Hepatitis-Autoimmune
Adult-Gerontology Acute Care Practice Guidelines
Definition
A.Chronic: Clinical or biochemical evidence of liver disease greater than 6 months duration.
B.Chronic inflammatory condition of the liver characterized by transaminase elevation in the presence of autoantibodies.
Incidence
A.The exact prevalence in the United States is unknown but is estimated to be 1.1 per 100,000 persons per year.
B.Female predominance but occurs in both genders and all age groups.
Pathogenesis
A.Thought to result from an environmental trigger in a genetically predisposed individual.
Predisposing Factors
A.Strong genetic association with other autoimmune diseases from 26% to 49%.
Subjective Data
A.Common complaints/symptoms.
1.Presentation can vary from asymptomatic (up to 25%) to fulminant hepatic failure (rare).
2.May see anorexia, arthralgia, rash, or fatigue.
B.Common/typical scenario.
1.Insidious onset with constitutional symptoms as listed earlier.
2.Asymptomatic patients will be diagnosed incidentally with laboratory testing.
C.Family and social history.
1.Not applicable.
D.Review of systems.
1.Flu-like symptoms.
a.Nausea.
b.Anorexia.
c.Fatigue.
d.Lethargy.
e.Malaise.
f.Abdominal pain.
g.Itching.
h.Arthralgia.
Physical Examination
A.Ranges from a normal physical examination to evidence of advanced disease, including:
1.Hepatomegaly.
2.Splenomegaly.
3.Jaundice.
4.Temporal wasting.
5.Spider angiomata.
Diagnostic Tests
A.Lab workup includes: Complete blood count (CBC), comprehensive metabolic panel (CMP), international normalized ratio (INR), hepatitis B and C serologies, antinuclear antibody (ANA), AMA, ASMA, LKM1, quantitative immunoglobulins, ceruloplasmin (Wilson’s disease), iron, ferritin (hemochromatosis), Alpha 1 antitrypsin.
B.Lab abnormalities include: Elevated aspartate aminotransferase (AST)/alanine aminotransferase (ALT) 5 to 10X upper limit, elevated immunoglobin G (IMG), positive ANA, ASMA, or LKM1.
C.Imaging: Abdominal ultrasound (US), MRI/magnetic resonance cholangiopancreatography (MRCP) to rule out other biliary disorders.
D.Liver biopsy: Gold standard to confirm diagnosis and severity of disease.
Differential Diagnosis
A.Hepatitis A, B, and C.
B.Primary biliary cholangitis (PBC).
C.Primary sclerosing cholangitis (PSC).
D.Wilson’s disease.
E.Hemochromatosis.
F.Alpha 1 antitrypsin deficiency.
G.Fatty liver disease.
H.Drug-induced liver injury.
I.Alcohol use.
Evaluation and Management Plan
A.General plan.
1.Decision to treat a patient is based on the severity of symptoms, magnitude of AST/ALT elevations, histologic findings, and the potential for side effects.
2.Supportive care.
3.Monitor liver function tests.
4.Glucocorticoid mono therapy or in combination with azathioprine.
B.Patient/family teaching points.
1.Avoid alcohol or over-the-counter (OTC) supplements.
2.Discuss side effect management of steroid therapy.
3.Discuss importance of steroid tapering schedule; do not stop abruptly.
C.Pharmacotherapy.
1.Glucocorticoids: Goal of therapy is to achieve quick remission and taper off steroids to minimize potential side effects.
a.Moderate–severe activity: Prednisolone IV or oral prednisone starting at 60 mg daily.
b.Mild activity: Lower dose of prednisone starting at 20 mg daily.
2.Nonsteroidal immunosuppressant: Imuran or 6 Mercaptopurine (6MP) in combination with steroids that are continued long term after steroid taper. This may also be appropriate for populations that are steroid sensitive. Thiopurine methyltransferase (TPMT) should be obtained prior to starting Imuran or 6MP. It measures the enzyme that breaks down azathioprine and helps guide dosing and risk for potential side effects.
D.Discharge instructions.
1.Teaching points as listed earlier.
2.Avoid alcohol or OTC supplements.
3.Discuss side effect management of steroid therapy.
4.Discuss importance of steroid tapering schedule; do not stop abruptly.
5.Weekly CBC, glucose, and liver tests.
6.Follow-up with gastroenterology or hepatologist.
Follow-Up
A.Get baseline bone density scan.
B.Follow-up with pneumocystis pneumonia (PCP) for lab monitoring.
C.Follow-up with gastroenterologist or hepatologist within 1 month.
Consultation/Referral
A.Refer to transplant center; 10%– to 20% of patients with autoimmune hepatitis will require a liver transplant for acute liver failure, decompensated cirrhosis, and hepatocellular carcinoma.
Special/Geriatric Considerations
A.Patients at increased risk for glucocorticoid side effects.
1.Prediabetes, diabetes.
2.Osteoporosis.
3.Emotional liability.
4.Sleep disturbance.
Bibliography
Fialho, A., Fialho, A., & Carey, W. (2015, July). Autoimmune hepatitis. Retrieved from http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/hepatology/chronic-autoimmune-hepatitis/
Heneghan, M. A. (2018, November 14). Autoimmune hepatitis: Treatment. In K. M. Robson (Ed.), UpToDate. Retrieved from https://www.uptodate.com/contents/autoimmune-hepatitis-treatment