Definition
Chronic sclerosing sialadenitis occurs almost exclusively in the submandibular glands. Once thought to be a chronic inflammatory condition, attributed to inspissation of secretions or calculi, it is not recognised to be due to infiltration by IgG4-positive plasma cells. This condition affects a range of exocrine glands, including the pancreas. There is an association with rheumatoid arthritis and other autoimmune conditions.
Patients are usually middle-aged to elderly, with a slight male predominance.
This condition presents with a hard tumorous swelling of the submandibular gland, which may be bilateral. There may be a circulating eosinophilia.
The lobular architecture of the salivary gland is preserved. There is variation from lobule to lobule in the degree of involvement. The lymphoplasmacytic infiltrate begins around the salivary ducts, to be followed by periductal fibrosis. The ducts may contain inspissated secretions. There is progressive spread to the remainder of the gland. Reactive follicles are commonly present. There is an Obliterative phlebitis.
The lymphocytic infiltrate comprises predominantly CD8-positive cytotoxic T-cells3. Abundant IgG4-positive plasma cells are present. with an IgG4/IgG ratio in excess of 45%2.
Molecular studies
The T-cells may variously show a monoclonal, oligoclonal or a polyclonal pattern of TCRg rearrangement.
There is an excellent response to steroid therapy. Some cases progress to extranodal marginal zone lymphoma
1 Cheuk W,Chan JK. Advances in salivary gland pathology. Histopathology 2007; 51:1-20
3 Tiemann M, Teymoortash A, Schrader C, et al. Chronic sclerosing sialadenitis of the submandibular gland is mainly due to a T lymphocyte immune reaction. Mod Pathol 2002; 15:845-52 FULL TEXT
This page last revised 15.7.2007.
©SMUHT/PW Bishop