IgG4-related disease in the abdomen: a great mimicker.

IgG4-related disease is a systemic disorder that can involve various abdominal organs. Abdominal manifestations include autoimmune pancreatitis, sclerosing cholangitis, gallbladder pseudotumours, multifocal renal abnormalities, retroperitoneal fibrosis, and sclerosing mesenteritis. Radiological appearances of the IgG4-related disease often resemble malignant tumors and other inflammatory processes, potentially leading to unnecessary invasive procedures. Moreover, a timely diagnosis of IgG4-related disease leads to an effective treatment with steroids. Therefore, it is important to understand the spectrum of imaging manifestations of IgG4-related disease and their differentiating features from other diagnoses.

IgG4 related disease - a retrospective descriptive study highlighting Canadian experiences in diagnosis and management.

BACKGROUND: Appreciating the utility of published diagnostic criteria for autoimmune pancreatitis, when compared to the characteristics of patients clinically managed as having disease, informs and refines ongoing clinical practice. METHODS: Comparative retrospective descriptive evaluation of patients with autoimmune pancreatitis including dedicated radiology review. RESULTS: 66 subjects with radiographic OR clinical features of autoimmune pancreatitis were initially identifiable (Male: n = 50), with 55 confirmed for evaluation. The most common presentation included pain (67%), weight loss (65%), and jaundice (62%). Diffuse enlargement of the pancreas was evident in 38%, whilst multifocal, focal, or atrophic changes were seen in 7%, 33% and 9% respectively. 13% had no pancreatic parenchymal involvement. Peripheral rim enhancement was seen in 23 patients (42%). Where discernible, disease was a) Sclerosing pancreatitis and cholangitis, n = 21; b) Sclerosing cholangitis, n = 9; c) Sclerosing pancreatitis, n = 4; d) Sclerosing pancreatitis and cholangitis with pancreatic pseudotumour, n = 7; e) Sclerosing cholangitis with hepatic pseudotumour, n = 3; f) Sclerosing pancreatitis with pancreatic pseudotumour, n = 1. 56% of the patients had systemic manifestations and the median serum IgG4 at diagnosis was 5.12 g/L. The Korean criteria identified most patients (82%) compared to HISORt (55%) or the Japan Pancreas Society (56%). The majority (HISORt 60%; Japan Pancreas Society 55%; Korean 58%) met diagnostic criterion by radiological findings and elevated serum IgG4. Treatment and response did not differ when stratified by diagnostic criteria. CONCLUSION: Our descriptive and retrospective dataset confirms that in non-expert practice settings, autoimmune pancreatitis scoring systems with a focus on radiology and serology capture most patients who are clinically felt to have disease.