Síntomas otológicos como primera manifestación de la enfermedad relacionada con IgG4

Abstract

Introduction and objective: IgG4-Related Systemic Disease (IgG4-RSD) is a multisystem fibroinflammatory process characterized by multiorgan involvement with tumefactive lesions characterized by IgG4-positive plasma cell infiltrates, often together with elevated serum levels of the immunoglobulin Ig G4.This clinical entity was described in 2003 by Kamisawa et al. describing that autoimmune pancreatitis and its associated diseases could be related to a new clinical entity. Subsequently, IgG4-RSD has been recognized as a systemic process that includes organ conditions known as independent entities that share common findings. Although the main affected organs include the pancreas, kidneys, orbital structures, salivary glands and retroperitoneum, different otologic alterations have been described in the context of this disease.Our aim is to describe the otologic symptomatology that may constitute the initial presentation of the disease and contribute to an early diagnosis by the otolaryngologist. Methods: We conducted a systematic review of published cases of gG4-RSD with otologic involvement as the first presenting symptom of said disease, completing the search on April 31, 2021 in the following databases: Pubmed, NCBI, CochraneWeb of Science and Scopus. In the search we used MeSH terms and free words. The review was performed according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) criteria guidelines, including only patients with histological confirmation of disease. The following data were collected: age and sex of the patient, plasma IgG4 levels, time of evolution of symptoms until diagnosis and initial symptoms of IgG4-RSD, among others. Results: 37 articles were selected based on inclusion and exclusion criteria with otologic manifestations (46 patients). Of these a total of 29 patients debuted with otologic manifestations that were finally included in our study. The most frequent otologic manifestation of disease debut was the presence of a picture similar to serous otitis that did not respond to usual treatment. Other less frequent manifestations were the presence of hypertrophic pachymeningitis causing sensorineural hearing loss, tinnitus and vertigo, pseudotumoral lesions causing otologic symptoms due to infiltration of temporal bone structures or eosinophilic otitis. Also infrequent was the involvement of the disease to the auricular cartilage simulating a recurrent polychondritis. It may even be associated with the latter.In the radiological findings by CT/MRI, nonspecific middle ear occupation findings and lesions with pseudotumoral appearance are frequent. Conclusions: Otologic manifestations may be the first clinical manifestation of IgG4-RS. The most frequent forms of presentation at the otic level are treatment-resistant serous otitis (unilateral or bilateral)