Autoimmune hepatitis US Medical PG Practice Questions and MCQs
Practice US Medical PG questions for Autoimmune hepatitis. These multiple choice questions (MCQs) cover important concepts and help you prepare for your exams.
Autoimmune hepatitis US Medical PG Question 1: A 75-year-old woman comes to the physician because of a 6-month history of fatigue. During this period, she has had fever, pain in both shoulders and her hips, and a 5-kg (11-lb) weight loss. She also reports feeling stiff for about an hour after waking up. She has a history of hypertension and hypercholesterolemia. There is no family history of serious illness. She has smoked a pack of cigarettes daily for the past 50 years. Her medications include hydrochlorothiazide and atorvastatin. She appears pale. Her temperature is 38°C (100.4°F), pulse is 90/min, and blood pressure is 135/85 mm Hg. Range of motion of the shoulders and hips is reduced due to pain. Examination shows full muscle strength. The remainder of the examination shows no abnormalities. Laboratory studies show an erythrocyte sedimentation rate of 50 mm/h and a C-reactive protein concentration of 25 mg/dL (N=0–10 mg/dL). Which of the following is the most appropriate next step in management?
- A. Chest x-ray
- B. Electromyography
- C. Muscle biopsy
- D. Low-dose of oral prednisone (Correct Answer)
- E. Antibody screening
Autoimmune hepatitis Explanation: ***Low-dose of oral prednisone***
- The patient presents with classic symptoms of **polymyalgia rheumatica (PMR)**, including bilateral shoulder and hip pain, significant morning stiffness (over 1 hour), fatigue, fever, and weight loss, in an elderly individual with elevated **ESR** and **CRP**.
- PMR responds dramatically to **low-dose corticosteroids**, making prednisone the most appropriate first-line treatment.
*Chest x-ray*
- While recommended in patients with unexplained **constitutional symptoms** and **elevated inflammatory markers** to rule out malignancy or infection, it is not the most immediate or specific next step given the clear picture of PMR.
- A chest X-ray should be considered during the initial work-up of PMR if there are **pulmonary symptoms** or suspected **malignancy**, but PMR treatment should not be delayed.
*Electromyography*
- **Electromyography (EMG)** is used to differentiate between **myopathic** and **neuropathic** causes of muscle weakness.
- This patient exhibits **painful limitation of range of motion** rather than true muscle weakness, and her muscle strength is full.
*Muscle biopsy*
- A **muscle biopsy** is useful for diagnosing inflammatory myopathies like **polymyositis** or **dermatomyositis**.
- However, the patient's presentation of pain and stiffness without objective muscle weakness, coupled with the age and inflammatory markers, makes PMR much more likely than an inflammatory myopathy.
*Antibody screening*
- **Autoantibody screening** (e.g., ANA, RF, anti-CCP) is typically performed to evaluate for conditions like **rheumatoid arthritis** or **lupus**.
- While these can cause joint symptoms and fatigue, the characteristic **proximal stiffness** and dramatic inflammatory markers in an elderly patient make PMR the more probable diagnosis, and these tests are usually negative in PMR.
Autoimmune hepatitis US Medical PG Question 2: A 35-year-old woman comes to the physician because of a 3-month history of progressive fatigue, shortness of breath, and pain in her knees and ankles. Her temperature is 37.6°C (99.7°F). Physical examination shows mild hepatomegaly and tender, red nodules on her shins. There are purple, indurated lesions on her nose, nasolabial fold, and cheeks. A biopsy of the liver shows scattered aggregations of multinucleated giant cells with cytoplasmic inclusions and eosinophilic, needle-shaped structures arranged in a star-like pattern. Which of the following is the most likely cause of this patient's symptoms?
- A. Hypereosinophilic syndrome
- B. Hemochromatosis
- C. Sarcoidosis (Correct Answer)
- D. Serum sickness
- E. Systemic lupus erythematosus
Autoimmune hepatitis Explanation: ***Sarcoidosis***
- The constellation of **fatigue**, **dyspnea**, **polyarthritis**, **hepatomegaly**, **erythema nodosum** (tender, red nodules on shins), and facial lesions consistent with **lupus pernio** (purple, indurated lesions on nose, nasolabial fold, and cheeks) strongly points to multisystem involvement characteristic of sarcoidosis.
