What comorbid diseases of immunoglobulin G4-related sialadenitis (IgG4-RS) in the head and neck region may develop, and how do they respond to immunomodulatory therapy?
Background: IgG4-RS is a newly recognized systemic immune-mediated disease characterized by dense lymphoplasmacytic infiltrates, storiform fibrosis, and elevated serum IgG4 levels. More than 40 different organs have reportedly been involved in IgG4-RS. Salivary gland involvement is the most common head and neck lesion, but the frequency of ocular adnexal, sinonasal cavity, ear, lymph node, and cranial nerve involvement is unclear.
Explore this issue:
September 2015Study design: Retrospective review of 51 patients diagnosed with IgG4-RS between August 2011 and November 2014.
Setting: Department of Oral and Maxillofacial Surgery at Peking University School of Stomatology, Peking, China.
Synopsis: Thirty patients complained of rhinosinusitis, with main symptoms of nasal obstruction, nasal xerosis, and hyposmia. Of these, 22 also had allergic rhinitis.
On CT analyses, 34 patients showed different degrees of sinonasal mucosa thickening, and lymphadenopathy was present in 38 patients, with the submandibular lymph nodes and superior deep cervical lymph nodes most commonly involved. Fifteen patients had lymph node swelling. Physical examinations revealed 14 patients with cervical lymph node swelling, mainly in the submandibular region. Two patients had lymph node swellings in the parotid gland tail. Ocular adnexal involvement, mainly eyelid swelling, was common. Palpation revealed bilateral and unilateral lacrimal gland enlargement in 33 patients and one patient, respectively; CT showed bilateral and unilateral lacrimal gland enlargement in 35 and five patients, respectively. Nine patients reported decreased audition; another two complained of dysaudia. Zygomatic and paraorbital region swelling was observed in one patient.
In all, 36 patients received immunomodulatory therapy. Besides the decreased volume of the involved salivary glands, all patients with rhinosinusitis reported experiencing a remission of their discomfort and regaining their olfactory sensation within seven days of treatment.
Bottom line: IgG4-RS could potentially develop with involvement of ocular adnexa, sinonasal cavities, ears, lymph nodes, skin and superficial soft tissue, and cranial nerves in the head and neck region.
Citation: Hong X, Sun ZP, Li W, et al. Comorbid diseases of IgG4-related sialadenitis in the head and neck region. Laryngoscope. 2015;125:2113–2118.