Case Series and Systematic Review of IgG4-Related Diseases in the Head and Neck

Abstract

Objectives:

Immunoglobulin G4-related disease (IgG4-RD) is a poorly understood chronic inflammatory disorder affecting the middle aged and elderly that can present to the otolaryngologist. We aim to summarize the current literature by performing a systematic review as well as describe our institutional experience with 8 patients.

Methods:

Our search used Pubmed, Ovid, and Scopus using the keywords IgG4 related disease, head and neck, orbit, salivary glands, sialadenitis, Kuttner, angiocentric eosinophilic fibrosis, submandibular, lacrimal, thyroid, dacryoadenitis, nasal, sinus, paranasal, and Mikulicz’s. Original research and review articles published in English from 1964 to 2013 whose major topic was IgG4-RD affecting the head and neck were included. Data regarding the various presentations and management of IgG4-RD were summarized. Additionally, we present our own experience with a series of 8 patients diagnosed with this condition.

Results:

One hundred twenty-four articles met our inclusion criteria. IgG4-RD most often presents as a mass lesion in the head and neck region. Common diagnostic features include: (1) elevated serum IgG4 level, (2) marked infiltration of exocrine glands by IgG4-positive plasma cells with fibrosis, and (3) marked improvement with corticosteroid therapy. Early diagnosis and involvement of rheumatology is important in management.

Conclusions:

IgG4-RD is a challenging nonsurgical disease that has multiple manifestations in the head and neck. It must be distinguished from various mimics including malignancy, systemic diseases, and infectious. Otolaryngologists should be aware of this condition and its management.