- The liver biopsy findings of **scattered aggregations of multinucleated giant cells with cytoplasmic inclusions** and **eosinophilic, needle-shaped structures arranged in a star-like pattern** describes **non-caseating granulomas** with **asteroid bodies** and **Schaumann bodies**, which are pathognomonic for sarcoidosis.
*Hypereosinophilic syndrome*
- This syndrome is characterized by **persistent eosinophilia** (>1.5 x 10^9/L for >6 months) and organ damage due to eosinophil infiltration, but the biopsy findings and specific skin lesions presented are not typical.
- While it can affect multiple organs, including the skin and heart, it would not typically present with the described granulomatous features or lupus pernio.
*Hemochromatosis*
- Hemochromatosis is an **iron overload disorder** leading to iron deposition in various organs, causing fatigue, arthralgia, and hepatomegaly, but the skin lesions described and the specific biopsy findings of granulomas are not characteristic.
- The classic skin presentation in hemochromatosis is generally a **bronze pigmentation**, not the nodular lesions or lupus pernio described.
*Serum sickness*
- Serum sickness is a **Type III hypersensitivity reaction** typically manifesting with fever, rash (often urticarial), arthralgia, and lymphadenopathy, usually arising 7-14 days after exposure to certain agents (e.g., antitoxins, medications).
- It does not cause the specific granulomatous liver changes or chronic skin lesions like erythema nodosum or lupus pernio seen in this patient.
*Systemic lupus erythematosus*
- SLE is a **multisystem autoimmune disease** that can present with fatigue, arthralgia, and skin manifestations, but the classical skin lesions are often malar rash or discoid lupus, and the liver biopsy findings of non-caseating granulomas are not typical.
- While lupus can affect the liver, it usually manifests as autoimmune hepatitis or fatty liver, not the specific granulomatous pathology seen here.
Autoimmune hepatitis US Medical PG Question 3: A 59-year-old woman comes to the physician for a routine health maintenance examination. She feels well. She has systemic lupus erythematosus and hypertension. She does not drink alcohol. Her current medications include lisinopril and hydroxychloroquine. She appears malnourished. Her vital signs are within normal limits. Examination shows a soft, nontender abdomen. There is no ascites or hepatosplenomegaly. Serum studies show:
Total bilirubin 1.2 mg/dL
Alkaline phosphatase 60 U/L
Alanine aminotransferase 456 U/L
Aspartate aminotransferase 145 U/L
Hepatitis A IgM antibody negative
Hepatitis A IgG antibody positive
Hepatitis B surface antigen positive
Hepatitis B surface antibody negative
Hepatitis B envelope antigen positive
Hepatitis B envelope antibody negative
Hepatitis B core antigen IgM antibody negative
Hepatitis B core antigen IgG antibody positive
Hepatitis C antibody negative
Which of the following is the most appropriate treatment for this patient?
- A. Pegylated interferon alpha therapy
- B. Tenofovir therapy (Correct Answer)
- C. Referral to a liver transplantation center
- D. Reassurance and follow-up
- E. Lamivudine therapy
Autoimmune hepatitis Explanation: ***Tenofovir therapy***
- This patient has **chronic hepatitis B** with evidence of **active viral replication** (positive HBsAg, HBeAg, and elevated liver enzymes), indicating a need for antiviral treatment.
- **Tenofovir** is a highly effective and well-tolerated oral antiviral agent for chronic hepatitis B, suitable for initial therapy.
*Pegylated interferon alpha therapy*
- While an option for chronic hepatitis B, **pegylated interferon alpha** has more significant side effects and is generally avoided in patients with **systemic lupus erythematosus (SLE)** due to the risk of exacerbating the autoimmune condition.
- It also requires subcutaneous injections and has a lower rate of HBeAg seroconversion compared to nucleos(t)ide analogs in many patient populations.
*Referral to a liver transplantation center*
- This patient currently shows **elevated liver enzymes** but no immediate signs of **decompensated liver disease** (e.g., ascites, encephalopathy, variceal bleeding) or severe liver failure that would warrant urgent transplantation.
- Treatment with antiviral medication is the first step to prevent progression to end-stage liver disease.
