Open AccessIgG4‐Related Sialadenitis: Pearls and Pitfalls in Management
Author(s) -
Ohta Nobuo,
Kurakami Kazuya,
Suzuki Yusuke,
Ishida Akihiro,
Kakehata Seiji
Publication year - 2012
Publication title -
otolaryngology–head and neck surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.232
H-Index - 121
eISSN - 1097-6817
pISSN - 0194-5998
DOI - 10.1177/0194599812451426a38
Subject(s) - sialadenitis , autoimmune pancreatitis , medicine , igg4 related disease , infiltration (hvac) , fibrosis , pancreatitis , pathology , salivary gland , submandibular gland , gastroenterology , physics , thermodynamics
Objective A new concept of IgG4‐related sialadenitis characterized by high serum IgG4 levels and tissue infiltration of IgG4‐expressing plasmacytes has recently been proposed. To determine appropriate serum levels of IgG4 for monitoring disease activity, a total of 36 serum samples and 8 tissue samples from patients with IgG4‐related sialadenitis were studied. Method The patient group consisted of 6 men and 4 women with an average age of 60 years (range, 47 to 74 years). Serum levels of IgG4 and the density of IgG4‐positive plasmacytes in affected tissues were studied. Results All patients had elevated serum IgG4 levels (>135 mg/dL), and IgG4‐positive plasmacytes (IgG4+ plasma cells/IgG+ plasma cells >50%) were observed in the involved salivary glands. Six patients with IgG4‐related sclerosing sialadenitis with high IgG4/IgG ratios and prominent infiltration of IgG4‐positive plasmacytes in the involved salivary glands had systemic complications, including pancreatitis, retroperitoneal fibrosis, and/or inflammatory pseudotumor of the lung after swelling of the salivary glands. All 6 of these patients were successfully treated with systemic corticosteroids. Conclusion In the 6 patients with systemic complications, treatment with systemic corticosteroids reduced the salivary gland enlargement and lowered serum IgG4 concentrations. These results suggest that IgG4 levels and IgG4/IgG ratios may be used as additional indicators of disease activity and as biomarkers for potential life‐threatening complications.