Autoimmune Hepatitis: A Case of Severe Hepatic Inflammation and Response to Immunosuppressive Therapy

Claudio Bonzano^*
*Correspondence: Claudio Bonzano, Department of Molecular and Cellular Biology, Wroclaw Medical University, Poland, Email:

Introduction

Autoimmune Hepatitis (AIH) is a chronic, progressive liver disease characterized by an immune system attack on the liver, leading to inflammation, liver cell damage, and, if untreated, potential cirrhosis and liver failure. Unlike other forms of hepatitis caused by viral infections or toxins, autoimmune hepatitis occurs when the body's immune system mistakenly targets its own liver cells. The exact cause of this abnormal immune response remains unclear, but genetic predisposition, environmental factors (such as viral infections or medications), and alterations in immune regulation are believed to play key roles in its development. AIH is often categorized into two types: Type 1 and Type 2, with Type 1 being the most common globally. Type 1 typically affects adults, particularly women, and is associated with the presence of specific autoantibodies such as Antinuclear Antibodies (ANA) and Smooth Muscle Antibodies (SMA). Type 2, although rarer, primarily affects children and is linked with antibodies against Liver Kidney Microsomal Antigen (LKM-1). The clinical presentation of AIH can vary widely, ranging from mild symptoms (such as fatigue and mild abdominal discomfort) to more severe manifestations, including jaundice, ascites, and liver failure. Many patients are asymptomatic at the time of diagnosis, while others may present with symptoms resembling acute viral hepatitis or other liver diseases. Because of the nonspecific nature of its symptoms, AIH can often be misdiagnosed, delaying treatment and increasing the risk of irreversible liver damage. Diagnosing autoimmune hepatitis typically involves a combination of clinical evaluation, laboratory testing (including liver function tests, autoimmune markers, and imaging studies), and liver biopsy, which may be necessary to confirm the diagnosis and assess the extent of liver damage. Early detection and appropriate treatment, typically with immunosuppressive medications like corticosteroids and azathioprine, are crucial for controlling inflammation, preventing progression to cirrhosis, and improving long-term outcomes. This condition underscores the complex interplay between the immune system and liver function, and highlights the importance of understanding autoimmune diseases in clinical practice. With prompt treatment, many patients with autoimmune hepatitis can achieve remission and live relatively normal lives, though lifelong monitoring is often necessary due to the chronic nature of the disease. [1]