*Reassurance and follow-up*
- The patient has **elevated transaminases** and markers of **active viral replication** (positive HBeAg), indicating ongoing liver injury and potential progression to cirrhosis.
- Simply observing the patient without treatment would be inappropriate and could lead to irreversible liver damage.
*Lamivudine therapy*
- **Lamivudine** is an older nucleos(t)ide analog for hepatitis B that has a significantly **higher rate of drug resistance** compared to newer agents like tenofovir.
- It is generally not recommended as a first-line treatment due to its resistance profile.
Autoimmune hepatitis US Medical PG Question 4: A 62-year-old woman with a history of hypertension, hyperlipidemia, and rheumatoid arthritis presents for evaluation of elevated serum liver chemistries. She has had three months of intense, unremitting itching. Current medications include chlorthalidone, atorvastatin, and ibuprofen. Physical exam is unremarkable. Laboratory studies show aspartate aminotransferase (AST) 42 units/L, alanine aminotransferase (ALT) 39 units/L, alkaline phosphatase 790 units/L, total bilirubin 0.8 mg/dL, and antimitochondrial antibody titer 1:80. What do you expect to see on liver biopsy?
- A. Intrahepatic bile duct destruction (Correct Answer)
- B. Bile plugging of hepatocytes and bile ducts
- C. Granulomas in portal tracts
- D. Intrahepatic and extra hepatic bile duct destruction
- E. Lymphoplasmacytic and eosinophilic infiltration of portal tracts
Autoimmune hepatitis Explanation: ***Intrahepatic bile duct destruction***
- The patient's presentation with **pruritus**, significantly **elevated alkaline phosphatase**, relatively normal AST/ALT, and a **positive antimitochondrial antibody (AMA)** strongly suggests **primary biliary cholangitis (PBC)**.
- The hallmark histopathological finding in PBC is the **destruction of small and medium-sized intrahepatic bile ducts** by chronic inflammation.
*Bile plugging of hepatocytes and bile ducts*
- This finding is characteristic of significant **biliary obstruction** (cholestasis), where bile flow is severely impaired, leading to the accumulation of bile within hepatocytes and bile ducts.
- While there is cholestasis in PBC (evidenced by high alkaline phosphatase), the primary injury is immune-mediated destruction of ducts, not simply plugging, and bilirubin is not significantly elevated, indicating less severe obstruction.
*Granulomas in portal tracts*
- While **epithelioid granulomas** can be seen in PBC (in up to 30% of cases), they are not the **defining histologic feature**—the bile duct destruction is.
- Granulomas are also more commonly associated with **sarcoidosis** and certain **drug-induced liver injuries**.
- The constellation of symptoms and labs points directly to classic PBC with its characteristic bile duct destruction.
*Intrahepatic and extra hepatic bile duct destruction*
- **Extrahepatic bile duct destruction** is typical of conditions like **primary sclerosing cholangitis (PSC)**, which primarily affects larger intrahepatic and extrahepatic bile ducts and is often associated with inflammatory bowel disease.
- The positive AMA and specific pattern of liver enzyme elevation are highly suggestive of PBC, which is confined to the small and medium intrahepatic ducts, rather than PSC.
*Lymphoplasmacytic and eosinophilic infiltration of portal tracts*
- **Eosinophilic infiltration** can be prominent in allergic reactions, parasitic infections, or some forms of drug-induced liver injury, but it's not the defining feature of PBC.
- While **lymphoplasmacytic infiltration** is present in PBC, it is specifically directed at and causes destruction of the bile ducts, rather than being a generalized, non-specific infiltration of the portal tracts.
Autoimmune hepatitis US Medical PG Question 5: A 45-year-old man comes to the physician for a routine health maintenance examination. He feels well. He has type 2 diabetes mellitus. There is no family history of serious illness. He works as an engineer at a local company. He does not smoke. He drinks one glass of red wine every other day. He does not use illicit drugs. His only medication is metformin. He is 180 cm (5 ft 11 in) tall and weighs 100 kg (220 lb); BMI is 31 kg/m2. His vital signs are within normal limits. Examination shows a soft, nontender abdomen. The liver is palpated 2 to 3 cm below the right costal margin. Laboratory studies show an aspartate aminotransferase concentration of 100 U/L and an alanine aminotransferase concentration of 130 U/L. Liver biopsy shows hepatocyte ballooning degeneration, as well as inflammatory infiltrates with scattered lymphocytes, neutrophils, and Kupffer cells. Which of the following is the most likely diagnosis?
