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Pathology – Cholecystitis
Cholecystitis is defined as gallbladder inflammation.
Epidemiology: • Very prevalent. Aetiology • The most common cause is gallstones (calculous cholecystitis). • Acalculous cholecystitis is also seen, particularly in the elderly.
Pathogenesis • Chemical harm to the mucosa by bile is thought to be the cause of biliary stasis. • A gallstone or inadequate gallbladder motility might block the gallbladder exit. Biliary colic causes acute upper abdomen pain that disappears spontaneously after several hours. • Acute cholecystitis is a serious condition characterized by persistent upper abdominal pain, fever, and tachycardia. Macroscopy reveals thicker gallbladder walls and potentially friable mucosa. • Gallstones are often present.
Histopathology • Acute cholecystitis is characterized by oedema, inflammatory cells, and granulation tissue. • Chronic cholecystitis is characterized by muscular hypertrophy and fibrous tissue, mild chronic inflammation, and the development of mucosal diverticula that herniate through the muscular layer (Rokitansky-Aschoff sinuses). • Xanthogranulomatous cholecystitis is a type of chronic cholecystitis characterized by the presence of macrophages and fibroblasts, likely due to a ruptured Rokitansky-Aschoff sinus.
Prognosis: Most individuals with calculous cholecystitis can be treated with cholecystectomy.
Cholecystitis is defined as gallbladder inflammation.
Epidemiology: • Very prevalent. Aetiology • The most common cause is gallstones (calculous cholecystitis). • Acalculous cholecystitis is also seen, particularly in the elderly.
Pathogenesis • Chemical harm to the mucosa by bile is thought to be the cause of biliary stasis. • A gallstone or inadequate gallbladder motility might block the gallbladder exit. Biliary colic causes acute upper abdomen pain that disappears spontaneously after several hours. • Acute cholecystitis is a serious condition characterized by persistent upper abdominal pain, fever, and tachycardia. Macroscopy reveals thicker gallbladder walls and potentially friable mucosa. • Gallstones are often present.
Histopathology • Acute cholecystitis is characterized by oedema, inflammatory cells, and granulation tissue. • Chronic cholecystitis is characterized by muscular hypertrophy and fibrous tissue, mild chronic inflammation, and the development of mucosal diverticula that herniate through the muscular layer (Rokitansky-Aschoff sinuses). • Xanthogranulomatous cholecystitis is a type of chronic cholecystitis characterized by the presence of macrophages and fibroblasts, likely due to a ruptured Rokitansky-Aschoff sinus.
Prognosis: Most individuals with calculous cholecystitis can be treated with cholecystectomy.
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Pathology - Pancreatic malformations
Ectopic pancreas is a common developmental defect that occurs when pancreatic tissue is placed outside of its normal position. • The duodenum is the most common location, but it can also be found in the jejunum, ileum, and Meckel's diverticulum • While most cases are unintentional, some patients may experience symptoms such as bleeding or blockage.
Pancreas Divisum • A common developmental abnormality in which the dorsal and ventral pancreatic buds fail to merge. • The Santorini duct becomes the pancreas' major ductal system. This duct drains into the duodenum via the minor papilla, leading to pancreatic secretory stasis and susceptibility to pancreatitis. It is typically asymptomatic and detected during imaging, however some patients may develop pancreatitis later in life.
Annular pancreas • A rare developmental defect occurs when the dorsal and ventral pancreatic buds unite around the duodenum. • The ring of pancreas can cause obstruction to the duodenum. • Patients often arrive around 1 year of age with vomiting and abdominal distention after meals.
Ectopic pancreas is a common developmental defect that occurs when pancreatic tissue is placed outside of its normal position. • The duodenum is the most common location, but it can also be found in the jejunum, ileum, and Meckel's diverticulum • While most cases are unintentional, some patients may experience symptoms such as bleeding or blockage.
Pancreas Divisum • A common developmental abnormality in which the dorsal and ventral pancreatic buds fail to merge. • The Santorini duct becomes the pancreas' major ductal system. This duct drains into the duodenum via the minor papilla, leading to pancreatic secretory stasis and susceptibility to pancreatitis. It is typically asymptomatic and detected during imaging, however some patients may develop pancreatitis later in life.
