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Although patients with autoimmune pancreatitis (AIP) tend to have concurrent diverse disorders, very few studies have focused on diabetes mellitus (DM) coexisting with AIP. In total 102 AIP patients with DM were divided into three groups. Those with DM before the onset of AIP were labeled group A ( n = 35), those who developed DM and AIP simultaneously were labeled group B ( n = 58) and those who developed DM after steroid therapy for AIP were labeled group C ( n = 9). The characteristics of DM among the three groups were evaluated. No significant differences were noted in the age of DM onset among the three groups. However, the mean duration of DM was significantly longer in group A (8.7 years) than in groups B and C. AIP developed 6.8 years after DM onset in group A, whereas it developed 1.8 years after steroid therapy in group C. Group A had the highest rate (25.7%) of family members with a history of AIP. Levels of serum albumin, total cholesterol and triglyceride were significantly lower in group A. No correlations were found between glycated hemoglobin and benzoyl-tyrosyl para-aminobenzoic acid. Hypoglycemia was observed in 20% of patients under insulin therapy. Most of them were habitual drinkers and received no pancreatic enzymes. Group A showed a high prevalence of retinopathy, nephropathy and macrovascular disorders than group B. Aspects of AIP-associated pancreatic diabetes were clarified. AIP-associated DM must be controlled by a full assessment of the pancreatic endocrine and exocrine function. [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis (AIP) is a unique form of chronic pancreatitis characterised by a high serum IgG4 concentration and complications that include various extrapancreatic manifestations, one of which is sclerosing cholangitis. In AIP patients, infiltration of abundant IgG4-positive plasma cells and dense fibrosis are commonly observed in the pancreas and wall of the bile duct and gallbladder. The major symptom at onset of AIP is obstructive jaundice caused by stricture of the bile duct, and this requires differential diagnosis of AIP from pancreato-biliary malignancies and primary sclerosing cholangitis (PSC). Recently, there have been reports of particular cases of sclerosing cholangitis with a high serum IgG4 level and cholangiographic and pathological findings comparable to those observed in AIP patients. Being apparently different from PSC and similar to that in AIP, sclerosing cholangitis with and without AIP shows a clinical response to steroid therapy and thus is designated as ‘IgG4-associated sclerosing cholangitis’. The pathogenesis of AIP and IgG4-associated sclerosing cholangitis remains at yet undetermined. [Copyright &y& Elsevier]