- A. Nonalcoholic steatohepatitis (Correct Answer)
- B. Autoimmune hepatitis
- C. Viral hepatitis
- D. Primary biliary cholangitis
- E. Alcoholic fatty liver disease
Autoimmune hepatitis Explanation: ***Nonalcoholic steatohepatitis***
- The patient's **obesity (BMI 31)**, **type 2 diabetes mellitus**, and elevated liver enzymes (ALT > AST) in the absence of significant alcohol intake or other causes of liver disease are highly suggestive of **nonalcoholic fatty liver disease (NAFLD)**, with the biopsy findings of **hepatocyte ballooning degeneration** and **inflammatory infiltrates** confirming progression to **nonalcoholic steatohepatitis (NASH)**.
- **NASH** is a severe form of NAFLD characterized by **steatosis**, **inflammation**, and hepatocyte injury (ballooning degeneration), which can progress to cirrhosis and liver failure.
*Autoimmune hepatitis*
- This condition is typically characterized by high levels of **autoantibodies** (e.g., ANA, anti-smooth muscle antibodies), which are not mentioned and would be an important diagnostic clue.
- Although it can cause elevated transaminases and inflammatory infiltrates, the biopsy typically shows **interface hepatitis** and prominent plasma cell infiltrates, rather than significant steatosis and ballooning degeneration.
*Viral hepatitis*
- While viral hepatitis (e.g., hepatitis B or C) causes elevated transaminases and inflammatory changes, the biopsy findings of **hepatocyte ballooning** are not characteristic.
- The patient's presentation does not include risk factors or symptoms typically associated with acute or chronic viral hepatitis, and serological markers would be required for diagnosis.
*Primary biliary cholangitis*
- This is a chronic autoimmune cholestatic liver disease primarily affecting **interlobular bile ducts**, usually seen in middle-aged women.
- It is characterized by elevated **alkaline phosphatase** levels and positive **antimitochondrial antibodies (AMA)**, which are not described in this patient, and the biopsy would show granulomatous destruction of bile ducts.
*Alcoholic fatty liver disease*
- Although the biopsy findings of **steatosis**, **hepatocyte ballooning**, and **inflammation** can be seen in alcoholic liver disease, the patient's reported alcohol consumption of "one glass of red wine every other day" is well below the threshold for causing significant alcoholic liver damage.
- **Alcoholic hepatitis** typically involves an AST:ALT ratio of >2 and a history of heavy, prolonged alcohol use.
Autoimmune hepatitis US Medical PG Question 6: A 42-year-old woman presents complaining of pain in her hands. She reports that the pain is in both hands, and that it is usually worse in the morning. She reports that her hands are also stiff in the morning, but that this gradually improves throughout the morning. She notes, however, that her symptoms seem to be getting worse over the last three months. What is the most likely pathogenesis of her disease process?
- A. Production of antibodies against smooth muscle
- B. Anti-neutrophil cytoplasmic antibody production
- C. Production of antibodies against antibodies (Correct Answer)
- D. Type 1 hypersensitivity reaction
- E. Repetitive microtrauma
Autoimmune hepatitis Explanation: ***Production of antibodies against antibodies***
- The patient's symptoms of **bilateral hand pain and morning stiffness** improving with activity, worsening over three months, are classic for **Rheumatoid Arthritis (RA)**.
- RA is characterized by the production of **rheumatoid factor (RF)**, an antibody (typically IgM) directed against the Fc portion of IgG, which is essentially an antibody against an antibody.
*Production of antibodies against smooth muscle*
- This describes the presence of **anti-smooth muscle antibodies (ASMA)**, which are characteristic of **Autoimmune Hepatitis type 1**.
- Autoimmune hepatitis primarily affects the liver, leading to symptoms like fatigue, jaundice, and elevated liver enzymes, not primarily joint pain.
*Anti-neutrophil cytoplasmic antibody production*
- This refers to **ANCA (anti-neutrophil cytoplasmic antibodies)**, which are associated with various forms of **vasculitis**, such as Granulomatosis with Polyangiitis (Wegener's), Microscopic Polyangiitis, and Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss).
