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Clinical Problem Solving: Pathology
February 2013

Pathology Quiz Case 1: Diagnosis

Author Affiliations


JAMA Otolaryngol Head Neck Surg. 2013;139(2):194-195. doi:10.1001/jamaoto.2013.1268b

Diagnosis: IgG4-related sialadenitis as a presenting symptom of IgG4-related disease

IgG4-related sialadenitis, known previously in the literature as chronic sclerosing sialadenitis, or Küttner tumor, presents as unilateral or bilateral salivary gland enlargement that is inflammatory, extensively firm, and well defined.13 It most commonly affects the submandibular gland, although parotid gland involvement has been reported.2 It is typically diagnosed in middle-aged or elderly patients and is slightly more common in males.2,3 It is a rare disease with a differential diagnosis that includes sialolithiasis-associated sialadenitis,3 Sjögren syndrome, mucosa-associated lymphoid tumor, lymphoma, lymphoepithelial sialadenitis, and chronic sialadenitis, not otherwise specified.2 Although IgG4-related sialadenitis was thought to be a localized chronic inflammatory disease of unknown etiology for much of the past century, more recent studies have shown that it may be a feature of IgG4-related disease.15 IgG4-related disease classically presents with autoimmune pancreatitis and sclerosing lesions in various organs such as the liver, gallbladder, kidney, lung, and lymph nodes.14IgG4-related disease involvement of the head and neck has been much less commonly reported, with only 3 prior publications in the otolaryngology head and neck surgery literature (to our knowledge).1,6,7 IgG4-related sialadenitis was recently recognized as part of the spectrum of IgG4-related disease in both Asian and Western populations.1,2

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