corneal chromophore
Introduction
Introduction The corneal color ring is one of the clinical manifestations of biliary cirrhosis. Biliary cirrhosis is caused by long-term intrahepatic biliary retention or more obstruction of the extrahepatic biliary tract. The former is called primary biliary cirrhosis, and the latter is called secondary biliary sclerosis. 90% of the disease occurs in women, especially in women aged 40 to 60 years old, male: female is 1:8. Early symptoms were only mild fatigue and intermittent itching, 1/2 with hepatomegaly, 1/4 with splenomegaly, and elevated serum alkaline phosphatase and -GT were often the only positive findings. End-stage manifestations of liver failure, variceal rupture, hepatic encephalopathy, ascites, edema with deep jaundice. Astragalus as the first performer accounted for 13%, such patients often have hepatosplenomegaly, may have jaundice, corneal color ring, liver palm, spider mites, scratches on the site of butterfly skin pigmentation, skin becomes thicker, change thick. Early patients can be treated with medication, and liver transplantation should be performed at the end of the period.
Cause
Cause
Cause
Biliary cirrhosis is divided into primary biliary cirrhosis (PBC) and secondary biliary cirrhosis (Secondary Biliary Cirrhosis). The latter is caused by long-term obstruction of the extrahepatic bile duct. PBC is generally considered to be an autoimmune disease. When lymphocytes are activated, they attack the middle and small bile ducts, causing an inflammatory reaction. Histologically, it resembles the host's rejection of the graft. There are many similarities in rejection with liver allografts. Clinically, the condition is relieved and worsened, often accompanied by other autoimmune diseases such as Sjogren's syndrome, systemic lupus erythematosus, rheumatoid arthritis and chronic lymphocytic thyroiditis. Humoral immunity was significantly abnormal, the anti-mitochondrial antibody positive rate was 90% to 100%, and 80% of patients had a titer greater than 1:80. Some people even included anti-mitochondrial antibody positive as a case when the PBC was studied. Some patients still have anti-nuclear antibodies, rheumatoid factors, thyroid antibodies, etc. These antibodies and the corresponding antigen can form a large immune complex, causing immune damage through the complement system.
pathology
The liver is swollen, pale green, smooth or showing fine particles and a hard texture. Tissue damage is roughly as follows: lymphocytes, plasma cell infiltration, IgM and immune complex deposition are the causes of granulomas. Bile regurgitation, bile duct injury and inflammation around the bile duct lead to bile duct destruction and small bile duct hyperplasia. The inflammation and scar formation around the portal area are separated, and the surrounding area is cholestatic and copper and iron deposits, further damage the liver cells, and the fiber becomes stretched. Causes cirrhosis of the liver. According to the occurrence and development of PBC, the pathological changes of PBC were divided into 4 stages.
The first phase, the cholangiocarcinoma, is characterized by chronic non-purulent inflammation of the bile duct or septal bile duct of the hepatic lobules, inflammatory cell infiltration in the lumen of the bile duct, the wall of the bile duct, and the surrounding. Mainly lymphocytes, plasma cells. The portal area is enlarged by inflammatory cell infiltration and has granuloma, but the liver cells and the boundary plate are normal.
In the second stage, the bile duct proliferative phase, due to the progressive destruction of chronic inflammation, replaced by fibrous tissue, most of the portal area is difficult to find interlobular bile duct, but there is atypical small bile duct hyperplasia, granuloma can still be seen in this period . The capillary bile duct around the hepatic lobules is extremely dilated, containing concentrated bile thrombus, rupture of the capillary bile duct, forming a bile paste, and the surrounding hepatocytes are swollen, and the cytoplasm is loose and translucent, ie feathery degeneration.
In the third stage, during the scar formation period, the collagen content in the portal area increased and the inflammatory cells and bile ducts decreased. Occasionally, the lymphoid follicles were associated with the germinal center. The medium-sized portal area was the most fibrotic, and the granuloma was not common. The fibers were separated from the portal area to another. A confluence area stretches, or extends to the hepatic lobules, due to the coexistence of debris-like necrosis and the deposition of cholestatic, iron and copper, causing damage to the liver cells, so that the boundary plate is blurred.
In the fourth stage, during the cirrhosis stage, the fibrous septum in the portal area expands and connects with each other, and the hepatic lobule is divided into pseudolobules. The regenerative nodules are visible, generally small nodular cirrhosis, or incompletely separable. Necrosis.
Examine
an examination
Related inspection
Venous gallbladder, cholangiography, corneal examination
Clinical manifestation
90% occur in women, especially in women aged 40 to 60 years old, male: female is 1:8. It has been reported that about 10% of the cases occur during pregnancy, and the onset of the disease is insidious and the onset is slow.
1. Early symptoms are only mild fatigue and intermittent itching, 1/2 have hepatomegaly, 1/4 have splenomegaly, and serum alkaline phosphatase and -GT are often the only positive findings. Light night pruritus as a first symptom of 47%. One in four patients have a feeling of fatigue and can cause depression, followed by itching. Astragalus as the first performer accounted for 13%, such patients often have hepatosplenomegaly, may have jaundice, corneal color ring, liver palm, spider mites, scratches on the site of butterfly skin pigmentation, skin becomes thicker, change Thick, may be related to scratches and vitamin A deficiency.
