Chronic sclerosing sialadenitis as one of the immunoglobulin G4-related diseases: a clinicopathological study of six cases from Central Europe.

Chronic sclerosing sialadenitis (CSS) has been proposed recently to be a member of the group of IgG4-related diseases in Japanese and American series. The aim of our study was to validate these results in a cohort of European patients. Our CSS series included four females and two males, aged 32-76 years, all presenting with unilateral swelling of submandibular gland. Microscopically, all CSS-cases showed similar morphology with preservation of lobular architecture accentuated by cellular fibrous bands, dense lymphoplasmacytic inflammation and varied acinar atrophy. Ductal lymphocytes were detected in three cases. In five cases, the presence of intraductal secretory material accompanied by parenchymal neutrophils was observed. Obliterative phlebitis was seen in three cases. The inflammatory infiltrate was composed of T and B lymphocytes and polyclonal plasma cells. The median number of IgG-positive plasma cells per high-power field (HPF) was 157; median number of IgG4-positive plasma cells per HPF was 133. Median value of the IgG4:IgG ratio was 0.84. This is the first European series to demonstrate that CSS belongs to the family of IgG4-related disease. Unlike previous studies, in CSS we found rarely described ductal lymphocytes and parenchymal neutrophils. CSS displays consistent morphology with increased numbers of IgG4-positive plasma cells, and should be regarded as a member of the IgG4-related disease group.

Laco J ，Ryska A ，Celakovsky P ，Dolezalova H ，Mottl R ，Tucek L ... -  《-》

Chronic sclerosing sialadenitis (Küttner tumor) is an IgG4-associated disease.

Geyer JT ，Ferry JA ，Harris NL ，Stone JH ，Zukerberg LR ，Lauwers GY ，Pilch BZ ，Deshpande V ... -  《-》

[IgG4 expression in chronic sclerosing submaxillaritis].

Sun K ，Wang LJ ，Yao HT ，Xiang H ，Ding W ... -  《-》

Idiopathic cervical fibrosis--a new member of IgG4-related sclerosing diseases: report of 4 cases, 1 complicated by composite lymphoma.

Cheuk W ，Tam FK ，Chan AN ，Luk IS ，Yuen AP ，Chan WK ，Hung TC ，Chan JK ... -  《-》

Clinical and pathological characteristics of IgG4-related sclerosing sialadenitis.

A new concept of IgG4-related sclerosing 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 eight tissue samples from patients with IgG4-related sclerosing sialadenitis were studied. A retrospective clinical study at Yamagata University School of Medicine. The patient 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. 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 six of these patients were successfully treated with systemic corticosteroids. In the six patients with systemic complications, treatment with systemic corticosteroids reduced the salivary gland enlargement and lowered serum IgG4 concentrations. These results suggest that IgG4 plays an important role in the pathogenesis of IgG4-related sclerosing sialadenitis, 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.

Ohta N ，Kurakami K ，Ishida A ，Furukawa T ，Saito F ，Kakehata S ，Izuhara K ... -  《-》