Annular pancreas • A rare developmental defect occurs when the dorsal and ventral pancreatic buds unite around the duodenum. • The ring of pancreas can cause obstruction to the duodenum. • Patients often arrive around 1 year of age with vomiting and abdominal distention after meals.
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Pathology – Acute Pancreatitis
Acute pancreatitis is defined as acute inflammation of the pancreas and surrounding tissues.
Epidemiology • Uncommon.
Aetiology • Gallstones and alcohol are responsible for the majority of instances. • Other reasons include abdominal trauma, endoscopic retrograde cholangiopancreatography (ERCP), medications, hypercalcemia, pancreatic divisum, and viral infection. • Most cases are idiopathic.
Pathogenesis • Pancreatic injury triggers the release of digestive enzymes, resulting in necrosis of pancreatic and peripancreatic tissues. • Exudation of plasma into the retroperitoneal area causes hypovolemia and cardiovascular instability. • Paralytic ileus can also be caused by severe inflammation near the gut.
Presentation: Sudden onset of severe upper abdominal discomfort spreading to the back, accompanied by nausea, vomiting, and fever. Hypotension may occur, leading to shock.
Biochemistry: A significant increase in serum amylase can indicate acute pancreatitis in the appropriate clinical situation.
Macroscopy reveals an enlarged and squishy pancreas. • Peripancreatic tissues show white flecks of fat necrosis. • Severe instances result in bleeding into the necrotic pancreas.
Histopathology reveals acute inflammation, oedema, and localized necrosis of the pancreas. • There is fat necrosis around the peripancreatic tissue. • Severe cases have extensive necrosis and bleeding within the gland.
Prognosis: • Most instances are moderate and treatable with supportive measures. • Severe cases may require organ support in an intensive care unit. • Infection of necrotic pancreatic tissue can result in disseminated intravascular coagulation and multiple organ failure. • Pancreatic pseudocysts, an accumulation of fluid within the pancreas, are a typical late consequence.
Acute pancreatitis is defined as acute inflammation of the pancreas and surrounding tissues.
Epidemiology • Uncommon.
Aetiology • Gallstones and alcohol are responsible for the majority of instances. • Other reasons include abdominal trauma, endoscopic retrograde cholangiopancreatography (ERCP), medications, hypercalcemia, pancreatic divisum, and viral infection. • Most cases are idiopathic.
Pathogenesis • Pancreatic injury triggers the release of digestive enzymes, resulting in necrosis of pancreatic and peripancreatic tissues. • Exudation of plasma into the retroperitoneal area causes hypovolemia and cardiovascular instability. • Paralytic ileus can also be caused by severe inflammation near the gut.
Presentation: Sudden onset of severe upper abdominal discomfort spreading to the back, accompanied by nausea, vomiting, and fever. Hypotension may occur, leading to shock.
Biochemistry: A significant increase in serum amylase can indicate acute pancreatitis in the appropriate clinical situation.
Macroscopy reveals an enlarged and squishy pancreas. • Peripancreatic tissues show white flecks of fat necrosis. • Severe instances result in bleeding into the necrotic pancreas.
Histopathology reveals acute inflammation, oedema, and localized necrosis of the pancreas. • There is fat necrosis around the peripancreatic tissue. • Severe cases have extensive necrosis and bleeding within the gland.
Prognosis: • Most instances are moderate and treatable with supportive measures. • Severe cases may require organ support in an intensive care unit. • Infection of necrotic pancreatic tissue can result in disseminated intravascular coagulation and multiple organ failure. • Pancreatic pseudocysts, an accumulation of fluid within the pancreas, are a typical late consequence.
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Pathology - diabetic nephropathy
Definition: Chronic kidney disease caused by diabetes mellitus.
Epidemiology • A leading cause of chronic renal disease.
Aetiology • Diabetes. • Only 30-40% of diabetics develop nephropathy, suggesting that other factors play a role.
