Gastrointestinal/liver/pancreas/gallbladder

Front 1

Not truly papillary or truly cystic
Appears to be unique to the pancreas, but origin is unclear, as it lacks clear evidence of ductal, acinar or endocrine differentiation

Back 1

Solid pseudopapillary tumor of pancreas

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Pseudocyst
no epithelial lining, fluid has high amylase content, cyst arises from drainage of pancreatic secretions from damaged ducts into interstitial tissue, which becomes walled off by fibrous tissue; cyst wall contains histiocytes, giant cells, granulation tissue, rarely eosinophils; cyst may communicate with ductal system

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Serous cystadenoma. A, Cross-section through a serous cystadenoma. Only a thin rim of normal pancreatic parenchyma remains. The cysts are relatively small and contain clear, straw-colored fluid. B, The cysts are lined by cuboidal epithelium without atypia.

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Pancreatic mucinous cystadenoma. A, Cross-section through a mucinous multiloculated cyst in the tail of the pancreas. The cysts are large and filled with tenacious mucin. B, The cysts are lined by columnar mucinous epithelium, and a dense "ovarian" stroma is noted.

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Intraductal papillary mucinous neoplasm. A, Cross-section through the head of the pancreas showing a prominent papillary neoplasm distending the main pancreatic duct. B, The papillary mucinous neoplasm involved the main pancreatic duct (left) and extending down into the smaller ducts and ductules (right).

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Mucinous cystic neoplasm (MCN)
MCN versus IPMN
F >> M M > F
40-50 60-70
Tail Head
Not in duct In duct
Ovarian stroma No ovarian type stroma

Front 7

Back 7

Intraductal papillary mucinous neoplasm (IPMN)
MCN versus IPMN
F >> M M > F
40-50 60-70
Tail Head
Not in duct In duct
Ovarian stroma No ovarian type stroma

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Back 8

Progression model for the development of pancreatic cancer.
K-ras occur at early stages,
that inactivation of the p16 tumor suppressor gene occurs at intermediate stages,
inactivation of the p53, SMAD4 (DPC4), and BRCA2 tumor suppressor genes occur at late stages.

Front 9

A 40 y/o male presented with mildly elevated AST and ALT ( 3 ½ times normal) lab values.
Studies for hepatitis A, B, and C were negative. ANA titer was 1:80, but no history of autoimmune disease
was present.

Back 9

Steatohepatitis

Front 10

A 55 y/o male undergoes a colon resection for an unrelated mass and is found to have a lesion
within the wall of the colon.

Back 10

Ganglioneuromas are characterized by spindle cells and fibrillar matrix with ganglion cells
present in the lamina propria. S-100 is positive. Usually, these lesions may appear like a tubular adenoma or
hyperplastic polyp to the clinician.

Front 11

A 50-year-old female is found to have a 5 cm pancreatic tumor in the tail. A partial
pancreatectomy is performed, and representative histologic images are shown for this case.

Back 11

Mucinous cystadenoma of the pancreas, exclusively in women.

Front 12

A 55-year-old man undergoes an EGD for dysphasia. While examining the duodenum, and
nodularity was noted in the duodenal bulb.

Back 12

Brunner's gland adenoma/hyperplasia

Front 13

A patient with a history of HIV and disseminated histoplasmosis undergoes screening
colonoscopy for intractable G.I. bleeding. Several red to purple patches are found along the colonic mucosa,
and partial resection is performed.

Back 13

Kaposi's sarcoma
HHV8 positive, also seen in
primary effusion lymphomas, Kaposi's sarcoma, and plasma cell variant of
Castleman's disease.

Front 14

95% women, usually child-bearing age (very rare in children), history of 5+ years of oral contraceptives in 85% (occasionally regress after discontinuation); also associated with anabolic steroids (in men), anti-estrogens, Klinefelter’s syndrome or other abnormal secretion of sex steroids

Back 14

Hepatocellular adenoma (liver cell adenoma)
Positive stains: ER, PR
Negative stains: p53

Front 15

Most common primary liver tumor in children (50% of liver malignancies in children)
90% occur by age 5 years, 70% by age 2 years; 2/3 male, prevalence of 1 per 120,000 (1 per million children under age 15 years)
Associated with hemihypertrophy (Beckwith-Wiedemann syndrome), Wilm’s tumor, glycogen storage disease, familial colonic polyposis (APC gene, 500x risk); not associated with cirrhosis

