a major difference in the way termine the efficacy of this medication in that PBC and PSC are diagnosed. A diag- PSC. Immunosuppression medications, nosis of PSC requires a special imaging including corticosteroids, azathioprine, study called endoscopic retrograde chol- cyclosporine, and MTX, have been used to angiopancreatography (ERCP) in which slow the progression of PSC with minimal a lighted endoscope is inserted into the success. Some research studies suggest patient’s mouth and through the esopha- that oral vancomycin may improve the gus, stomach, and small intestine. The patient’s signs and symptoms and liver ampulla of Vater—a small opening in the chemistries, especially in the pediatric small intestine that provides access to the population. Balloon dilation during ERCP extrahepatic bile ducts—is identified. A may be helpful to reduce bile duct stric- very small guide wire is inserted through tures and remove gallstones to improve the endoscope into the ducts. A contrast the patient’s symptoms. Pruritus is man- medium is injected through the endoscope aged by using the same medications that and images are obtained to determine if are used in PBC. www.NursingMadeIncrediblyEasy.com January/February 2019 Nursing made Incredibly Easy! 45

• Assess for osteoporosis due • Assess for symptoms related to • Assess ALT, AST, ALP, and to malabsorption of vitamin D; malabsorption of vitamins A, D, immunoglobulins. calcium supplements should be E, and K. • Priority assessments and teaching are prescribed. • Assess for symptoms of infection, related to medication therapy to induce • Assess for visual problems at including fever, elevated WBC and maintain remission. night, neurologic deficits, and count, erythrocyte sedimentation Prednisone bleeding tendencies due to rate, and C-reactive protein related • Assess for bleeding and infection. malabsorption of vitamins A, to bacterial cholangitis. • Assess complete blood cell (CBC) count, E, and K; vitamin supplements • Assess pancreatic enzymes, blood glucose, BP, daily weight, and should be prescribed. including lipase and amylase, mental status. • Assess cholesterol levels; because chronic pancreatitis may • Teach the patient to practice good body some research studies coexist with PSC. hygiene and avoid people who are sick. suggest that patients with • Assess for colorectal and biliary • Teach the patient to take medication as hyperlipidemia in PBC are at tract cancer. prescribed. risk for atherosclerosis. • Assess for abdominal pain that • Teach the patient to eat a low-sodium diet. • Assess for abdominal pain that may indicate acute cholecystitis. • Teach the patient to take vitamin D to may indicate acute cholecystitis. • Assess results of MRCP and/or avoid osteoporosis. • Assess ALT, AST, and ALP ERCP that indicate bile strictures • Teach the patient to have regular eye in response to UDCA, MTX, and gallstones. exams. and colchicine therapy, and • Assess ALT, AST, and ALP in Azathioprine correlate with the patient’s signs response to UDCA therapy and • Assess for bleeding and infection. and symptoms. correlate with the patient’s signs • Assess CBC count, ALT, AST, ALP, • Assess the patient’s responses and symptoms. albumin, and proteins. to medications and other • Assess the patient’s responses • Assess for enlarged lymph nodes; comfort measures to treat to medications and other comfort azathioprine places the patient at risk intense pruritus. measures to treat intense pruritus. for lymphoma. • Provide the patient with good • Teach the patient to practice good body oral hygiene, lubricating oral hygiene and avoid people who are sick. solutions, and eye drops due to • Teach the patient to report any skin dry mucous membranes (Sicca lesions; azathioprine places the patient at syndrome). risk for skin cancer.

