Introduction IgG4 related disease is now well recognised as a multisystem disease. This condition, originally discovered in Japan in 1995 is now being increasingly recognised in the Western World. Apart from diagnosis, treatment can also present as a challenge in a small group of patients. We report our experience from a tertiary referral hospital in North-East England.
Methods Data were obtained from retrospective case note review from 2005 to 2011. Only patients diagnosed with AIP and IgG4 disease based on accepted international criteria were included in the study.1
Results 16 patients were identified during this period. Mean age group was 64 years (Range 43–83 yrs). Male=15, Female=1. Abnormal LFTs were present in 62.5% of patients. Mean bilirubin was 97 mmols/l (range 4–354). Mean total IgG was 15.9 g/l (range 7.5–26.7). Mean IgG4 subclass levels was 6.4 g/l (Range 0.27–24.6). Pancreas was affected in 88% (15) and biliary abnormalities were seen in 62.5% (10) of the cases. Other organs noted to be involved were pericardium (1), retroperitoneum (2), gall bladder (2). Two patients had duodenal obstruction due to inflammation of the duodenum, stomach, peripancreatic area and the gall bladder bed. CT scan revealed enlarged head of pancreas (HOP) in nine patients (57%), extra-pancreatic mass in 4 (25%), extra hepatic bile duct involvement in 10 (62.5 %) and intrahepatic bile duct involvement in 9 (37.5%). 3 (18.75%) patients underwent ERCP and two had stenting of biliary strictures. EUS was performed in nine patients—showed enlarged HOP in 4 (44 %) and changes of chronic pancreatitis—4 (44 %). Nine patients (56%) had a raised serum IgG4. Diagnosis was made at surgery or by laparoscopic biopsy in 7 (44%) patients. Final diagnosis: Type I AIP in 15 patients (88%), Type II AIP in 1 (6%) and IgG4 cholangiopathy with no pancreatic involvement in 1 (6%). Other autoimmune diseases that were associated were Raynauds disease (1) and Sjogren's syndrome (1). Steroids were initiated in 12 (75%) patients (mean dose 37.5 mg). Disease relapsed in three patients (25%). Azathioprine was started on five patients. One patient was switched to 6MP due to side effects.
Conclusion Extrapancreatic disease, especially biliary structuring appears to be common. As this condition mimics malignancy, a combination of modalities were needed to arrive at a diagnosis. Relapse in not uncommon and a small group of patients will require additional immunosuppression for control of the disease.
Competing interests None declared.
Reference 1. Okazaki K, Kawa S, Kamisawa T, et al. Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol 2006;41:626–31.
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