Back 15

Hepatoblastoma: epithelial and mesenchymal elements in varying proportions and at variable stages of differentiation; pseudocapsule, canaliculi with bile formation, cords 2-3 cells thick with alternating light and dark pattern due to glycogen and fat;

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Bile duct hamartoma: von Meyenburg complex or biliary micro-hamartoma, is composed of a cluster of irregularly dilated bile ducts in a fibrotic background

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Bile duct adenoma:
is composed of closely packed, well-formed, and relatively uniform small ducts that form a 1- to 20-mm diameter, sharply demarcated nodule

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Epithelioid hemangioendothelioma
is a low- or intermediate-grade malignancy. The tumor cells appear epithelioid (with abundant eosinophilic cytoplasm) or dendritic (stellate and spindle shaped), and are arranged as single cells or small clusters in a dense or myxoid fibrous stroma. Some tumor cells contain an intracytoplasmic vascular lumen simulating signet-ring-cell carcinoma ;tumor cells should express at least one endothelial marker such as CD34, CD31, or factor VIII.

Front 19

Formerly called lupoid hepatitis
Indolent or severe
70% women, usually ages 20-45 years
A diagnosis of exclusion
Associated with HLA-B8 or HLA-DRw3
60% have other autoimmune disorders (rheumatoid arthritis, thyroiditis, Sjogren’s syndrome, ulcerative colitis)

Back 19

Autoimmune hepatitis
Laboratory: viral markers negative, elevated IgG, high ALT and AST (300-500 mg/dl); elevated anti-nuclear antibody, anti-smooth muscle antibody, anti liver-kidney-microsomal antibody, anti-mitochondrial antibody

Front 20

Visible bile in the liver. The acute form is characterized by canalicular cholestasis with bile accumulation in dilated canaliculi between hepatocytes.

Back 20

Cholestasis

Front 21

An increase in the number of ductules or cholangioles, which are small tubular structures without an apparent lumen typically found at the periphery of portal tracts or fibrous septa. It is virtually always associated with inflammatory cell infiltrates, particularly neutrophils, and should not be interpreted as acute cholangitis.

Back 21

Ductular reaction (proliferation)

Front 22

Irregular, dense eosinophilic cytoplasmic aggregates of cytokeratins, usually seen in ballooning hepatocytes. They stain positive for ubiquitin.

Back 22

Mallory hyaline (bodies)

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carcinoma at the left has a glandular appearance that is most consistent with a cholangiocarcinoma. A liver cancer may have both hepatocellular as well as cholangiolar differentiation. Cholangiocarcinomas do not make bile, but the cells do make mucin, and they can be almost impossible to distinguish from metastatic adenocarcinoma on biopsy or fine needle aspirate.

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Cholangiocarcinoma (intrahepatic)
Positive stains:
mucin
CEA (cytoplasmic and luminal, not canalicular),
CAM 5.2
CK7 (90-96%),
CK19 (84%),
CK20 (30-70%, more often positive in non-peripheral tumors),
MOC31, BerEP4
Negative stains: AFP

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Chronic hepatitis C with a portal lymphoid aggregate.

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Epstein-Barr virus hepatitis
is characterized by a diffuse lymphocytic infiltrate in the sinusoids in a “beads on a string” pattern

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Adenovirus hepatitis
causes random foci of coagulative necrosis with minimal inflammatory response. The infected hepatocytes show smudgy nuclei with chromatin margination

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Autoimmune hepatitis
typically affects women who often have other types of immunologic disorders. Autoimmune hepatitis is characteristically associated with hypergammaglobulinemia and/or high titers of antinuclear, anti-smooth muscle, anti-liver-kidney microsomal type 1, or antisoluble liver antigen autoantibodies.
In classic cases, there is a dense portal and lobular mononuclear cell infiltrate enriched in plasma cells

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PBC
is a progressive cholestatic disease that leads to the destruction of intrahepatic bile ducts. It occurs mainly in middle-aged women. The early stage of the disease (stage 1) is defined by mixed portal inflammatory cell infiltrates and the pathognomonic florid duct lesion characterized by granulomatous or lymphocytic infiltration of the duct epithelium

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HCC;
A reticulin stain is extremely useful for highlighting distorted architecture and widened cell plates, and for showing a reduction or loss of reticulin fibers

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HCC

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Hepatocellular carcinoma, fibrolamellar variant

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Hepatoblastoma. Note the presence of extramedullary hematopoiesis.

