Chapter 18 – Autoimmune Hepatitis Flashcards
What is an Autoimmune hepatitis?
chronic and progressive hepatitis of unknown etiology.
What is the pathogenesis of Autoimmune hepatitis?
The
pathogenesis is attributed to T cell–mediated autoimmunity, in which hepatocyte injury is
caused by IFN-γ producedbyCD4+ and CD8+ T cells and by CD8+ T-cell–mediated
cytotoxicity.
A defect in regulatory T-cells may underlie the uncontrolled activation of pathogenic, self-reactive lymphocytes.
Genetic factors likely play a role in the autoimmunity (Chapter 6 ).
What injurious immune reaction may be triggered in the pathogenesis of Autoimmune hepatitis?
- viral infections,
- certain drugs
- such as minocycline,
- atorvastatin,
- simvastatin,
- methyldopa,
- interferons
- , nitrofurantoin, and
- pemoline, and
- herbal products (such as black cohosh).
Autoimmune hepatitis commonly occurs
concurrently with other autoimmune disorders, such as what?
- celiac disease,
- systemic lupus erythematosus,
- rheumatoid arthritis,
- thyroiditis,
- Sjögren syndrome, and
- ulcerative colitis.
What is the clinical course of Autoimmune hepatitis?
indolent or severe course (including fulminant hepatitis).
What gender is predominated by Autoimmune hepatitis?
female predominance (78%), particularly in young and perimenopausal women.
What is the epidemiology of Autoimmune hepatitis?
The annual incidence is highest among white northern Europeans at 1.9 per 100,000, but all ethnic groups
are susceptible.
What are the salient features of autoimmune hepatitis?
The salient features [38] include:
- the absence of serologic markers of viral infection,
- elevated serum IgG and γ-globulin levels (1.2 to 3.0 times normal), and
- high serum titers of autoantibodies
What are the 2 classification of Autoimmune hepatitis?
types 1 and 2,
based on the patterns of circulating antibodies.
What is the Type 1 Autoimmune hepatitis?
is characterized by the presence of :
- antinuclear (ANA),
- anti–smooth muscle (SMA),
- anti–actin (AAA), and
- anti–soluble liver antigen/liver-pancreas antigen (anti-SLA/LP) antibodies.
The main antibodies detected in Type 2 autoimmune hepatitis
are anti–liver kidney microsome-1 (ALKM-1) antibodies, which are mostly directed against
CYP2D6, and anti–liver cytosol-1 (ACL-1). Type 1 is much more common than Type 2 in the
United States and is associated with the HLA-DR3 serotype. There is a female predominance,
but the disease occurs in children and adults of both sexes
What is the Type 2 Autoimmune hepatitis?
The main antibodies detected in Type 2 autoimmune hepatitis
are
- anti–liver kidney microsome-1 (ALKM-1) antibodies, which are mostly directed agains CYP2D6, and
- anti–liver cytosol-1 (ACL-1).
What is more common type of Autoimmune hepatitis?
Type 1 is much more common than Type 2 in the
United States and is associated with the HLA-DR3 serotype.
There is a female predominance,
but the disease occurs in children and adults of both sexes
What is seen in autoimmune hepatitis histologically?
The entire histologic spectrum of chronic hepatitis may be seen in autoimmune hepatitis, but it
is marked by prominent inflammatory infiltrates of lymphocytes and plasma cells
*_**Clusters of plasma cells in the interface of portal tracts and hepatic lobules are fairly characteristic for autoimmune hepatitis ( Fig. 18-21 ).**_*
What is fairly a characteristic feature of autoimmune hepatitis?
*_**Clusters of plasma cells in the interface of portal tracts** and **hepatic lobules**_* are fairly characteristic for autoimmune hepatitis ( Fig. 18-21 ).
What are the clinical sympotms of Autoimmue hepatitis?
Symptomatic patients with autoimmune hepatitis tend to show substantial liver destruction and scarring at the time of diagnosis.
Alternatively,
autoimmune hepatitis may present in an atypical fashion with symptoms primarily from involvement of other organ systems, or may be asymptomatic and progress to cirrhosis without
clinical diagnosis.
An acute appearance of clinical illness is common (40%), and a fulminant presentation with onset of hepatic encephalopathy within 8 weeks of disease onset is possible.
In a small subset of patients, autoimmune hepatitis diagnosed clinically may show histologic
destruction of bile ducts (“autoimmune cholangitis”), making distinction from primary biliary
cirrhosis (PBC) or primary sclerosing cholangitis (PSC) quite difficult.
In some cases there is
overlap of the clinical and histologic features of autoimmune hepatitis with those of PBC or PSC.
FIGURE 18-21 Autoimmune hepatitis.
The photograph shows the interface hepatitis with
prominent plasma cells.
