Case report

北 京 大 学 人 民 医 院
l ’s Hos
t 3-year history of intermittent upper abdominal pain with jaundice.
3 years ago (Dec. 2006), the patient began to suffer from an upper abdominal pain, which would aggravate after fat meal or drinking. The pain would radiate to the back, with no hiccup or acid regurgitation. At the same time jaundice was found both in sclera and skin, accompanied by deeper color of urine, lighter color of feces and itches all over the body. No fever, nausea, vomiting, diarrhea or tarry stool was reported. He had a weight loss of 6 kg in 40 days and was admitted to our hospital for further treatment on De
Constant upper abdominal pain, which would relieve after taking stomach medicine, for more than 10 years Diabetes mellitus for 6 years, regularly taking metformin 0.5g tid and Gliquidone 30mg tid. Blood glucose Hypertension for 6 years, with the highest BP 140/90mmHg. He has been taking Fosinopril Sodium 1# Qd, and the blood pressure has been controlled well.
Denied any history or contact history of hepatitis. Denied long-term use of NSAIDs.
Had smoked 20 cigarettes a day for 20 years.
Had been drinking 36 gram of alcohol per day for more than 10 years, have quitted for 3 years.
E Jaundice of the skin and sclera could be found. Abdomen was soft, with light tenderness in the upper
part. Liver: palpable, 2cm below costal margin. Murphy’s sign: (+). Percussion pain of the liver (+).
ALT 283U/L (0-40 U/L), AST 113 U/L (0-40 U/L), ALP 261 U/L (45-132 U/L), GGT 294 U/L (0-60 U/L), T-Bil 118.9umol/L (1.7-25.7 umol/L), D-Bil 84.0 umol/L (1.7-6.8 umol/L); 北 京 大 学 人 民 医 院
l ’s Hos
HAV-Ab, HEV-Ab, anti-EBV, anti-CMV, ANCA, ANA, APCA, SMA, AMA: (-).
Abdominal CT: Mild expansion of intrahepatic bile duct; incrassation of the wall of gallbladder; expansion of upper common bile duct, with a sudden stenosis at the level of head of pancreas; general enlargement of the pancreas with coarse edge, no expansion of pancreatic duct.
MRCP: Obstruction of lower bile duct, expansion of both intrahepatic and extrahepatic bile duct, the obstruction located at the level of the head of pancreas, possible chronic inflammation. Possible diagnosis: Abdominal ultrasound: Size and morphology of the liver normal, with wild range of expansion of intrahepatic bile duct; gallbladder size normal, with cholestasis; obvious expansion of common bile duct, 19mm at the widest; diffuse enlargement of the pancreas, with low level echo of the essence.
ERCP: Ductus pancreaticus radiography: slightly stiffed pancreatic duct. Bile duct radiography: expansion of common bile duct, with stenosis at the end; the wall was smooth, with no filling defect; the large possibility was inflammation. Biliary stent drainage was embeded.
The results of our hospital: Cholestasis in part of the liver cell and micro bile duct, with formation of micro bile thrombus; vacuolar degeneration of a small number of the liver cells; focal inflammation cell Consultation of Peking University First Hospital: Severe cholestasis in lobular multifocal liver cell and micro bile duct, with formation of micro bile thrombus. No obvious expansion of centrilobular vein.
Irregular portal area was discovered, with scattered infiltration of lymphocytes.
Consultation of Ditan Hospital: (hepatic) Bile duct obstruction lesion. Drug-induced and autoimmune liver 北 京 大 学 人 民 医 院
l ’s Hos
(The relationship between bilirubin level and dose of steroid: See Table 1 ) Also, during the whole time, metformin 0.5g tid and Gliguidone 30mg tid was used to control blood glucose. The blood glucose was controlled well.
1 Enlagement of pancreas, cause to be determined 2 Cholestatic liver disease, cause to be determined After discharge from hospital, the patient kept taking Ursodeoxycholic Acid 750mg/d. The dosage of Prednisolone was gradually reduced from 25mg/d. In March, 2007, the patient stopped taking steroid, the whole therapy lasted for 75days. CT scan (Mar. 8th, 2007): Slight enlargement of the pancreas, smaller compared to Dec. 11th, 2006, with smooth edge. No abnormal density was seen inside and no expansion of pancreatic duct was found. No lymphadenopathy was found in abdominal cavity. Endoscopic removal of (plastic) bile duct cartridge was performed on Mar. 23th, 2007.