Front 34

Mean age 47 years, but occurs at any age, 60% women
No predisposing factors

Back 34

Epithelioid hemangioendothelioma
Positive stains: factor VIII related antigen and CD34 for vacuoles, CD31
Negative stains: AE1/AE3 (cytokeratin), CK7, CK20, alpha fetoprotein, bile, CEA, HepPar1, mucin
EM: Weibel-Palade bodies, intermediate filaments

Front 35

Most common sarcoma of the liver, characterized by spindle and pleomorphic malignant endothelial cells.; 25-42% associated with exposure to vinyl chloride, arsenic, Thorotrast (thorium dioxide) or androgen steroids

Back 35

Angiosarcoma
Positive stains: CD34, CD31, factor VIII related antigen, Ulex europaeus lectin type 1 (may not be present in poorly vasoformative areas)
Negative stains: keratin (but positive in 12-35%)
EM: Weibel-Palade bodies

Front 36

Also called arteriohepatic dysplasia
Autosomal dominant, due to mutations in Jagged1 gene on #20p, which encodes a ligand for Notch1 and plays a role in epithelial-mesenchymal interactions
Normal liver, but no portal tract bile ducts (progressive loss, may occasionally regrow)
Characteristic facies, vertebral arch anomalies, supravalvular pulmonic stenosis
May survive into adulthood, but increased risk of hepatic failure and hepatocellular carcinoma

Back 36

Alagille’s syndrome

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Liver biopsy of a patient with Bloom syndrome showing diffuse sclerosing fibrosis around central vein (open curved arrow) and centrilobularly distributed Mallory bodies (arrows) with scattered erythrocytes in the adjacent sinusoids

Front 38

This gastric biopsy (Fig. 6.5) was from a 63-year-old woman with a history of chronic atrophic gastritis. All of the following statements are true about this lesion EXCEPT:
a. This type of lesion usually arises from Enterochromaffin-like (ECL) cells of the stomach.
b. Chronic atrophic gastritis is a predisposing factor.
c. This lesion is expected to be positive for chromogranin A and negative for serotonin.
d. This lesion occurs in association with multiple endocrine neoplasia type 2 (MEN2).
e. Mutation and/or loss of heterozygosity (LOH) at the MEN1 locus is associated with the development of this lesion.

Back 38

d.
The section shows gastric lamina propria with small nests and gland-like lesion. The higher magnification (picture not shown) showed that tumor cells have uniform nuclei, diffuse chromatin and inconspicuous nucleoli. These features are those of
carcinoid tumor
. The formation of glands in gastric carcinoid tumor like this case can mimic adenocarcinoma. Five types of gastric carcinoid tumors are recognized, including ECL (enterochromaffin-like) cell tumor associated with type A chronic atrophic gastritis, ECL cell tumor with combined MEN-1 and Zollinger-Ellison syndrome, sporadic ECL tumor, Non-ECL tumors, and ECL cell tumor with achlorhydria and parietal cell hyperplasia. Hypergastrinemia, autoimmune chronic atrophic gastritis, MEN type 1, and Zollinger-Ellison syndrome are all predisposing factors for the development of gastric carcinoid tumor. There is no evidence that carcinoid tumor is associated with MEN type II.

Front 39

A 10-year-old girl was found to have hepatosplenomegaly and elevated liver function tests. Serum ceruloplasmin was 23 mg/dL (normal 18-45); serum copper 65 mg/dL (normal 66-145); and 24-hour urine copper level was 1135 mg (normal 15-50). H&E sections showed precirrhotic changes with extensive bridging fibrosis and incomplete nodules. A rhodanine stain for copper is shown (Fig. 6.17). All of the following are true about this disease EXCEPT:
a. It usually presents with signs of liver disease during adolescence.
b. Laboratory testing usually shows increased urinary copper and reduced serum copper and ceruloplasmin.
c. Positive copper or copper associated protein stains are more prominent in zone 1 distribution in early phase and is more generalized in late phase.
d. Characteristic mitochondrial abnormalities including dilatation of mitochondrial cristae and pleomorphic shapes are seen by electron microscopy.
e. The disease is symptomatic before 3 years of age

