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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.1-3 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.1-5 IgG4-related disease classically presents with autoimmune pancreatitis and sclerosing lesions in various organs such as the liver, gallbladder, kidney, lung, and lymph nodes.1-4IgG4-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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