Comorbid diseases of IgG4-related sialadenitis in the head and neck region.

OBJECTIVES/HYPOTHESIS: To further recognize the comorbid diseases of immunoglobulin G4-related sialadenitis (IgG4-RS) in the head and neck region and to observe the response of these conditions to immunomodulatory therapy.

STUDY DESIGN

Retrospective review.

METHODS

The symptoms of comorbid diseases and medical histories in 51 patients (24 men, 27 women; median age, 55 years) diagnosed with IgG4-RS were analyzed. Thirty-six patients received immunomodulatory therapy and were followed up for 10.4 ± 5.9 months. Computed tomography (CT) examination was performed before and after therapy.

RESULTS

Rhinosinusitis occurred in 58.8% patients, and manifested with the symptoms of nasal obstruction, nasal xerosis, and hyposmia. In addition, 43.1% patients had allergic rhinitis. Lymphadenopathy was identified in 74.5% patients. Lacrimal gland swelling occurred in 78.4% patients. Extraocular muscles, otologic organs, skin and superficial soft tissue, and cranial nerves were also involved. All of the lesions were relieved after immunomodulatory therapy. The Lund-Mackay scores decreased (9.6 ± 5.6 to 1.0 ± 2.2) according to CT analyses (P < .05). Mean CT volumes of the swollen lymph nodes and lacrimal glands decreased from 1.21 ± 0.61 cm(3) to 0.59 ± 0.35 cm(3) and from 2.25 ± 1.35 cm(3) to 0.70 ± 0.32 cm(3), respectively (P < .05) after treatment.

CONCLUSIONS

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. Immunomodulatory therapy could be effective in controlling both the comorbid diseases of IgG4-RS and sialadenitis of the major salivary glands.

LEVEL OF EVIDENCE