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e.
The lesion demonstrated here is
Wilson disease
. It is an autosomal recessive disease with mutations in gene ATPase that transports copper. Most Wilson disease patients have compound heterozygous mutation on the ATPase gene. The diagnosis should be considered in any patients with hepatocellular disease of unknown etiology, because histologic features of Wilson disease can mimic many varieties of parenchymal liver disease. Wilson disease should be ruled out in a young patient with the findings of fatty liver with chronic hepatitis. Fatty change in hepatocytes is the most common finding. Many patients may present with a chronic hepatitis-like histopathology with marked portal and periportal inflammation. Another notable finding is that hepatocytes often have eosinophilic cytoplasm and contain Mallory bodies. Wilson disease rarely presents before 3 years of age. Kayser-Fleischer rings are diagnostic for Wilson disease, but it is only present in about 50% of adult patients with Wilson disease.

Front 40

22-year-old man with a history of anemia, malnutrition, and abdominal pain had multiple polyps in the colorectal region. His older sister had a similar history. Which of the following is true about this lesion?
a. It is inherited as autosomal recessive pattern.
b. It is limited to the colon and rectum.
c. Hyperpigmentation around the mouth is commonly seen in patients with this lesion.
d. There is increased risk for the development of colorectal cancer.
e. Juvenile (retention) polyp is morphologically different from this lesion.

Back 40

d.
The lesion in this figure depicts histologic features for J
uvenile polyp
. Similar histologic features also can be found in hyperplastic polyps, inflammatory polyps, and Cronkhite-Canada syndrome. In conjunction with the patient's history and his clinical presentation, most likely this patient had Juvenile polyposis coli. Juvenile polyposis coli is a rare autosomal dominant disorder characterized by germline mutations in SMAD4/DPC4 tumor suppressor gene on chromosome 18q21. Juvenile polyps may extend to the stomach and small intestine, may coexist with adenomatous polyps, and are associated with increased risk for colorectal carcinoma. It is not associated with the extraintestinal features seen in other hamartomatous polyposis syndrome. Juvenile (retention) polyp, also known as solitary sporadic juvenile polyp, is the most frequent colonic polyp in children. It may represent a hamartomatous or inflammatory process.

Front 41

The most common form of congenital extrahepatic bile duct dilatation is:
a. Caroli disease
b. Choledochal cyst
c. Choledochocele
d. Diverticulum of the common bile duct
e. Cholangiectases

Back 41

b. Choledochal cyst
is a congenital cystic dilatation of the extrahepatic biliary tree, intrahepatic biliary radicles, or both and commonly presents with the triad of pain, jaundice, and mass in the right upper quadrant. They are more prevalent in Asia than in the United States and other Western countries. Caroli disease is characterized by dilatation of the intrahepatic biliary tree. Caroli disease is sporadic, whereas Caroli syndrome is generally inherited in an autosomal recessive manner. The latter is often associated with congenital hepatic fibrosis and portal hypertension. This form of Caroli disease is also often associated with autosomal recessive polycystic kidney disease (ARPKD). Diverticulum of the common bile duct and choledochocele are less common than choledochal cyst. Cholangiectases are not congenital and usually are caused by inflammatory process.

Front 42

Characteristic histologic findings of mechanical bile duct obstruction include all following features EXCEPT:
a. Portal edema and periportal inflammatory infiltrate, usually with more neutrophils
b. Cholestasis and cholate stasis
c. Ductule reaction
d. Parenchymal and/or portal granulomas
e. Cholangitis

Back 42

d.
Portal edema, periportal inflammatory infiltrates, cholangitis, cholestasis (bile thrombi in canaliculi, hepatocytes, and Kupffer cells) are usually seen in recent complete obstruction of bile ducts. If obstruction continues for weeks, chronic cholestasis (pseudoxanthomatous change, also known as cholate stasis, usually can be demonstrated by copper and or copper-associated protein staining), and portal and periportal fibrosis with ductular reaction are often seen. Giant cell transformation and cholestatic liver cell rosettes are also seen. Parenchymal and portal granulomas are features of primary biliary cirrhosis, and it is not a feature commonly seen in mechanical bile duct obstruction.