Description

A 32-year-old woman with no significant past medical history presents to the emergency department with a three-week history of progressively worsening fatigue, nausea, anorexia, and Right Upper Quadrant (RUQ) abdominal pain. She also reports the recent development of jaundice, darkcolored urine, and pale stools. Approximately six weeks prior to presentation, the patient experienced a viral-like illness, characterized by low-grade fever and sore throat, which she now believes could have been an episode of viral hepatitis. Although she did not seek medical attention for these symptoms, she managed them with over-the-counter pain relievers for body aches. She denies alcohol use, intravenous drug use, and has no known history of taking over-the-counter medications or herbal supplements. Her family history is notable for autoimmune diseases, with a mother diagnosed with rheumatoid arthritis and a sister with Hashimotoâ??s thyroiditis. Upon arrival at the emergency department, the patient appears visibly fatigued and jaundiced, with yellowing of both the sclera and skin. On physical examination, she is alert but displays signs of discomfort due to RUQ tenderness. No hepatomegaly is noted on palpation, but the liver edge is felt about 2 cm below the costal margin, suggesting mild hepatomegaly. There are no signs of encephalopathy, ascites, or peripheral edema. Vital signs are stable, with a blood pressure of 118/75 mmHg, heart rate of 90 beats per minute, respiratory rate of 18 breaths per minute, and temperature of 37â??. The patient's clinical presentation, with jaundice, RUQ pain, and fatigue, along with her family history of autoimmune diseases, raises concern for a possible liver pathology [2]. Laboratory results reveal significant abnormalities that suggest liver dysfunction. The patient's bilirubin levels are markedly elevated, indicating significant liver injury or cholestasis. Liver enzyme tests show substantial increases in AST and ALT, reflecting hepatocellular damage. Both alkaline phosphatase and gamma-glutamyl transferase are also elevated, which suggests some degree of cholestasis. Additionally, autoimmune testing is positive for Antinuclear Antibodies (ANA) and Smooth Muscle Antibodies (SMA), both of which are commonly associated with Autoimmune Hepatitis (AIH). The patient's Immunoglobulin G (IgG) levels are notably elevated, further supporting the diagnosis of autoimmune hepatitis. Given the elevated liver enzymes, positive autoimmune markers, and clinical presentation, Autoimmune Hepatitis (AIH) is strongly suspected. The next step in confirming this diagnosis is to conduct a liver biopsy. The liver biopsy reveals histological features characteristic of severe autoimmune hepatitis. These findings include marked periportal inflammation, interface hepatitis (inflammation at the interface between the portal tract and liver parenchyma), rosette formation of hepatocytes, and plasma cell infiltration. These histological features are hallmarks of autoimmune-mediated liver injury, which further supports the diagnosis of autoimmune hepatitis. Further autoimmune testing confirms the presence of Anti-Smooth Muscle Antibodies (SMA) and elevated IgG, which are typical of Type 1 autoimmune hepatitis [3]. A viral hepatitis panel, including tests for hepatitis A, B, and C, is negative, ruling out viral hepatitis as the cause of the liver inflammation. An abdominal ultrasound is performed, which shows mild hepatomegaly but no signs of cirrhosis or significant liver fibrosis, suggesting that while liver inflammation is significant, there is no evidence of advanced liver damage such as cirrhosis. With the diagnosis of severe autoimmune hepatitis established, the treatment plan is initiated. High-dose prednisone is started as first-line therapy to reduce the immune-mediated inflammation in the liver and prevent further liver damage. Prednisone is chosen because it can quickly alleviate inflammation and reduce symptoms such as fatigue, jaundice, and pain. After one week of prednisone therapy, the patient begins to show some improvement, but to minimize the long-term side effects of corticosteroid use (such as weight gain, hyperglycemia, and osteoporosis), azathioprine is added as a steroid-sparing agent. Azathioprine helps maintain remission and prevents relapse while allowing for the gradual tapering of prednisone.The patient is closely monitored for potential side effects of the therapy, which include leukopenia, thrombocytopenia, hyperglycemia, and weight gain from the prednisone, as well as gastrointestinal symptoms such as nausea and vomiting from the azathioprine. Blood tests, including Complete Blood Count (CBC) and Liver Function Tests (LFTs), are ordered regularly to track the effects of therapy and assess for any adverse reactions. The patient is scheduled for follow-up visits to monitor her progress [4]. After two weeks of treatment, the patient begins to show signs of clinical improvement. Her fatigue gradually diminishes, and her jaundice starts to resolve. Repeat liver function tests show a marked decrease in both transaminases and bilirubin levels indicating a significant reduction in liver inflammation. She reports improved appetite and a reduction in her abdominal discomfort. Six months after the initiation of therapy, the patient is in stable remission. Her liver enzymes have normalized, and follow-up imaging shows no further progression of fibrosis. The prednisone dose is gradually tapered, and azathioprine is continued at a lower dose for maintenance therapy. The patient is advised on the importance of regular follow-up visits to monitor her liver function and autoimmune markers, with the goal of preventing relapse and further liver damage. This case highlights the importance of early recognition and treatment of autoimmune hepatitis, which can present with a wide range of symptoms, from mild and nonspecific to severe, as in this patient. Autoimmune hepatitis can lead to acute liver failure if not properly diagnosed and treated. The patientâ??s family history of autoimmune diseases, along with her clinical presentation and laboratory findings, made autoimmune hepatitis a leading diagnosis. Prompt and appropriate treatment with immunosuppressive therapy, including prednisone and azathioprine, is critical in managing the condition. Prednisone provides rapid relief of inflammation, while azathioprine helps maintain long-term remission while reducing steroid dependency. The case also underscores the importance of liver biopsy in confirming the diagnosis of autoimmune hepatitis and assessing the degree of liver injury. The biopsy findings, including interface hepatitis, rosette formation of hepatocytes, and plasma cell infiltration, are key features that differentiate autoimmune hepatitis from other forms of liver disease. Additionally, regular follow-up and monitoring of liver function and autoimmune markers are crucial to ensure the long-term management of the condition, as well as to prevent relapse and complications such as cirrhosis. The successful management of this patientâ??s autoimmune hepatitis emphasizes the role of early intervention and the appropriate use of immunosuppressive therapy in preventing liver damage and improving patient outcomes [5].

Conclusion

Autoimmune hepatitis is a serious condition that can present with nonspecific symptoms, making diagnosis challenging. However, when recognized early, effective immunosuppressive therapy can lead to significant clinical improvement and prevent long-term complications such as cirrhosis. This case underscores the importance of a comprehensive diagnostic approach, including clinical evaluation, laboratory tests, and liver biopsy, to ensure timely and appropriate management. With prompt treatment and regular follow-up, patients with autoimmune hepatitis can achieve good longterm outcomes, though lifelong monitoring is often required.

References

1. Mack, Cara L., David Adams, David N. Assis and Nanda Kerkar, et al. "Diagnosis and management of autoimmune hepatitis in adults and children: 2019 practice guidance and guidelines from the American Association for the Study of Liver Diseases." Hepatology 72 (2020): 671-722.

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2. Strettell, M. D., PETER T. Donaldson, LINDA J. Thomson and PAULA J. Santrach, et al. "Allelic basis for HLA-encoded susceptibility to type 1 autoimmune hepatitis." Gastroenterology 112 (1997): 2028-2035.

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