- While vasculitis can cause systemic symptoms, the patient's presentation of symmetric, inflammatory arthritis is not typical for primary ANCA-associated vasculitis.
*Type 1 hypersensitivity reaction*
- A **type I hypersensitivity reaction** involves IgE-mediated mast cell degranulation, leading to immediate allergic reactions like asthma, anaphylaxis, or hives.
- This mechanism is completely unrelated to the pathogenesis of an autoimmune, chronic inflammatory arthritis like Rheumatoid Arthritis.
*Repetitive microtrauma*
- Repetitive microtrauma is more consistent with **osteoarthritis** or **occupational overuse injuries**.
- Osteoarthritis typically presents with pain that worsens with activity and improves with rest, **morning stiffness lasting less than 30 minutes**, and often affects weight-bearing joints or specific joints due to trauma or wear and tear, rather than the inflammatory pattern described.
Autoimmune hepatitis US Medical PG Question 7: A 44-year-old woman presents to the emergency department with jaundice and diffuse abdominal pain. She denies any previous medical problems and says she does not take any medications, drugs, or supplements. Her temperature is 97.6°F (36.4°C), blood pressure is 133/87 mmHg, pulse is 86/min, respirations are 22/min, and oxygen saturation is 100% on room air. Physical exam is notable for sclera which are icteric and there is tenderness to palpation over the right upper quadrant. Laboratory studies are ordered as seen below.
Hepatitis B surface antigen: Positive
Hepatitis B surface antibody: Negative
Hepatitis B core antibody IgM: Negative
Hepatitis B core antibody IgG: Positive
Hepatitis B E antigen: Positive
Hepatitis B E antibody (anti-HBe): Negative
Which of the following is the most likely diagnosis?
- A. Chronic hepatitis B infection (Correct Answer)
- B. Hepatitis B vaccination
- C. Acute hepatitis B infection
- D. Resolved hepatitis B infection
- E. No hepatitis B vaccination or infection
Autoimmune hepatitis Explanation: ### ***Chronic hepatitis B infection***
- The presence of **Hepatitis B surface antigen (HBsAg) positive** combined with **Hepatitis B core antibody IgG (anti-HBc IgG) positive** indicates infection that has persisted beyond 6 months.
- **Hepatitis B core antibody IgM (anti-HBc IgM) negative** rules out acute infection, as IgM antibodies appear early in acute hepatitis B.
- **Hepatitis B e antigen (HBeAg) positive** indicates active viral replication and high infectivity, consistent with HBeAg-positive chronic hepatitis B.
- The clinical presentation with jaundice and RUQ pain suggests an acute flare of chronic hepatitis B infection.
### *Hepatitis B vaccination*
- Successful hepatitis B vaccination produces **anti-HBs positive** with **HBsAg negative** and **anti-HBc negative**.
- This patient has **HBsAg positive** and **anti-HBc IgG positive**, indicating actual infection rather than vaccine-induced immunity.
### *Acute hepatitis B infection*
- Acute hepatitis B is characterized by **HBsAg positive** with **anti-HBc IgM positive** (IgM appears first in acute infection).
- This patient has **anti-HBc IgM negative** and **anti-HBc IgG positive**, indicating the infection occurred more than 6 months ago, consistent with chronic rather than acute infection.
### *Resolved hepatitis B infection*
- Resolved infection shows **HBsAg negative**, **anti-HBs positive**, and **anti-HBc IgG positive**.
- This patient's **HBsAg positive** status directly indicates ongoing infection, not resolution.
### *No hepatitis B vaccination or infection*
- Complete absence of exposure would show **HBsAg negative**, **anti-HBs negative**, and **anti-HBc negative** (all markers negative).
- This patient has multiple positive markers including **HBsAg** and **anti-HBc IgG**, confirming hepatitis B infection.
Autoimmune hepatitis US Medical PG Question 8: A 36-year-old woman presents for a pre-employment health assessment. She has no complaints. Her last annual physical examination 8 months ago was normal. She has no significant past medical history. She is a nonsmoker and says she quit all alcohol consumption last year. A complete hepatic biochemistry panel is performed, which is significant for a serum alkaline phosphatase (ALP) level 5 times the upper limit of the normal range. Immunologic tests are positive for antimitochondrial antibodies. A liver biopsy is performed and reveals an inflammatory infiltrate surrounding the biliary ducts. Which of the following is the most likely diagnosis in this patient?
