Clinical relevance of Küttner tumour and IgG4‐related dacryoadenitis and sialoadenitis

Abstract

ObjectivesKüttner tumour (KT), so-called chronic sclerosing sialoadenitis, is characterised by concomitant swelling of the submandibular glands secondary to strong lymphocytic infiltration and fibrosis independent of sialolith formation. However, recent studies have indicated that some patients with KT develop high serum levels of IgG4 and infiltration of IgG4-positive plasma cells, namely IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS), so-called Mikulicz's disease. The aim of this study was to clarify the clinical and pathological associations between KT and IgG4-DS.Materials and MethodsFifty-four patients pathologically diagnosed with KT or chronic sialoadenitis were divided into two groups according to the presence or absence of sialolith (KT-S (+) or KT-S (−), respectively).ResultsThere were no significant differences in the clinical findings, including the mean age, sex and disease duration, between the two groups. All patients in the KT-S (+) group showed unilateral swelling without infiltration of IgG4-positive plasma cells or a history of other IgG4-related diseases (IgG4-RD), while those in the KT-S (−) group showed bilateral swelling (37.5%), strong infiltration of IgG4-positive plasma cells (87.5%) and a history of other IgG4-RD (12.5%).ConclusionsThese results suggest an association between the pathogeneses of KT-S (−) and IgG4-DS, but not KT-S (+).

IgG4-related disease in the head and neck region: report of two cases and review of the literature

“…In the majority of studies with IgG4-related chronic sclerosing sialadenitis, patients were from either Japan or the United States [10,19,20]. Rare occurrences of the disease in Europe were confirmed in two retrospective studies evaluating the frequency of IgG4-RD in removed submandibular glands.…”

“…The mean age of patients with IgG4-related chronic sclerosing sialadenitis, according to various published series, ranges from 57 to 64.5 years [10,18,19,20]. The patient presented in Case 2 was younger, although Melo et al presented a case of IgG4-RD of the submandibular gland in an 11-year-old boy [21].…”

Cheilitis glandularis: Case report with respect to immunohistochemical findings

“…These authors identified IgG4-positive plasma cells in the glands, yet attributed this response to a non-specific chronic inflammatory reaction [ 29 ]. In order to better understand true Küttner cases, Furukawa et al [ 30 ] sorted out patients presenting with sialoliths in specimens. Interestingly, only patients with Küttner tumours without sialoliths showed bilateral swelling (37.5%), strong infiltration of IgG4-positive plasma cells (87.5%) and a history of other IgG4-related disease in a subset of affected individuals (12.5%) [ 30 ].…”

Immunoglobulin G4–Related Lymphadenopathy

“…5,6 IgG4-RD is a rare entity that most often occurs in older men. The estimated incidence in Japan for diagnosis of autoimmune pancreatitis is 0.28 to 1.08 per 100,000, 7 which likely underestimates the true incidence of all IgG4-RD.…”