Pathogenesis • High glucose levels harm endothelial cells in the glomeruli, resulting in glomerulosclerosis and nephron loss.
Presentation • A patient with diabetes mellitus develops proteinuria. • Microalbuminuria often precedes overt proteinuria, which can lead to nephrotic syndrome. • Hypertension is always present.
Histopathology • Glomerulosclerosis, a diffuse increase in the mesangial matrix, can lead to the formation of Kimmelsteil-Wilson nodules. • Thicker glomerular basement membranes. • Hyalinization of the afferent and efferent arterioles. • Tubulointerstitial fibrosis is proportional to glomerular injury. 2 A kidney biopsy is not essential to confirm a diagnosis of diabetic nephropathy if the clinical presentation is typical. Biopsies are often reserved for unusual cases where alternate diagnoses may be possible. immune fluorescence • IgG deposition in glomerular and tubular basement membranes can be observed. Electron microscopy • Thickened glomerular basement membrane and expanded mesangial matrix are observed. • There are no immunological complexes present.
Prognosis: Gradual decline in renal function. • Patients with severe hypertension and significant proteinuria fare poorly. • Proper blood pressure management is crucial for reducing disease progression.
Definition: Chronic kidney disease caused by diabetes mellitus.
Epidemiology • A leading cause of chronic renal disease.
Aetiology • Diabetes. • Only 30-40% of diabetics develop nephropathy, suggesting that other factors play a role.
Pathogenesis • High glucose levels harm endothelial cells in the glomeruli, resulting in glomerulosclerosis and nephron loss.
Presentation • A patient with diabetes mellitus develops proteinuria. • Microalbuminuria often precedes overt proteinuria, which can lead to nephrotic syndrome. • Hypertension is always present.
Histopathology • Glomerulosclerosis, a diffuse increase in the mesangial matrix, can lead to the formation of Kimmelsteil-Wilson nodules. • Thicker glomerular basement membranes. • Hyalinization of the afferent and efferent arterioles. • Tubulointerstitial fibrosis is proportional to glomerular injury. 2 A kidney biopsy is not essential to confirm a diagnosis of diabetic nephropathy if the clinical presentation is typical. Biopsies are often reserved for unusual cases where alternate diagnoses may be possible. immune fluorescence • IgG deposition in glomerular and tubular basement membranes can be observed. Electron microscopy • Thickened glomerular basement membrane and expanded mesangial matrix are observed. • There are no immunological complexes present.
Prognosis: Gradual decline in renal function. • Patients with severe hypertension and significant proteinuria fare poorly. • Proper blood pressure management is crucial for reducing disease progression.
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Pathology - Extrahepatic bile duct carcinoma
Definition: A malignant epithelial tumor that develops in an extrahepatic bile duct.
Epidemiology • Incidence is rare and does not vary by geography. Aetiology • PSC. • Infestation of liver fluke. • Choledochal cysts. 2 Choledocholithiasis doesn't appear to be important. Carcinogenesis • Mutations in KRAS and TP53 are identified.
Presentation: • Obstructive jaundice. • Fever and rigors can be caused by superimposed cholangitis. Macroscopy reveals a tumor in the bile duct, which can be polypoid, stenosing, or diffusely infiltrative.
Histopathology: • The majority of adenocarcinomas are well or moderately differentiated, with malignant epithelial cells forming glandular structures similar to bile ducts.
Prognosis: Patients with resectable tumors and clear surgical margins typically have a 5-year survival rate of 20-40%. • Tumors associated with PSC have a low 5-year survival rate (<10%).< />pan>
Definition: A malignant epithelial tumor that develops in an extrahepatic bile duct.
Epidemiology • Incidence is rare and does not vary by geography. Aetiology • PSC. • Infestation of liver fluke. • Choledochal cysts. 2 Choledocholithiasis doesn't appear to be important. Carcinogenesis • Mutations in KRAS and TP53 are identified.
