OBJECTIVES/HYPOTHESIS: A new concept of IgG4-related sclerosing @entity7841 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 eight tissue samples from patients with IgG4-related sclerosing @entity7841 were studied. STUDY DESIGN: A retrospective clinical study at Yamagata University School of Medicine. METHODS: The @entity1 group consisted of six males and four females with an average age of 60 years (range, 47-74 years). Serum levels of IgG4 and the density of IgG4-positive plasmacytes in affected tissues were studied. RESULTS: All @entity1 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 @entity7841 with high IgG4/IgG ratios and prominent infiltration of IgG4-positive plasmacytes in the involved salivary glands had systemic complications, including @entity788 , @entity3807 , and/or inflammatory @entity6437 of the lung after swelling of the salivary glands. All six of these @entity1 were successfully treated with systemic corticosteroids. CONCLUSIONS: In the six @entity1 with systemic complications, treatment with systemic @entity288 reduced the @entity3359 and lowered serum IgG4 concentrations. These results suggest that IgG4 plays an important role in the pathogenesis of IgG4-related sclerosing @entity7841 , and that IgG4 levels and IgG4/IgG ratios may be used as additional indicators of disease activity and as biomarkers for potential life-threatening complications.

Clinical and pathological characteristics of IgG4-related sclerosing XXXX .

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity7841 :: ('MESH:D012793', 'Disease') :: ['sialadenitis'] @entity788 :: ('MESH:D010190', 'Disease') :: ['pancreatitis'] @entity3359 :: ('MESH:D012466', 'Disease') :: ['salivary gland enlargement'] @entity3807 :: ('MESH:D012185', 'Disease') :: ['retroperitoneal fibrosis'] @entity6437 :: ('MESH:D006104', 'Disease') :: ['pseudotumor'] @entity288 :: ('MESH:D000305', 'Chemical') :: ['corticosteroids']
@entity7841 :: (MESH:D012793,Disease) :: ['sialadenitis']