- A. Pancreatic cancer
- B. Primary biliary cholangitis (Correct Answer)
- C. Hepatic amyloidosis
- D. Choledocolithiasis
- E. Fascioliasis
Autoimmune hepatitis Explanation: ***Primary biliary cholangitis***
- The combination of significantly elevated **alkaline phosphatase** (indicating cholestasis), positive **antimitochondrial antibodies (AMA)**, and an inflammatory infiltrate around the bile ducts on biopsy is highly diagnostic of primary biliary cholangitis (PBC).
- PBC is a **chronic autoimmune liver disease** predominantly affecting middle-aged women, characterized by progressive destruction of small intrahepatic bile ducts.
*Pancreatic cancer*
- Pancreatic cancer typically presents with symptoms like **jaundice**, weight loss, and abdominal pain, none of which are present in this asymptomatic patient.
- While it can cause cholestasis and elevated ALP, it would usually be associated with a **dilated common bile duct** on imaging and often obstructive symptoms.
*Hepatic amyloidosis*
- Hepatic amyloidosis can elevate ALP, but it would not typically be associated with **positive antimitochondrial antibodies**.
- Liver biopsy would show **amyloid deposits** rather than an inflammatory infiltrate surrounding biliary ducts.
*Choledocolithiasis*
- Choledocolithiasis (bile duct stones) would cause an **obstructive pattern** of elevated ALP, but it is often acutely symptomatic with **biliary colic** or cholangitis.
- It would not typically involve positive **antimitochondrial antibodies** or an inflammatory infiltrate characteristic of an autoimmune bile duct disease on biopsy.
*Fascioliasis*
- Fascioliasis is a **parasitic infection** that can cause eosinophilia and liver enzyme elevation, but it is associated with exposure to contaminated watercress or aquatic plants.
- It would not typically present with positive **antimitochondrial antibodies** and the specific inflammatory infiltrate seen in PBC.
Autoimmune hepatitis US Medical PG Question 9: A 32-year-old woman comes to the physician with increasing jaundice and fatigue for the past week. She has no history of a serious illness. She takes no medications and denies use of recreational drugs. She does not drink alcohol. Her vital signs are within normal limits. Her body mass index is 21 kg/m2. On physical examination, she has icteric sclera. Otherwise, her heart and lung sounds are within normal limits.
Hemoglobin 15 g/dL
Leukocyte count 6,000/mm3 with a normal differential
Serum bilirubin
Total 6.5 mg/dL
Direct 0.9 mg/dL
Alkaline phosphatase 70 U/L
Aspartate aminotransferase (AST, GOT) 430 U/L
Alanine aminotransferase (ALT, GPT) 560 U/L
γ-Glutamyltransferase (GGT) 43 U/L (N=5-50 U/L)
Hepatitis A antibody Negative
Hepatitis B surface antigen Negative
Hepatitis C antibody Negative
Rheumatoid factor 80 IU/mL (N=0-20 IU/mL)
Antinuclear antibody (ANA) titer is 1:1280. Polyclonal immunoglobulin gamma is 5 g/dL. Which of the following antibodies is most likely to be positive in this patient?
- A. Anti-cyclic citrullinated peptide
- B. Anti-liver kidney microsomal type 2
- C. Anti-mitochondrial
- D. Anti-smooth muscle (Correct Answer)
- E. Anti-double stranded DNA
Autoimmune hepatitis Explanation: ***Anti-smooth muscle***
- This patient's presentation with **jaundice**, elevated **transaminases**, high **ANA titer (1:1280)**, and increased **polyclonal immunoglobulin gamma** is highly suggestive of **autoimmune hepatitis (AIH) type 1**.
- **Anti-smooth muscle antibodies (ASMA)** are the hallmark and most frequently associated autoantibody with **AIH type 1**, found in 70-80% of cases.
*Anti-cyclic citrullinated peptide*
- These antibodies are highly specific for **rheumatoid arthritis**, a systemic inflammatory condition primarily affecting joints.
- While the patient has an elevated **rheumatoid factor**, her primary symptoms and liver enzyme abnormalities do not point to rheumatoid arthritis.
