In recent years, an immune-mediated disorder involving IgG4 has been described, which targets multiple organs and explains a number of disorders previously regarded as “idiopathic” or of unknown origin. Furthermore, the discovery of IgG4- related disease (IgG4-RD) has placed a number of pathologies within its spectrum, linking symptoms and conditions formerly considered isolated. Reports of the manifestations of IgG4-RD in the head and neck are scarce. Otological manifestations have been reported, but only a handful of cases are available in the literature. This is the first report of recalcitrant serous otitis media secondary to IgG4-RD, confirmed by immunohistopathology. A case of IgG4-RD of the middle ear is presented, manifesting itself as recalcitrant serous otitis media. The case is presented from an otolaryngological and histopathological perspective and briefly reviews this rare disorder. The importance of the awareness of IgG4-RD resides mainly in the fact that it is a treatable condition. This can potentially improve the quality of life of a number of patients, some of whom may not have had a clear diagnosis. A favorable response to glucocorticoids has been reported. In cases of persistent symptoms, immunosuppressive therapy has been used with success.
IgG4-related disease (IgG4-RD) is a recently described, immune-mediated, multi-organ (in 60%–90% of cases)1 condition. It has been misdiagnosed in the past due to its similarity to other pathologies and its widespread, unspecific, seemingly disjointed symptomatology. Its description has enabled the inclusion and connection of unspecific symptoms/disorders within its spectrum.2 At present, the pancreas, salivary and lacrimal glands seem to be the most affected organs.2–4 However, the numbers may be misleading, since many other forms of non-specific inflammation or autoimmune disorders may emerge in the future as new subclasses of IgG4-RD.
In otolaryngology, Mikulicz’s disease (recurrent dacryoadenitis and enlargement of the major salivary glands) and Küttner’s tumor (isolated, non-infectious, recurrent submandibular sialadenitis) are now widely recognized as part of the IgG4-RD spectrum because they share particular pathological, serological and clinical features (Table 1).5 Still, other forms of diffuse inflammation potentially leading to permanent tissue disruption in the head and neck such as recurrent mastoiditis, some forms of thyroiditis and of laryngeal and tracheal stenosis, among others, are being linked to IgG4-RD.10–12.