Mortality in PSC may be as high Pathophysiology as 30% at 5 years. Medical treatment The immune system loses tolerance and and medications don’t conclusively causes dysregulation in T and B lym- slow the progression of the disease. phocytes, leading to inflammation and The best option for patients with PSC destruction of hepatocytes by cytotoxic T is liver transplantation. The 5-year lymphocytes. AIH is sometimes referred survival rate after transplantation is to as a T-cell disease. Antibodies are favorable at 85%. produced in response to immunologic intolerance and considered markers in Autoimmune hepatitis AIH, although they aren’t specific to the AIH is a chronic inflammatory autoim- disease and don’t cause it. AIH is classi- mune disease that causes damage to the fied based on these antibodies. liver because the liver is being attacked Genetically linked to HLA-DR3 and by the immune system. HLA-DR4, type 1 AIH, or classic AIH,

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Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved. constitutes 80% to 90% of cases and biliary tract disease, such as PBC and includes ANA, SMA, or both. Genetically PSC. Immunoglobulins are also elevated linked to HLA-DR4, type 2 AIH occurs in AIH. when patients have anti-liver kidney In AIH, an elevation of liver enzymes microsomal antibody (anti-LKM-1), anti- indicates that the immune system is liver-soluble cytosol antigen (anti-ACL-1), attacking the liver. Declining serum albu- Support or both. Type 2 AIH is more common in min and proteins, prolonged prothrombin Europe and typically affects girls and time, and elevated serum bilirubin indi- groups are young women. cate that liver failure is occurring. Differ- helpful to Patients with AIH may also have other ential diagnoses must also be excluded as patients antibodies, including ANCA and asialo- a cause of elevated liver enzymes, includ- glycoprotein receptor antibodies. Some ing viral hepatitis, drug-induced liver and families patients may be antibody negative and injury, alcohol-induced liver injury, liver coping with still have the signs, symptoms, and histo- tumor, heart disease, and ischemic injury the chronicity logic features of the disease. Patients may to the heart. or may not be symptomatic at the onset of An urgent liver biopsy should be of AILD. the disease. However, some patients may performed to determine the extent of present with decompensated cirrhosis inflammation and fibrosis. Interface hepa- with ascites, encephalopathy, and bleed- titis and bridging necrosis are defining ing from esophageal varices. features of AIH, and there are portal tract lymphocytic cells and an abundance of Epidemiology and risk factors plasma cells. These cells cross the connec- Approximately 1 to 2 in 100,000 indi- tive tissue surrounding the portal tracts viduals are diagnosed with AIH each (called the limiting plate) and invade the year. The prevalence of the disease is surrounding parenchyma. Hepatocytes approximately 24 people in 100,000; 90% become engulfed in these inflammatory of patients are female and 10% are male. cells and undergo a process of cell death Patients with coexisting autoimmune dis- called apoptosis. eases, such as thyroiditis, diabetes mel- Histologic changes suggestive of bili- litus, rheumatoid arthritis, and systemic ary tract disease, such as granulomas, lupus erythematosus, are at increased aren’t usually present. The degree of risk for AIH. Research indicates that vari- fibrosis after intense inflammation in AIH ables in genetically predisposed individu- is classified in the following manner from als trigger an immune system dysregula- the results of the liver biopsy: tion and attack on the liver, which may • Stage 0: no fibrosis include infections and medications, such • Stage 1: mild fibrosis as minocycline, methyldopa, and possibly • Stage 2: moderate fibrosis herbs. • Stage 3: severe fibrosis (precirrhosis) • Stage 4: cirrhosis. Signs and symptoms Research indicates that the prognosis Patients are likely to have fatigue, mal- in AIH is favorable when fibrosis hasn’t aise, anorexia, jaundice, abdominal dis- extended to the cirrhosis stage and imme- comfort, and a low-grade fever. A lab diate immunosuppression therapy is evaluation indicates that the ALT and instituted. AST are significantly elevated at least 10 times the upper level of normal out of Treatment strategies proportion to ALP, which may be normal The priority goal in AIH is to stop the or mildly elevated. A mildly elevated immune attack on the liver through im- or near-normal ALP generally excludes munosuppression therapy. The induction www.NursingMadeIncrediblyEasy.com January/February 2019 Nursing made Incredibly Easy! 47

Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved. of remission is achieved by administering Assess the patient’s mental status and corticosteroids, primarily prednisone at degree of joint pain, abdominal pain, or 40 to 60 mg/day with a subsequent taper fatigue. Pay close attention to skin symp- as liver enzymes dramatically decrease toms. The patient may have jaundice over weeks to months after starting manifested by a yellow discoloration of therapy. Budesonide is another corticoste- the sclera and skin, with pale oral mucous Providing the roid that may be considered. A standard membranes. Correlate jaundice with an patient with approach is to combine prednisone with elevated bilirubin level and pruritus, which the steroid-sparing immunosuppression may be a major source of discomfort, emotional agent azathioprine to induce remission, especially for a patient with PBC or PSC. support is which is maintained with either a low- In addition to medication administration, crucial dose of prednisone or azathioprine. Re- pruritus can be managed by bathing in search indicates that azathioprine alone is cool, tepid water; taking a colloidal oatmeal following a highly effective in maintaining remission. bath; and applying moisturizers to the skin. diagnosis of Other immunosuppression agents may A high bilirubin level will also cause the AILD. be considered in the treatment plan, includ- patient’s stool to be a pale color. ing mycophenolate mofetil, cyclosporine, Assess liver enzymes because an eleva- tacrolimus, and rituximab. Remission is tion indicates that the hepatobiliary system achieved when there’s a normalization is under attack by the patient’s immune of liver enzymes and immunoglobulins, system. For example, a markedly elevated improvement in the patient’s symptoms, ALP out of proportion to ALT and AST may and histologic improvement in the patient’s point to biliary pathology in PBC and PSC. liver. Immunosuppression can reduce and Conversely, a markedly elevated ALT and reverse the fibrotic process in AIH. A repeat AST out of proportion to ALP may point to liver biopsy should be explored. AIH. Liver enzymes should be correlated Approximately 80% of patients achieve with the presence of antibodies as indicated; full remission in 18 to 24 months after start- findings from a liver biopsy; and the results ing immunosuppression therapy. Even from liver function tests, such as albumin, patients with cirrhosis may experience some proteins, and prothrombin time. Decreasing histologic improvement. Approximately serum albumin and proteins, along with a 10% of patients achieve partial remission prolonged prothrombin time, may indicate and 10% fail to respond to therapy and decompensated cirrhosis and that liver fail- progress to ESLD requiring liver trans- ure with portal hypertension is underway. plantation. The relapse rate is 80% when Assess for easy bruising and bleed- immunosuppression therapy is stopped. It’s ing, ascites, and peripheral edema. Also crucial for patients to be adherent to medi- assess the patient’s weight and abdominal cation therapy and report any adverse reac- girth daily. Patients are generally placed tions to the healthcare provider. on a low-protein or no-protein diet when they have ascites. Sodium intake is also Nursing care reduced. Potassium-sparing and loop The nurse should have a general under- diuretics may be prescribed to manage standing of AILD features (see Selected fluid volume excess. Fluid restriction is features of AILD). Nursing care begins with often necessary. Cardiopulmonary status establishing a caring rapport with the pa- should be assessed for fluid volume over- tient. Providing the patient with emotional load. Monitor serum creatinine because support is crucial following a diagnosis of liver failure often leads to renal failure. AILD. Correlate the patient’s health his- Patients should be adherent to medica- tory, head-to-toe assessment, and signs tion therapy; report medication adverse and symptoms to develop a care plan. reactions to healthcare providers; avoid