*Anti-liver kidney microsomal type 2*
- These antibodies are characteristic of **autoimmune hepatitis type 2**, which typically affects children and young adults and is associated with different HLA haplotypes.
- While AIH type 2 involves liver inflammation, the extremely high ANA titer and significantly elevated polyclonal immunoglobulins are more typical of **AIH type 1**.
*Anti-mitochondrial*
- These antibodies are the serological hallmark of **primary biliary cholangitis (PBC)**, a chronic cholestatic liver disease primarily affecting interlobular bile ducts.
- The patient's liver enzyme profile shows predominantly **hepatocellular injury** (markedly elevated AST/ALT) rather than cholestasis (normal alkaline phosphatase and GGT).
*Anti-double stranded DNA*
- These antibodies are highly specific for **systemic lupus erythematosus (SLE)**, a systemic autoimmune disease affecting multiple organ systems.
- Although SLE can cause liver involvement, the prominent liver enzyme elevations and the specific pattern of serological markers are more indicative of **autoimmune hepatitis**.
Autoimmune hepatitis US Medical PG Question 10: A 47-year-old Caucasian woman presents with a 2-month history of general fatigue, slight jaundice, and mild itching. She has also noticed that her urine has been darker and stools have been lighter in color recently. She denies any fevers, chills, or alcohol use. She has no significant past medical or surgical history and is not taking any medications. She recalls that her mother saw a doctor for eye and mouth dryness but cannot remember the name of her diagnosis. She denies any illicit drug use, recent change in diet, or recent travel. On physical exam, her abdomen is soft and non-distended. There is right upper quadrant tenderness to deep palpation but a negative Murphy’s sign. Her laboratory findings were significant for increased liver enzymes, direct bilirubin, and alkaline phosphatase with normal levels of iron and ceruloplasmin. Ultrasound revealed no stones in the gallbladder or common bile duct and endoscopic retrograde cholangiopancreatography (ERCP) revealed normal extrahepatic biliary ducts. Which of the following findings is most likely to also be found in this patient?
- A. Anti-centromere antibody
- B. Anti-mitochondrial antibody (Correct Answer)
- C. Anti-gliadin antibody
- D. Anti-neutrophilic cytoplasmic antibodies (ANCA)
- E. Rheumatoid factor
Autoimmune hepatitis Explanation: ***Anti-mitochondrial antibody***
- The patient's symptoms (fatigue, jaundice, itching, dark urine, pale stools) and lab findings (**elevated liver enzymes, direct bilirubin, alkaline phosphatase**) are classic for **primary biliary cholangitis (PBC)**.
- **Anti-mitochondrial antibodies (AMAs)** are highly specific for PBC, being present in over 95% of cases. The mother's history of "eye and mouth dryness" suggests **Sjögren's syndrome**, which is commonly associated with PBC.
*Anti-centromere antibody*
- This antibody is primarily associated with **limited cutaneous systemic sclerosis (CREST syndrome)**, which presents with symptoms like calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias.
- The patient's presentation of cholestasis and associated symptoms is not characteristic of systemic sclerosis.
*Anti-gliadin antibody*
- This antibody is associated with **celiac disease**, an autoimmune disorder characterized by gluten intolerance, leading to gastrointestinal symptoms such as diarrhea, abdominal pain, and malabsorption.
- There is nothing in the patient's presentation to suggest celiac disease, and the liver enzyme pattern points away from it.
*Anti-neutrophilic cytoplasmic antibodies (ANCA)*
- **ANCA** are associated with various autoimmune vasculitides, such as **granulomatosis with polyangiitis (GPA)** and **microscopic polyangiitis (MPA)**.
- While p-ANCA can be seen in primary sclerosing cholangitis (PSC), the patient's clinical picture (female, age) and typical lab pattern is more suggestive of PBC, and ANCA are not a hallmark of PBC.
*Rheumatoid factor*
- **Rheumatoid factor (RF)** is a primary marker for **rheumatoid arthritis**, an autoimmune disease causing chronic joint inflammation.
- While RF can be positive in other autoimmune conditions, it is not a specific diagnostic marker for PBC, and the patient's symptoms are not indicative of rheumatoid arthritis.
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