Abstract
IgG4-related disease is a new disease entity involving IgG4 in its clinical presentation and having 6 characteristic features: (1) systemic involvement; (2) solitary or multiple lesions showing diffuse or localized swelling, masses, nodules, and/or wall thickening on imaging; (3) high serum IgG4 concentration >135 mg/dL; (4) abundant infiltration of lymphoplasmacytes and IgG4-bearing plasma cells; (5) a positive response to corticosteroid therapy; and (6) complications of other IgG4-related diseases. To date, most IgG4-related diseases have been recognized as extrapancreatic lesions of autoimmune pancreatitis. This paper will discuss the utility of IgG4 as a biomarker of IgG4-related diseases, including in the diagnosis of autoimmune pancreatitis and its differentiation from pancreatic cancer, in the prediction of relapse, in the long-term follow-up of patients with autoimmune pancreatitis and normal or elevated IgG4 concentrations, and in patients with autoimmune pancreatitis and extrapancreatic lesions, as well as the role of IgG4 in the pathogenesis of IgG4-related disease.
Highlights
IgG4-related disease is a new disease entity involving IgG4 in its clinical presentation
6 characteristic features of IgG4-related disease have been identified: (1) systemic involvement; (2) solitary or multiple lesions showing diffuse or localized swelling, masses, nodules, and/or wall thickening on imaging; (3) high serum IgG4 concentrations >135 mg/dL; (4) abundant infiltration of lymphoplasmacytes and IgG4-bearing plasma cells; (5) a positive response to corticosteroid therapy; (6) complications of other IgG4related diseases [1,2,3,4]
Differences in sensitivity and specificity may be partly due to the use of different assays to measure serum IgG4 and different cutoffs for the upper limit of normal around the world, as well as variations in diagnostic criteria used in individual countries, which may be associated with histological differences between lymphoplasmacytic sclerosing pancreatitis (LPSP) [13] and idiopathic duct-centric chronic pancreatitis (IDCP) [14]
Summary
IgG4-related disease is a new disease entity involving IgG4 in its clinical presentation. 6 characteristic features of IgG4-related disease have been identified: (1) systemic involvement; (2) solitary or multiple lesions showing diffuse or localized swelling, masses, nodules, and/or wall thickening on imaging; (3) high serum IgG4 concentrations >135 mg/dL; (4) abundant infiltration of lymphoplasmacytes and IgG4-bearing plasma cells; (5) a positive response to corticosteroid therapy; (6) complications of other IgG4related diseases [1,2,3,4]. The major manifestations of IgG4-related disease include autoimmune pancreatitis, lacrimal and salivary gland lesions known as Mikulicz’s disease, sclerosing cholangitis, retroperitoneal fibrosis, lung disease, and tubulointerstitial nephritis. Many minor lesions have been reported in patients with IgG4-related disease, including hypophysitis, thyroiditis, hepatopathy, and prostatitis At present, it is not clear whether these lesions are caused by the same etiology or merely show clinical and pathological findings associated with IgG4. Topics will include IgG4 concentration and the diagnosis of autoimmune pancreatitis, as well as its differentiation from pancreatic cancer; IgG4 and the prediction of relapse; long-term followup of patients with autoimmune pancreatitis and either normal or elevated IgG4 concentration; IgG4 and extrapancreatic lesions in patients with autoimmune pancreatitis; and the role of IgG4 in the pathogenesis of IgG4-related disease
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