Front 43

AMA-negative cholangitis (autoimmune cholangitis) is characterized by all of the following EXCEPT:
a. Presence of antibodies against the pyruvate dehydrogenase enzyme complex in inner mitochondrial membrane
b. Portal lymphoid aggregates with germinal centers
c. Granulomatous cholangitis
d. Periductal fibrosis
e. Progressive disease

Back 43

a.
AMA-negative cholangitis is a variant of primary biliary cirrhosis (PBC). Its clinical, biochemical, and histologic features are those of typical PBC with exceptions of its autoantibody profile (negative for AMA and positive for ANA and anti-alpha smooth muscle actin antibody).

Front 44

The best method of distinguishing neonatal hepatitis from biliary atresia is:
a. The degree of liver enzymes elevation
b. Ultrasound
c. Hepatobiliary scanning
d. Liver biopsy
e. Cholangiography

Back 44

d. Liver biopsy
is reliable in making the correct diagnosis in up to 95% of cases. Multinucleated hepatocytes and portal and lobular mononuclear cell infiltration are more prominent in neonatal hepatitis than biliary obstruction, while cholestasis and ductular reaction are more severe in biliary atresia.

Front 45

2-year-old boy presented with a large mass in the right lobe of the liver and elevated serum alpha-fetoprotein. A representative section from the mass is shown (Fig. 6.26). Good survival from this lesion is expected if the tumor:
a. Is diagnosed at 1 year of age
b. Secretes alpha-fetoprotein
c. Shows a macrotrabecular pattern
d. Is well circumscribed and surgically resectable
e. Shows mutation or deletions of p53

Back 45

d.
The section shows a hepatoblastoma, mixed epithelial and mesenchymal type. Light and dark cells are seen in epithelial types of hepatoblastoma. The mesenchymal components consist of osteoid-like material and primitive fibrous stroma. Hepatoblastoma is the most frequent liver tumor in children less than 5 years old. Its survival correlates with a complete resection of the tumor. The stage of the tumor at the time of initial resection is an important prognostic factor. Except for the small cell undifferentiated variant, histologic patterns do not correlate with survival when adjusted for age, sex, and stage. Age and level of alpha-fetoprotein are not prognostic factors. Wnt signaling pathway involving mutation of the β-catenin and APC genes plays an important role in hepatoblastoma. Mutations in β-catenin can be found in about 50% of hepatoblastoma patients. Mutations and deletions of p53 are not common in this tumor.

Front 46

45-year-old man had a long history of chronic liver disease including episodes of jaundice and cirrhosis. The patient died after an upper GI bleed. Autopsy revealed a nodular liver weighing 1900 grams. H&E stained sections showed a well-established cirrhosis. The image shown here is from a PAS-diastase stained section. The diagnosis of this lesion is supported by all of the following pathologic findings EXCEPT:
a. The demonstration of PASD-positive globules in periportal hepatocytes
b. Positive immunohistochemical stain for alpha-1 antitrypsin
c. The ultrastructural demonstration of electrondense material inside the cisternae of the rough endoplasmic reticulum
d. Serum electrophoresis of alpha-1-antitrypsin molecules
e. The finding of macronodular cirrhosis with cholestasis

Back 46

e.
Cirrhosis associated with alpha-1 antitrypsin (AAT) deficiency is usually micronodular, but it may be macronodular or mixed type. Since many liver diseases can lead to cirrhosis, the finding of cirrhosis is not specific to this disease. The production of AAT is controlled by a pair of genes at the protease inhibitor (Pi) locus. The normal allele is M (PiM) and homozygous individuals (MM) produce normal amounts of AAT. The most common form of AAT deficiency is associated with allele Z, homozygous PiZ (ZZ). Serum levels of AAT in these patients are about 10%-15% of normal serum levels. Other genotypes associated with severe AAT deficiency include PiSZ, PiZ/Null, and PiNull. Patients with the null gene for AAT will not produce any AAT and are high risk for emphysema. But, patients with PiNull gene will not develop liver disease because they do not produce any AAT, thus no ATT accumulation in liver cell. Individuals with heterozygous MZ, MS or M/Null do not usually develop disease of AAT deficiency.

Front 47

22-year-old man who was found to have multiple small intestinal polyps. All sections show similar histologic features as demonstrated in this image. Which of the following genes is involved in its pathogenesis?
a. APC gene
b. TP53
c. STK11
d. SMAD4
e. PTEN

Back 47

c.
The section shows a polyp with an infrastructure of arborizing smooth muscle dividing the nondysplastic glands into lobules, which is the characteristic histologic feature of
hamartomatous polyp
, Peutz-Jeghers type. Peutz-Jeghers syndrome is an inherited cancer syndrome with autosomal dominant trait. Mucocutaneous melanin pigmentation and hamartomatous intestinal polyposis are characteristic features. It is associated with gene SKT11 (Serine/Threonine protein kinase), with nearly complete penetrance.