Presentation: • Obstructive jaundice. • Fever and rigors can be caused by superimposed cholangitis. Macroscopy reveals a tumor in the bile duct, which can be polypoid, stenosing, or diffusely infiltrative.
Histopathology: • The majority of adenocarcinomas are well or moderately differentiated, with malignant epithelial cells forming glandular structures similar to bile ducts.
Prognosis: Patients with resectable tumors and clear surgical margins typically have a 5-year survival rate of 20-40%. • Tumors associated with PSC have a low 5-year survival rate (<10%).< />pan>
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Pathology - Hypertensive Nephropathy
Definition: Chronic renal disease associated with hypertension. Epidemiology: • Common causes of chronic kidney disease.
Aetiology • Hypertension
Pathogenesis • Two pathophysiological explanations have been proposed and may not be mutually exclusive. • Arteriole constriction leads to glomerular ischemia and scarring, while glomerular hypertension produces glomerular hyperfiltration and eventual glomerulosclerosis. Presentation: Renal failure and proteinuria in a long-standing hypertensive with no additional causes of renal illness. • Additional indicators of hypertension, such as left ventricular hypertrophy, may also be present.
Macroscopy: • Both kidneys are shrunken, with finely granular cortical surfaces. Histopathology • Afferent arterioles have hyaline deposits. • Medial hypertrophy and intimal thickening are seen in interlobular and larger arteries. • Glomeruli may show wrinkling and shrinkage followed by scarring of the whole tuft or enlargement of the tuft followed by segmental scarring. • These pathological features are referred to as nephrosclerosis, which simply means hardening of the kidney.
The appearance of nephrosclerosis may not necessarily indicate hypertensive nephropathy, as similar alterations might occur with aging or diabetes. However, a kidney biopsy can confirm a diagnosis of hypertensive nephropathy if the clinical presentation matches.
Prognosis: • Chronic kidney disease tends to progress. • Aggressive blood pressure control can reduce the deterioration in renal function.
Definition: Chronic renal disease associated with hypertension. Epidemiology: • Common causes of chronic kidney disease.
Aetiology • Hypertension
Pathogenesis • Two pathophysiological explanations have been proposed and may not be mutually exclusive. • Arteriole constriction leads to glomerular ischemia and scarring, while glomerular hypertension produces glomerular hyperfiltration and eventual glomerulosclerosis. Presentation: Renal failure and proteinuria in a long-standing hypertensive with no additional causes of renal illness. • Additional indicators of hypertension, such as left ventricular hypertrophy, may also be present.
Macroscopy: • Both kidneys are shrunken, with finely granular cortical surfaces. Histopathology • Afferent arterioles have hyaline deposits. • Medial hypertrophy and intimal thickening are seen in interlobular and larger arteries. • Glomeruli may show wrinkling and shrinkage followed by scarring of the whole tuft or enlargement of the tuft followed by segmental scarring. • These pathological features are referred to as nephrosclerosis, which simply means hardening of the kidney.
The appearance of nephrosclerosis may not necessarily indicate hypertensive nephropathy, as similar alterations might occur with aging or diabetes. However, a kidney biopsy can confirm a diagnosis of hypertensive nephropathy if the clinical presentation matches.
Prognosis: • Chronic kidney disease tends to progress. • Aggressive blood pressure control can reduce the deterioration in renal function.
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Pathology - Acute renal failure
Definition: Significant deterioration in renal function over hours or days.
Epidemiology • Common. • This condition is typically a complication of a pre-existing sickness that disrupts circulation.
Aetiology • Hypoperfusion, such as hypovolaemia or sepsis, occurs prior to renal function.
• Renal ('intrinsic'): acute tubular injury, drug-induced interstitial nephritis, anti-glomerular basement membrane disease, small vessel vasculitis, hemolytic uraemic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP) and severe hypertension.
• Post-renal: bilateral obstruction (or obstruction of a single kidney). 2 The most prevalent causes are hypoperfusion and acute tubular damage.
Pathogenesis: • Kidney disease causes sudden decline in function.
Presentation: • Oliguria (passing little amounts of urine) is the most common symptom, but some cases may show no symptoms. • Severe cases result in pulmonary edema, encephalopathy, and pericarditis.
