Igg4-associated chronic sclerosing sialadenitis K11.8

IgG4-associated chronic sclerosing sialadenitis, also known as Küttner tumor, is considered a partial manifestation of IgG4-associated diseases (Putra J et al.2016). The salivary gland inflammation can occur monoorganically, but also as an initial symptom of an autoimmunological, IgG4-associated multisystem disease. The chronic inflammation of the submandibular gland leads to a chronic swelling of the gland(s), fibrosis and induration. Later on, parenchyma shrinkage, calcification and ganglionic reflux occur.

Medieval and older adults; m>w (Wei TW et al. 2015)

It impresses with a very rough, shiftable and indolent mass in the caudal area of the lower jaw, mostly unilaterally, rarely bilaterally. It is clinically reminiscent of a malignant tumor.

IgG4 serum levels may be elevated.

Salivary gland tissue predominantly atrophic, occasionally still recognizable mucinous (light) and serous (purple) end pieces (Glandula submandibularis). Dense lymphoplasmacellular inflammatory infiltrate with formations of lymph follicles, storiform fibrosis, obliterative phlebitis (Tanaka K et al. 2015). The IgG4/IgG positive plasma cell ratio >40 % is an important diagnostic parameter (Culver EL et al. 2015).

Extranodal marginal zone lymphoma, Sjögren's syndrome, lymphoepithelial sialadenitis.

Long-term therapy with glucocorticoids, if necessary combined with "steroid-saving" immunosuppressive drugs (Hong X et al. 2018).

The chronic sclerosing sialadenitis of the submandibular glandula is a rare disease. The relative frequency is low at 2% compared to all - especially obstructive sialadenitis.

1. Bledsoe JR et al (2018) IgG4-related disease: review of the histopathologic features, differentialdiagnosis, and therapeutic approach. APMIS 126:459 476.
2. Culver EL et al (2015) Immunoglobulin G4 related chronic sclerosing sialadenitis. J Laryngol Otol 129:226-231.
3. Hong X et al (2018) Treatment of immunoglobulin G4 related sialadenitis: outcomes of glucocorticoid therapy combined with steroid-sparing agents. Arthritis Res Ther 20:12.
4. Putra J et al (2016) Küttner tumor: IgG4-Related Disease of the Submandibular Gland. Head Neck Pathol 10:530-532.
5. Tanaka K et al (2015) Chronic Sclerosing Sialadenitis of the Submandibular Gland as the Initial Symptom of IgG4-Related Disease: A Case Report. Tohoku J Exp Med 236:193-8.
6. Wei TW et al (2015) Chronic sclerosing sialadenitis of the submandibular gland: an entity of IgG4-related sclerosing disease. Int J Clin Exp Pathol 8:8628-8631.