2, a small number of patients without jaundice serum cholesterol can be as high as 8g / L, palm, sputum, chest and back skin with nodular jaundice, also along the knee, elbow, gluteal muscle spasm, nerve sheath distribution, clubbing, long bone periostitis May be accompanied by pain and tenderness.
3. The appearance of clinical jaundice in the jaundice stage marks the beginning of the jaundice stage. The yellow time is often accompanied by osteoporosis, osteomalacia, vertebral compression, and even rib and long bone fractures, which are related to vitamin D metabolic disorders.
4, the terminal serum bilirubin rises linearly, the liver and spleen are swollen, itching, fatigue is aggravated. Signs of chronic liver disease are increasing, and patients with esophageal varices bleeding and ascites are increasing. Due to the deposition of copper, a few visible corneal pigment rings. Due to the lack of bile salts in the intestine, fat emulsification and malabsorption, fat diarrhea can occur, vitamin A, D, K malabsorption at this time, can produce night blindness, skin keratinization, bone changes and coagulation mechanism disorders. The cholangiography showed that the bile duct was normal and the bile duct was distorted. Finally, liver failure, variceal rupture, hepatic encephalopathy, ascites, edema with deep jaundice, often end-stage performance.
Accompanying diseases and related manifestations, 2/3 have connective tissue disease, autoimmune thyroiditis is also common, but also with scleroderma, calcinosis, Raynaud's phenomenon, 75% have dry keratoconjunctivitis, 35% have Asymptomatic bacteriuria, hypertrophic osteoarthrosis, 1/3 of pigmented gallstones, and membranous glomerulonephritis and renal tubular acidosis.
Auxiliary inspection
1, laboratory inspection
(1) The increase of blood bilirubin is mainly caused by direct bilirubin, and urinary bilirubin is positive. Because bilirubin is discharged from bile, the jejunal and urinary tract are reduced.
(2) Serum alkaline phosphatase increases the source of alkaline phosphatase is the bile duct epithelium, PBC can have a significant increase in alkaline phosphatase before the onset of clinical symptoms.
(3) The increase of blood lipids, especially phospholipids and cholesterol, is most obvious, and triglycerides can be increased normally or moderately.
(4) Liver function test Increased serum bile acid concentration, prolonged prothrombin time, can return to normal after early injection of vitamin K, and can not be corrected in advanced liver failure. Serum albumin is normal in the early and middle stages of the disease, and decreased in the late stage; globulin increases, mainly due to the increase of 2, and globulin.
(5) Immunological examination The positive rate of anti-mitochondrial antibodies can reach 90%100%, which can be used as an important reference for diagnosis. The positive rate of this antibody in chronic active hepatitis is 10% to 25%, and a small number of occult cirrhosis, systemic lupus erythematosus and rheumatoid arthritis can also be positive. One third of the patients had anti-cholangiocyte antibodies, a few had anti-smooth muscle antibodies and anti-nuclear antibodies, and 1/2 had rheumatoid factor positive. Serum IgM can be increased. The coexistence of anti-mitochondrial antibodies, alkaline phosphatase and IgM has a diagnostic significance for PBC.
2, cholangiography. Exclusion of extrahepatic biliary obstruction can be performed by intravenous, percutaneous transhepatic cholangiography or endoscopic retrograde cholangiography.
3. Liver biopsy. Live pathological examination, there is a diagnostic value, but if the cholestasis is serious, or bleeding tendency should be carefully considered, if necessary, laparotomy and biopsy.
Diagnose based on:
1, middle-aged women, the skin is obviously itchy, liver, yellow tumor;
2, serum total cholesterol increased significantly, serum bilirubin light, moderately elevated, alkaline phosphatase increased, bile acid concentration increased;
3. IgM is elevated, anti-mitochondrial antibody is positive and the titer is high. If you can get a piece of histological evidence, it will help to confirm the diagnosis.
Diagnosis
Differential diagnosis
The differential diagnosis of biliary cirrhosis includes obstructive jaundice, such as common bile duct stones, total cholangiocarcinoma, pancreatic head and bile duct stricture, etc. The main identification method is cholangiography. Should be identified with the following diseases:
1. Chronic active hepatitis Where anti-mitochondrial antibodies are positive, accompanied by cholestasis and histologically abnormal bile ducts, the slow-lived liver should be excluded first. The slow-lived liver and gallbladder type in China is more common than PBC. The effect of short-term corticosteroid therapy Helps to distinguish between the two diseases.
2, sclerosing cholangitis This disease is rare, mainly involving the bile duct, the above immunological markers are negative, and there are many bacterial infectious fever, biliary tract angiography can help identify.
3, drug-induced jaundice such as chlorpromazine, methyl testosterone, sulfonamide, arsenic preparations. Mostly caused by individual allergies, there is a history of medication, within a few weeks after taking the drug, jaundice can last for several years, often accompanied by elevated blood eosinophils, liver biopsy without typical PBC histology.
