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Spontaneous Remission Of Autoimmune Pancreatitis: Four Case Reports

Zhang, B. B. 2022Other/Unknown

Zhang, B. B., Huo, J. W., Yang, Z. H., Wang, Z. C., & Jin, E. H. (2022). Spontaneous remission of autoimmune pancreatitis: Four case reports. World journal of clinical cases, 10(23), 8232–8241. https://doi.org/10.12998/wjcc.v10.i23.8232

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Abstract

BACKGROUND: Autoimmune pancreatitis (AIP) is a particular type of chronic pancreatitis, and steroid treatment of AIP is effective. Spontaneous remission (SR) of AIP without steroids is relatively rare. The international consensus for the treatment of autoimmune pancreatitis suggests that patients with AIP with obstructive jaundice, abdominal pain, and back pain related to the pancreas or the bile duct should be treated with steroids; most asymptomatic patients with AIP may improve without steroids. However, in our clinical work, we found that the clinical characteristics of AIP patients with SR vary. Four of these cases are described here. In addition, to our knowledge, there is no previously published report of dynamic imaging before and after SR of AIP at present. CASE SUMMARY: We present the cases of four patients with AIP (two females and two males) in which the AIP improved spontaneously without steroid treatment. Two patients were asymptomatic, one patient had abdominal pain with obstructive jaundice, and one patient had intermittent right upper abdominal pain. Three patients presented with localized pancreatic enlargement and one with diffuse pancreatic enlargement. In addition to the pancreatic lesions, bile duct involvement was seen in two patients, and no extra-pancreatic organ involvement was found in the other two patients. The serum IgG4 level of all patients was more than twice the normal level. After SR in the four patients, the affected pancreases exhibited three types of image features: Return to normal, progressive fibrosis, and atrophy and calcification. CONCLUSION: The clinical features of SR in our four patients with AIP differ, but the imaging findings share some characteristics. After SR, in some cases the affected pancreas could return to normal, although some patients suffer from progressive fibrosis and atrophy as well as calcification.

Case Details

Disease Location

Pancreas

Personal Characteristics

78-year-old man with a history of hypertension for more than 7 years; he took 40 mg nimodipine three times a day. He also had abnormal liver function in the last 2 years, as well as chronic gastritis in the past year without treatment. He drank about 10 g of alcohol per day for more than 40 years.

Clinical Characteristics

Six days prior to examination, this patient had right upper abdominal pain without an obvious cause, accompanied by intermittent vomiting. He went to a local hospital where an abdominal ultrasound showed mild dilatation of the common bile duct and a solid mass in the pancreatic head. Abdominal contrast-enhanced CT showed that the pancreatic head was slightly enlarged with delayed enhancement. The bile duct wall in the pancreatic head was thickened and enhanced abnormally. Magnetic resonance cholangiopancreatography showed that the main pancreatic duct was narrowed in a longer segment, and the upstream pancreatic duct was slightly dilated, with a diameter of 4 mm. Moreover, the common bile duct of the pancreatic segment was severely narrowed, and the proximal intra- and extra-hepatic bile ducts were dilated. Biopsy showed degenerative small round cells were seen, but no malignant tumor cells were found. The patient was diagnosed with focal aip of the pancreatic head. He was treated with liquid food, acid suppression, and liver protection. After discharge, he received ursodeoxycholic acid (50 mg) and pancreatin enteric-coated capsules (50 mg) three times a day.

Remission Characteristics

1 year later, the pancreatic head swelling had dissipated, and it appeared to contain multiple punctate calcifications

Treatment & Mechanisms

Clinical Treatment

Biopsy