lactoferrin and Hypergammaglobulinemia

Autoimmune pancreatitis (AIP) is a new clinical entity recently described. Histologically it consists of a lymphoplasmacytic infiltration with diffuse fibrosis. Pancreatic imaging on US, CT and ERCP shows diffuse enlargement of the pancreatic gland and an irregular narrowing of the pancreatic duct. Hypergammaglobulinemia, increased serum levels of total IgG or IgG4, positive antilactoferrin and anti carbonic anhydrase II autoantibodies are quite frequently found in autoimmune pancreatitis. Laboratory data, pancreatic images and diabetes mellitus improve under oral steroid therapy.

Topics: Age of Onset; Autoantibodies; Autoimmune Diseases; Carbonic Anhydrase II; Cholangiopancreatography, Endoscopic Retrograde; Female; Humans; Hypergammaglobulinemia; Lactoferrin; Male; Middle Aged; Pancreas; Pancreatitis, Chronic; Steroids

Several investigators have reported on autoimmune-related pancreatitis, but the clinical findings and pathophysiology still remain unclear. To clarify it, we analyzed eight patients with autoimmune pancreatitis.. We evaluated clinical findings in eight patients (four men and four women) with autoimmune-related pancreatitis. Patients were aged 45-73 yr (mean, 57.5 yr). We examined blood chemistry and immunological studies, including autoantibodies against lactoferrin or carbonic anhydrase II, and compared ERCP images with clinical findings. In two patients, we studied the subset of lymphocytes infiltrating in the pancreas by immunohistochemistry and flow cytometry.. Four of eight patients had jaundice, two had renal dysfunction, two had abdominal pain, and two had back pain. Three patients were complicated with other autoimmune diseases. Three patients showed abnormal pancreatic exocrine function by an N-benzoyl-L-tyrosyl-para-aminobenzoic acid excretion test. Antinuclear antibody was detected in four of eight patients, antilactoferrin antibody in three of six, anticarbonic anhydrase II antibody in two of six, antismooth muscle antibody in two of seven, and rheumatoid factor in one of eight. All eight patients showed segmental stenosis of the main pancreatic duct by ERCP. Four patients showed stenosis of the common bile duct as well as the pancreatic duct. Microscopic findings showed infiltration of CD4-positive lymphocytes around the pancreatic duct, and HLA-DR was expressed on both CD4-positive cells and pancreatic duct cells. In two patients, stenosis of the pancreatic duct improved by prednisolone.. Autoimmune mechanism may be involved in some patients with idiopathic pancreatitis associated with hypergammaglobulinemia.

Topics: Aged; Autoantibodies; Autoimmune Diseases; Carbonic Anhydrases; Cholangiopancreatography, Endoscopic Retrograde; Female; Flow Cytometry; Humans; Hypergammaglobulinemia; Lactoferrin; Lymphocyte Subsets; Male; Middle Aged; Pancreas; Pancreatitis