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PBC PSC AIH

• Mostly affects female patients • Mostly affects male patients • Mostly affects female patients • History of autoimmune • History of autoimmune disease is a risk • History of autoimmune disease is a risk disease is a risk factor factor factor • Associated with AMA and/ • Most patients have IBD • Associated with SMA, ANA, anti-LKM-1, or ANA; it’s possible to have • Not associated with specific antibodies and anti-ALC-1; it’s possible to have antibody-negative PBC • ALP disproportionately elevated to ALT antibody-negative AIH • ALP disproportionately and AST • ALT and AST elevated at least 10 times elevated to ALT and AST • Damage to intrahepatic and extrahepatic the upper level of normal; ALP mildly • Damage only to the bile ducts elevated intrahepatic bile ducts • Associated with an onion skin tissue • Interface hepatitis with prominent • Associated with a florid duct pattern by liver biopsy lymphocytic and plasma cells; bile ducts tissue pattern by liver biopsy • Fatigue, jaundice, abdominal pain, and are generally spared • Fatigue, jaundice, abdominal joint pain • Fatigue, jaundice, pruritus, abdominal pain, joint pain, dry mouth • Pruritus is a major source of discomfort discomfort, and joint pain and eyes, xanthelasma, and • UDCA and other medications and • Rapid reduction in ALT and AST in xanthoma treatments haven’t conclusively been response to prednisone • Pruritus is a major source of proven to slow disease progression • Remission maintained through prednisone discomfort • ERCP with balloon dilation may and/or azathioprine • The use of UDCA is reduce bile duct strictures and remove • 80% of patients achieve remission within associated with an improved gallstones to improve the patient’s 18 to 24 months of the start of therapy; disease course and prognosis symptoms some patients take longer in the early stages • Risk of chronic pancreatitis, colorectal • 80% of patients relapse within 6 months • Liver transplantation for ESLD cancer, and cancer of the bile ducts of stopping immunosuppression therapy • May overlap with AIH • Liver transplantation is the best option • Liver transplantation for ESLD • May overlap with AIH • May overlap with PBC or PSC taking hepatotoxic medications such as Berzigotti A. Advances and challenges in cirrhosis and portal hypertension. BMC Med. 2017;15(1):200. acetaminophen, alcohol, and smoking; and Brennan F. The pathophysiology of pruritus: a review for get plenty of rest. Patients receiving immu- clinicians. Prog Palliat Care. 2016;24(3):133-146. nosuppression medications should receive Bunnett NW. Neuro-humoral signalling by bile acids and the TGR5 receptor in the gastrointestinal tract. J Physiol. influenza and pneumonia vaccines because 2014;592(14):2943-2950. they’re at increased risk for infection. Clinical Advisor. Primary biliary cholangitis: a clinical prac- Support groups are helpful to patients tice guideline. 2017. www.clinicaladvisor.com/hepatology- information-center/clinical-practice-guidelines-for-pbc/ and families coping with the chronicity of article/664410. AILD. Cropley A, Weltman M. The use of immunosuppression in autoimmune hepatitis: a current literature review. Clin Mol Hepatol. 2017;23(1):22-26. Partners for positive outcomes Curry MP, Chopra S. Chronic non-viral hepatitis. In: Early recognition and prompt treatment Greenberger NJ, Blumberg RS, Burakoff R, eds. Current Diagnosis and Treatment: Gastroenterology, Hepatology, and are crucial in AILD. Nursing assessment Endoscopy. 3rd ed. New York, NY: McGraw-Hill; 2016: and patient adherence are integral to this 508-517. Dyson J, Jones D. Diagnosis and management of patients process. The nurse and patient become with primary biliary cirrhosis. Clin Liver Dis. 2014;3(3): partners in the care plan to achieve opti- 52-55. mal outcomes. ■ European Association for the Study of the Liver. EASL clinical practice guidelines: autoimmune hepatitis. REFERENCES J Hepatol. 2015;63(4):971-1004. BC Centre for Palliative Care. BC inter-professional pal- European Association for the Study of the Liver. EASL liative symptom management guidelines. www.bc-cpc. clinical practice guidelines: the diagnosis and man- ca/cpc/wp-content/uploads/2017/11/04.-SMG-Clinical- agement of patients with primary biliary cholangitis. Best-Practices-print-BW-pruritus.pdf. J Hepatol. 2017;67(1):145-172. www.NursingMadeIncrediblyEasy.com January/February 2019 Nursing made Incredibly Easy! 49

Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved. Ferguson LA. Autoimmune hepatitis: a noninfectious featuring a women’s health perspective. J Clin Transl killer. J Am Assoc Nurse Pract. 2014;26(1):13-18. Hepatol. 2014;2(4):266-284. Gill S. Autoimmune hepatitis: clinical overview and path- Pereira MP, Steinke S, Bruland P, et al. Management of ological findings. N Am J Med Sci. 2018;11(1):6-10. chronic pruritus: from the dermatological office to the specialized itch center: a review. Itch. 2017;2(2):e6. Greenberger NJ, Saltzman JR. Primary sclerosing cholangitis. In: Greenberger NJ, Blumberg RS, Burakoff R, Pratt DS. Primary biliary cirrhosis. In: Greenberger NJ, eds. Current Diagnosis and Treatment: Gastroenterology, Blumberg RS, Burakoff R, eds. Current Diagnosis and Hepatology, and Endoscopy. 3rd ed. New York, NY: Treatment: Gastroenterology, Hepatology, and Endoscopy. 3rd McGraw-Hill; 2016:610-614. ed. New York, NY: McGraw-Hill; 2016:605-609. Hirschfield GM, Dyson JK, Alexander GJM, et al. The Sirpal S, Chandok N. Primary sclerosing cholangi- British Society of Gastroenterology/UK-PBC primary tis: diagnostic and management challenges. Clin Exp biliary cholangitis treatment and management guidelines. Gastroenterol. 2017;10:265-273. Gut. 2018;67(9):1568-1594. Song J, Xian D, Yang L, Xiong X, Lai R, Zhong J. Pruritus: Hirschfield GM, Heathcote EJ, eds. Autoimmune Hepatitis: progress toward pathogenesis and treatment. Biomed Res A Guide for Practicing Clinicians. New York, NY: Humana Int. 2018;2018:9625936. Press; 2012. Tanaka A, Takikawa H, Mochida S, Koike K, Miwa H, Invernizzi P, Floreani A, Carbone M, et al. Primary biliary Shimosegawa T. Changing nomenclature for PBC from cholangitis: advances in management and treatment of “primary biliary cirrhosis” to “primary biliary cholangi- the disease. Dig Liver Dis. 2017;49(8):841-846. tis”. J Gastroenterol. 2016;51(7):748-749. Iwakiri Y. Pathophysiology of portal hypertension. Clin Terziroli Beretta-Piccoli B, Mieli-Vergani G, Vergani D. Liver Dis. 2014;18(2):281-291. Autoimmune hepatitis: standard treatment and system- atic review of alternative treatments. World J Gastroenterol. Karlsen TH, Folseraas T, Thorburn D, Vesterhus M. 2017;23(33):6030-6048. Primary sclerosing cholangitis—a comprehensive review. J Hepatol. 2017;67(6):1298-1323. Trivedi HD, Lizaola B, Tapper EB, Bonder A. Management of pruritus in primary biliary cholangitis: a narrative Levy C, Carrion AF, Mayo MJ. Primary biliary cholangi- review. Am J Med. 2017;130(6):744.e1-744.e7. tis. In: Schiff ER, Maddrey WC, Reddy KR, eds. Schiff’s Diseases of the Liver. 12th ed. Oxford, UK: Wiley-Blackwell; Vierling JM. Primary sclerosing cholangitis. In: Schiff ER, 2018:523-545. Maddrey WC, Reddy KR, eds. Schiff’s Diseases of the Liver. 12th ed. Oxford, UK: Wiley-Blackwell; 2018:491-522. Lindor KD, Kowdley KV, Harrison ME. ACG clinical guideline: primary sclerosing cholangitis. Am J At Texas Tech University Health Sciences Center School of Nursing Gastroenterol. 2015;110(5):646-659. in Lubbock, Tex., Richard L. Pullen, Jr., is a Professor and a Nursing made Incredibly Easy! Editorial Board Member and Patricia Francis- Lohse AW, Weiler-Normann C. Autoimmune hepatitis. In: Johnson is an Assistant Professor and Director of Diversity and Sanyal J, Boyer TD, Lindor KD, Terrault NA, eds. Zakim Inclusion. & Boyer’s Hepatology: A Textbook of Liver Disease. 7th ed. Philadelphia, PA: Elsevier; 2018:594-609. The authors and planners have disclosed no potential conflicts of interest, financial or otherwise. Marchioni Beery RM, Vaziri H, Forouhar F. Primary biliary cirrhosis and primary sclerosing cholangitis: a review DOI-10.1097/01.NME.0000549616.82525.47

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