Biochemistry: Serum urea and creatinine are key indicators. • Hyperkalemia and metabolic acidosis are also prevalent. Severe hyperkalemia is pro-arrhythmic and can cause cardiac arrest, requiring rapid treatment. Prognosis • Pre-renal and post-renal acute renal failure (ARF) can be reversible if promptly addressed by restoring circulation volume or alleviating obstruction. • Intrinsic types of ARF typically necessitate dialysis while the underlying etiology is managed and renal function recuperates.
Definition: Significant deterioration in renal function over hours or days.
Epidemiology • Common. • This condition is typically a complication of a pre-existing sickness that disrupts circulation.
Aetiology • Hypoperfusion, such as hypovolaemia or sepsis, occurs prior to renal function.
• Renal ('intrinsic'): acute tubular injury, drug-induced interstitial nephritis, anti-glomerular basement membrane disease, small vessel vasculitis, hemolytic uraemic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP) and severe hypertension.
• Post-renal: bilateral obstruction (or obstruction of a single kidney). 2 The most prevalent causes are hypoperfusion and acute tubular damage.
Pathogenesis: • Kidney disease causes sudden decline in function.
Presentation: • Oliguria (passing little amounts of urine) is the most common symptom, but some cases may show no symptoms. • Severe cases result in pulmonary edema, encephalopathy, and pericarditis.
Biochemistry: Serum urea and creatinine are key indicators. • Hyperkalemia and metabolic acidosis are also prevalent. Severe hyperkalemia is pro-arrhythmic and can cause cardiac arrest, requiring rapid treatment. Prognosis • Pre-renal and post-renal acute renal failure (ARF) can be reversible if promptly addressed by restoring circulation volume or alleviating obstruction. • Intrinsic types of ARF typically necessitate dialysis while the underlying etiology is managed and renal function recuperates.
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Pathology – Non Alcoholic Fatty Liver Disease
• The metabolic syndrome's hepatic manifestation, which includes hyperlipidemia, poor glucose tolerance, and central obesity. Simple steatosis (fatty liver), non-alcoholic steatohepatitis (NASH), and cirrhosis are among the disorders that fall under the umbrella of non-alcoholic fatty liver disease (NAFLD).
Epidemiology: Due to increased obesity rates, this condition is very frequent and is becoming more common. • The most frequent reason for abnormal liver function tests these days. • A large number of cirrhosis cases that were previously believed to be cryptogenic are now believed to be end-stage NAFLD. The cause • The most frequent correlations are between diabetes and obesity. • Also connected to parenteral feeding and some medications.
The pathogenesis • Obesity and insulin resistance appear to be the main contributing factors. • Hepatocyte damage and fat storage are brought on by insulin resistance. • Inflammation in reaction to hepatocyte damage causes fibrosis and, in certain cases, cirrhosis.
Presentation: Abnormal liver function tests are used to identify the majority of asymptomatic cases. • Cirrhosis-related problems can occasionally be seen in patients.
The macroscopy • Cirrhotic livers are diffusely nodular; the liver is swollen, squishy, and greasy.
The study of histopathology • NASH exhibits steatosis together with the presence of enlarged hepatocytes and neutrophils; steatosis is characterized by the buildup of fat within hepatocytes without discernible inflammatory activity. Depending on the disease's stage, there may be variable fibrosis. Keep in mind that these histology results are nearly the same as those observed in alcoholic liver disease. It can occasionally be challenging to rule out alcoholic liver disease since many individuals significantly underreport their alcohol consumption. The outlook • There is extremely little chance that steatosis may develop into chronic liver disease.
• About 10–15% of NASH cases progress to cirrhosis over the course of 8 years. • Compared to individuals with cirrhosis brought on by alcoholic liver disease, those with cirrhosis brought on by nonalcoholic fatty liver disease typically have a higher survival rate.