北 京 大 学 人 民 医 院
l ’s Hos
In Aug. 2007, five months after stopped taking steroid, the patient had a recurrence of jaundice in both sclera and skin, with deeper color of urine and itching of the skin, with intermittent right upper abdominal pain. He was admitted to our ward on Aug. 20th, 2007.
E Jaundice in both sclera and skin. Liver: not palpable below costal margin, palpable 2cm below the
ALP 306U/L，GGT 79U/L，T-Bil 69.2umol/L，D-Bil 66.1umol/L; Protein electrophoresis: Alb 52.4% （ 55.8-66.1% ）， α 1 globin 5.2% （ 2.9-4.9% ）， β 1 globin 7.3% （4.7-7.2%），β2 globin 7.6%（3.2-6.5%），rest was normal； Complement C3, C4, CRP, RF, ASO, immunoglobin: (-); Anti ds-DNA, ANCA, ANA, APCA, SMA, AMA : (-).
Abdominal CT: 1 Enlargement of pancreas, expansion of pancreatic duct at the tail with pancreatic head cysts, chronic pancreatitis was considered; 2 stenosis of the lower bile duct, chronic pancreatitis should be considered as differential diagnosis; 3 chronic cholesystitis not exluded.
Abdominal ultrasound: Mild expansion of wide range of intrahepatic bile duct, tertiary bile duct 2.5mm; the size of gallbladder normal, whole common bile duct obviously expanded, 16mm at the widest, pressed at the level of head of pancreas. Diffuse pancreas cancer could not be excluded.
ERCP: Row-like stenosis of lower part of common bile duct, about 2-3cm long, upper end expanded to 2.6cm; slim, stiff main pancreatic duct, with rough wall. Endoscopic biliary stent drainage was performed.
北 京 大 学 人 民 医 院
l ’s Hos
Extrahepatic obstructive jaundice was considered, with a high possibility of autoimmune pancreatitis.
Using Ursodeoxycholic Acid 750mg/d for treatment. The patient refused to take a pancreas biopsy, so Prednisolone 40mg/d was added since 2007-8-31. The symptoms were eased and bilirubin level was decreased from T-Bil 47umol/L, D-Bil 41.2umol/L (Aug, 31st) to T-Bil 34.6umol/L, D-Bil 28.5umol/L (Sep.
4th). (See Table 2) 4 days after using steroid, there was an elevation of blood glucose level, oral administration was not satisfied, so insulin 36U/d was used to control blood glucose. The patient was 2 Obstructive jaundice, post endoscopic biliary stent drainage 4 Hypertension (normal after drug using, extremely high risk) After discharge, the patient took Prednisolone 40mg/d, and Ursodeoxycholic Acid was taken irregularly.
Bilirubin, ALP, GGT level was normal. 4 months later, the quantity of steroid was reduced gradually, and the bilirubin level remained normal. When the dosage of steroid was reduced to 15mg/d, the ALP was elevated, so steroid was increased to 20mg/d, and the patient was told to take Ursodeoxycholic Acid regularly. Nov. 2008, the removal of cartridge was performed. In Oct. 2009 (25 months after using steroid), steroid was reduced to 7.5mg/d, and the patient again had an elevated ALP. So the steroid was increased to 10mg/d, Ursodeoxycholic Acid 1000mg/d. (See. Table 3) Prednisolone 10mg Qd, Ursodeoxycholic Acid 500mg Bid, Insulin 30U/d.
北 京 大 学 人 民 医 院
l ’s Hos
Table 1：The relationship between bilirubin level and dose of steroid during the 1st hospitalization Table 1 (continued) ：The relationship between bilirubin level and dose of steroid during the 1st hospitalization 北 京 大 学 人 民 医 院
l ’s Hos
Table 2：The relationship between T-Bil, D-Bil, ALP and GGT and dose of steroid during the 2nd hospitalization 北 京 大 学 人 民 医 院
l ’s Hos
Table 3：The relationship between ALP, GGT and dose of drugs during outpatient follow-up Table 3 (continued)：The relationship between ALP, GGT and dose of drugs during outpatient follow-up

Source: http://www.pkuph.cn/mass/ksjs/xhk/conference/immu/2010/file/Autoimmune-pancreatitis-case.pdf