Autoimmune pancreatitis as a component of autoimmune polyglandular syndrome

Autoimmune pancreatitis (AIP) is a rarely diagnosed autoimmune illness with a characteristic clinical, histological, and radiological image [1]. The frequency of AIP occurrence is estimated to be 1.9–4%; however, this illness must be differentiated from the pancreatitis [2, 3].
The illness has two histological clinical sub-types: type 1: lymphoplasmacytic sclerosing pancreatitis (LPSP) – it accompanies other autoimmune illnesses: primary sclerosing cholangitis, primary biliary cirrhosis, Sjögren syndrome, inflammatory bowel disease, diabetes type 1; type 2: idiopathic duct-centric pancreatitis (IDCP) – isolated autoimmune pancreatitis [4, 5].
Recent reports indicate that patients with diagnosed autoimmune pancreatitis are more prone to develop ulcerative colitis (CU) as was suggested by Korean scientists [6]. The diagnosis can be based on the following diagnostic criteria for autoimmune pancreatitis [7]. It has been postulated in recent years that autoimmune pancreatitis belongs to the systemic disease group related to IgG4, otherwise called the IgG4-associated systemic disease (ISD)/IgG4-related sclerosing disease [8]. Extrapancreatic lesions occur in 80% of AIP patients and include sclerosing cholangitis (58%), lymphadenopathy recess lung (28%), inflammation of the salivary glands and tear ducts (15–39%), interstitial pneumonia (8–13%), retroperitoneal fibrosis, and tubulointerstitial nephritis. Elevated levels of IgG4 in plasma cells have also been reported in pseudoinflammatory liver, breast, mediastinum, orbit tumours, and in the pituitary gland and prostate inflammation [9, 10].
Due to the fact that autoimmune pancreatitis type 1 (LPSP) may be accompanied by other autoimmune diseases, AIP may be one of the autoimmune polyendocrinopathies, autoimmune polyglandular syndrome (APS) type 1, 2, 3, and 4 [11].
In every person suffering from autoimmune disease, particularly of the endocrine glands, the possibility of APS occurrence needs to be considered in the differential diagnosis, testing for the possible coexistence of diabetes type 1, thyroid dysfunction, Addison’s disease, celiac disease, and autoimmune hepatitis, also smooth muscle, and antigens LMC-1 and SLA is recommended [12, 13].
The patient, aged 72 years, was first interviewed in 2006 because of obstructive jaundice, which occurred in the course of the pancreatic head tumour diagnosis based on magnetic resonance cholangiopancreatography (MRCP) and endoscopic...


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