• The metabolic syndrome's hepatic manifestation, which includes hyperlipidemia, poor glucose tolerance, and central obesity. Simple steatosis (fatty liver), non-alcoholic steatohepatitis (NASH), and cirrhosis are among the disorders that fall under the umbrella of non-alcoholic fatty liver disease (NAFLD).
Epidemiology: Due to increased obesity rates, this condition is very frequent and is becoming more common. • The most frequent reason for abnormal liver function tests these days. • A large number of cirrhosis cases that were previously believed to be cryptogenic are now believed to be end-stage NAFLD. The cause • The most frequent correlations are between diabetes and obesity. • Also connected to parenteral feeding and some medications.
The pathogenesis • Obesity and insulin resistance appear to be the main contributing factors. • Hepatocyte damage and fat storage are brought on by insulin resistance. • Inflammation in reaction to hepatocyte damage causes fibrosis and, in certain cases, cirrhosis.
Presentation: Abnormal liver function tests are used to identify the majority of asymptomatic cases. • Cirrhosis-related problems can occasionally be seen in patients.
The macroscopy • Cirrhotic livers are diffusely nodular; the liver is swollen, squishy, and greasy.
The study of histopathology • NASH exhibits steatosis together with the presence of enlarged hepatocytes and neutrophils; steatosis is characterized by the buildup of fat within hepatocytes without discernible inflammatory activity. Depending on the disease's stage, there may be variable fibrosis. Keep in mind that these histology results are nearly the same as those observed in alcoholic liver disease. It can occasionally be challenging to rule out alcoholic liver disease since many individuals significantly underreport their alcohol consumption. The outlook • There is extremely little chance that steatosis may develop into chronic liver disease.
• About 10–15% of NASH cases progress to cirrhosis over the course of 8 years. • Compared to individuals with cirrhosis brought on by alcoholic liver disease, those with cirrhosis brought on by nonalcoholic fatty liver disease typically have a higher survival rate.
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Pathology - Autoimmune hepatitis
Definition: A hepatic disorder resulting from an autoimmune reaction directed at the liver.
Epidemiology: Rare occurrence. • Generally impacts middle-aged women. Aetiology • Definitively unknown, yet believed to be precipitated by infection or pharmacological agents.
Pathogenesis • Contemporary understanding posits that liver injury resulting from an infection or pharmacological agent leads genetically predisposed individuals to develop sensitization to their liver, subsequently eliciting an immune response against it.
Presentation • The majority of cases are asymptomatic during the initial stages, although may be identified accidentally through irregularities in liver function testing. • Certain patients exhibit delayed presentation with manifestations of chronic liver disease or end-stage cirrhosis. Approximately 25% of cases manifest abruptly with an episode of severe hepatitis accompanied by jaundice. Occasionally, significant acute liver injury manifests, resulting in abrupt hepatic failure in the patient.
Serology • Serum IgG levels are typically elevated. • Various autoantibodies may be detected, including anti-nuclear antibodies, liver-kidney microsomal antibodies, and smooth muscle antibodies.
Macroscopy • Limited macroscopic alterations, observed mostly in patients with cirrhosis or in instances of severe acute hepatitis characterized by extensive hepatocyte necrosis.
Histopathology • Chronic hepatitis exhibits a pattern of injury characterized by portal inflammation, interface hepatitis, lobular inflammation, and variable fibrosis. • Unlike chronic viral hepatitis, interface hepatitis and lobular inflammation are typically more pronounced. Plasma cells are frequently a prominent element of the inflammatory cell infiltrate.
Prognosis • The majority of cases exhibit favorable responses to immunosuppressive therapy. • The long-term prognosis is contingent upon the degree of fibrosis present in the liver at the time of diagnosis.
Definition: A hepatic disorder resulting from an autoimmune reaction directed at the liver.
Epidemiology: Rare occurrence. • Generally impacts middle-aged women. Aetiology • Definitively unknown, yet believed to be precipitated by infection or pharmacological agents.
Pathogenesis • Contemporary understanding posits that liver injury resulting from an infection or pharmacological agent leads genetically predisposed individuals to develop sensitization to their liver, subsequently eliciting an immune response against it.