Front 48

The liver biopsy stained with Masson trichrome in Figure 6.18 was from a 27-year-old woman with mildly elevated liver tests for approximately 1 year. There was no history of alcohol abuse, but the patient was obese. Which of following statements is true about the condition depicted in this biopsy EXCEPT:
a. This histopathologic process can be caused by alcohol, diabetes mellitus, obesity, or tamoxifen.
b. In pediatric cases, there is periportal pericellular fibrosis, while in adults the fibrosis is predominantly in zone 3 area.
c. Interface hepatitis is often present.
d. Mallory bodies are usually present in ballooned hepatocytes.
e. Mallory bodies surrounded by neutrophils are more often seen in alcoholic patients with this entity than nonalcoholic patients.

Back 48

c.
The picture shows pericellular fibrosis (chicken-wire fibrosis), steatosis, and ballooned hepatocytes with poorly formed Mallory bodies.
Steatohepatitis
is a histologic entity that is characterized by steatosis (can be macrovesicular or mixed macrovesicular and microvesicular steatosis); pericellular fibrosis (chickenwire fibrosis); and/or Mallory bodies. Both alcoholic and nonalcoholic (diabetes, obesity, toxins, and drugs) causes are recognized. Interface hepatitis seen in viral hepatitis is not a feature of steatohepatitis.

Front 49

2-year-old girl had hepatosplenomegaly, lymphadenopathy, cough swallowing difficulties since she was 2 months of age. She died from aspiration pneumonia. The liver section taken from autopsy for this patient is shown (Fig. 6.22). Which of the statements about this lesion is true?
a. It is caused by deficiency in α-glucosidase A, mainly in macrophages.
b. Affected cells are Kupffer cells.
c. Affected cells are Kupffer cells and hepatocytes.
d. Among lysosomal storage diseases, this lesion is the least common type.
e. Genetic mutation is the sole factor responsible for the presentation of this lesion.

Back 49

b.
The patient's clinical presentation and histology shown in this section is typical for
Gaucher disease
, type II. Gaucher disease is caused by a deficiency in acid β-glucosidase, mainly in macrophage. It is the most common type of lysosomal storage diseases. There are three types of Gaucher disease, including type I (chronic non-neuronopathic/adulthood); type II (infantile disease); and type III (juvenile disease). About 200 mutations in the gene responsible to β-glucosidase have been described; there is a poor genotype-phenotype correlation. The diagnosis of Gaucher disease is made by assay of acid β-glucosidase enzyme in blood. Enlarged cells with wrinkled tissue paper appearance in cytoplasm are affected Kupffer cells, not hepatocytes.

Front 50

The histologic features shown in Figure 6.21 can be found in:
a. Q fever
b. Hepatitis A
c. Hodgkin disease
d. Cytomegalovirus and Epstein-Barr virus infection
e. All of above

Back 50

e.
The photo shows a
fibrin-ring granuloma
, central fatty vacuole surrounded by histocytes and a mesh of brightly eosinophilic ring of fibrin. It can be found in all listed diseases. It is a distinctive lesion, but is not specific.

Front 51

Clinicopathologic features of primary biliary cirrhosis (PBC) including all of the following, EXCEPT:
a. Damage to the intrahepatic and extrahepatic bile ducts
b. Presence of antimitochondrial antibodies (AMA)
c. Presence of ruptured bile ducts surrounded by lymphocytes, plasma cells, and non-caseating epithelioid granulomas in portal areas
d. Bile duct injury is mediated by CD8 and CD4 lymphocytes.
e. Predilection to middle-aged and elderly women

Back 51

a.
Primary biliary cirrhosis (PBC) is primarily a disease of the intrahepatic bile ducts with variable injury of hepatocytes. The image (Fig. 6.33) shows chronic inflammation and ill-defined granuloma surrounding a damaged bile duct. More than 50% of PBC patients have some features of Sjögren syndrome. It has a strong female predominance of 9-10 to 1. It is a slowly progressive liver disease with final outcome of cirrhosis after many years of precirrhotic stages.