Atypical presentation of immunoglobulin G4-related disease as subglottic stenosis: a case-based review

Abstract

Background Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory pathology that has been reported to affect principally the retroperitoneum, hepatobiliary system, salivary glands, orbital structures or lymph nodes. However, IgG4-RD with laryngeal involvement is a very rare entity. Our aims were to describe a case of subglottic stenosis as first and only manifestation of IgG4-RD and review the literature. A patient with IgG4-RD affecting the larynx that presented as subglottic stenosis is described. A MEDLINE database search of IgG4-RD cases with laryngopharyngeal manifestations was also conducted. A 30-year-old Caucasian woman was referred to a tertiary care hospital for dyspnea on exertion, which had been increasing for the last 4 months. Medical and surgical procedures revealed a subglottic stenosis, with a histological finding of IgG4 positive plasma cell infiltration. There was no evidence of other organ involvement. She was successfully treated with oral glucocorticoids and rituximab infusions. Glucocorticoids were rapidly tapered and the rituximab regimen was optimized, with no evidence of relapses. In the literature review, we found a total of 12 reported cases with laryngopharyngeal involvement, two of them with subglottic stenosis. IgG4-RD of the larynx is rare but should be considered after excluding more common disorders.

Immunoglobulin G4-Related Disease of The Larynx Mimicking Malignancy: A Case Report and Review of the Literature

“…Fibrosis was present in 71% of the cases. In one of the cases obliterative phlebitis was observed, while in another, marked eosinophilic infiltration was noted [4][5][6][7][8][9][10][11][12][13][14]. While lymphocytic infiltration rich in plasma cells, and fibrosis were evident in our case, obliterative phlebitis was not observed.…”

“…It was observed that patients presented with symptoms such as hoarseness, dysphonia, dysphagia, throat discomfort, cough, weight loss, odynophagia, dyspnea, and sore throat. Hoarseness was the most commonly seen complaint, as also our patient [4][5][6][7][8][9][10][11][12][13][14]. Although concomitant diseases vary, it was seen that one patient was found to be accompanied by retroperitoneal fibrosis which is in the spectrum of IgG4-RD.…”

“…IgG4-RD can be seen in any organ in the body, but in the larynx has been reported very few cases. When the literature was examined, it was observed that 14 patients, consisting of 9 case reports, had laryngeal IgG4-RD [4][5][6][7][8][9][10][11][12][13][14].…”

“…The IgG4-RD may infrequently involve a variety of head and neck sites primarily affecting the submandibular gland and less often seen in the thyroid gland, other salivary glands, lacrimal gland, sinonasal tract, oral cavity, orbit and lymph nodes [3]. Whereas, IgG4 RD cases identified in the larynx are very few [4][5][6][7][8][9][10][11][12][13][14].…”

Una inusual causa de estrechez de la vía aérea laringotraqueal

“…Con respecto al compromiso laríngeo, éste es muy poco frecuente, no conociendo su incidencia real. En la actualidad, existen solamente 12 casos reportados en la literatura, los cuales se describen en la Tabla 1 [8][9][10][11][12][13][14][15][16][17] .…”