Chronic sclerosing sialadenitis as one of the immunoglobulin G4-related diseases: a clinicopathological study of six cases from Central Europe
Abstract
Chronic sclerosing sialadenitis (CSS) has been recently proposed to be member of IgG4-related disease in Japanese and American series. The aim of our study was to validate these results in a cohort of European patients.
Our CSS-series includes 4 females and 2 males, aged 32-76 years, all presenting with unilateral swelling of submandibular gland. Microscopically, all CSS-cases showed similar morphology - preservation of lobular architecture accentuated by cellular fibrous bands, dense lymphoplasmacytic inflammation and varied acinar atrophy.
Ductal lymphocytes were detected in 3 cases. In 5 cases, the presence of intraductal secretory material accompanied by parenchymal neutrophils was observed.
Obliterative phlebitis was seen in 3 cases. The inflammatory infiltrate was composed of T- and B-lymphocytes and polyclonal plasma cells.
Median number of IgG-positive plasma cells per 1 HPF was 157; median number of IgG4-positive plasma cells per 1 HPF was 133. Median value of IgG4/IgG ratio was 0.84.
Ours is the first European series to prove that CSS belongs to the family of IgG4-related disease. Unlike prior studies, we found in CSS rarely described ductal lymphocytes and parenchymal neutrophils.
CSS displays consistent morphology, with increased numbers of IgG4-positive plasma cells and should be regarded as member of IgG4-related disease group.