Presentation • The majority of cases are asymptomatic during the initial stages, although may be identified accidentally through irregularities in liver function testing. • Certain patients exhibit delayed presentation with manifestations of chronic liver disease or end-stage cirrhosis. Approximately 25% of cases manifest abruptly with an episode of severe hepatitis accompanied by jaundice. Occasionally, significant acute liver injury manifests, resulting in abrupt hepatic failure in the patient.
Serology • Serum IgG levels are typically elevated. • Various autoantibodies may be detected, including anti-nuclear antibodies, liver-kidney microsomal antibodies, and smooth muscle antibodies.
Macroscopy • Limited macroscopic alterations, observed mostly in patients with cirrhosis or in instances of severe acute hepatitis characterized by extensive hepatocyte necrosis.
Histopathology • Chronic hepatitis exhibits a pattern of injury characterized by portal inflammation, interface hepatitis, lobular inflammation, and variable fibrosis. • Unlike chronic viral hepatitis, interface hepatitis and lobular inflammation are typically more pronounced. Plasma cells are frequently a prominent element of the inflammatory cell infiltrate.
Prognosis • The majority of cases exhibit favorable responses to immunosuppressive therapy. • The long-term prognosis is contingent upon the degree of fibrosis present in the liver at the time of diagnosis.
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Pathology - Primary biliary cirrhosis
Definition: A chronic liver disease with autoimmune destruction of tiny intrahepatic bile ducts and anti-mitochondrial antibodies.
Epidemiology • Uncommon. • Most common in middle-aged women and linked to other autoimmune disorders. The cause is unknown, but infections with organisms that resemble antigens on the biliary epithelium may be a contributing factor.
Pathogenesis: • Identified as an autoimmune disease when the immune system reacts abnormally to the biliary epithelium. Early stages of the disease may be asymptomatic, but high alkaline phosphatase values may indicate a diagnosis. Patients may experience weariness or pruritus owing to bile salt buildup. More than 95% of cases have anti-mitochondrial antibodies targeting a component of the pyruvate dehydrogenase complex in the inner mitochondrial matrix. Early disease exhibits few macroscopic changes in the liver, while advanced disease causes cirrhosis and bile staining.
Histopathology: • The earliest feature is the invasion and destruction of interlobular bile ducts by lymphocytes and macrophages, known as a 'fluid duct lesion'. Macrophages may form clusters and create granulomas. As the condition advances, it causes inflammation and loss of hepatocytes along the portal tract margins (interface hepatitis), leading to periportal fibrosis, portal-portal bridging, and cirrhosis.
Prognosis: Gradual progression to cirrhosis over 15-20 years. Ursodeoxycholic acid therapy slows the progression.
Definition: A chronic liver disease with autoimmune destruction of tiny intrahepatic bile ducts and anti-mitochondrial antibodies.
Epidemiology • Uncommon. • Most common in middle-aged women and linked to other autoimmune disorders. The cause is unknown, but infections with organisms that resemble antigens on the biliary epithelium may be a contributing factor.
Pathogenesis: • Identified as an autoimmune disease when the immune system reacts abnormally to the biliary epithelium. Early stages of the disease may be asymptomatic, but high alkaline phosphatase values may indicate a diagnosis. Patients may experience weariness or pruritus owing to bile salt buildup. More than 95% of cases have anti-mitochondrial antibodies targeting a component of the pyruvate dehydrogenase complex in the inner mitochondrial matrix. Early disease exhibits few macroscopic changes in the liver, while advanced disease causes cirrhosis and bile staining.
Histopathology: • The earliest feature is the invasion and destruction of interlobular bile ducts by lymphocytes and macrophages, known as a 'fluid duct lesion'. Macrophages may form clusters and create granulomas. As the condition advances, it causes inflammation and loss of hepatocytes along the portal tract margins (interface hepatitis), leading to periportal fibrosis, portal-portal bridging, and cirrhosis.
Prognosis: Gradual progression to cirrhosis over 15-20 years. Ursodeoxycholic acid therapy slows the progression.
