Clinical study on IgG4-related autoimmune pancreatitis / 中华风湿病学杂志

ABSTRACT

Objective To investigate the clinical features of IgG4-related autoimmune pancreatitis (IgG4-related AIP).Methods A prospective cohort study on IgG4 related disease (IgG4-RD) was carried out in Peking Union Medical College Hospital during December 2010 to June 2012,a total of 50 patients were recruited,including 14 IgG4-related AIP patients.The clinical manifestations,laboratory tests results,radiographic findings,histopatholo-gical lesions and response to treatment were analyzed.Results Fourteen AIP patients accounted for 28％ of IgG4-RD,of whom 9 cases were male,5 females,with the mean age of (55±10) (range 41-71) years.Painless obstructive jaundice was the major manifestation of AIP.All patients had extra-pancreatic organ involvement,including salivary glands enlargement (10 cases),lymph nodes swollen (7 cases),and bile duct tree (6 cases),lung (6 cases),kidney (2 cases),lacriminal gland (2 cases),retroperitoneal tissue (1 case),mesentery (1 case),and prostate (1 case) involvement.Serum levels of IgG4 subclass in all patients were significantly increased,with mean concentration of (11 ±10) (range 1.4-35.2) g/L.Radiographic findings showed diffuse (13 cases) or focal (1 case) pancreatic enlargement with irregular narrowing of the pancreatic duct (4 cases).Histopathological examinations were performed in 12 patients,which revealed massive infiltration with lymphocytes and IgG4 positive plasma cells with remarkable tissue fibrosis.Response to glucocorticosteroid or combined steroid and immunosuppressants were good.Conclusion IgG4-related AIP is a subtype of chronic pancreatitis,which is characterized by specific clinical spectrum,serological markers,radiographic and histopathological features.Glucocorticosteroid is the first choice,which can significantly improve the clinical and imaging abnormalities.