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

Abstract

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.

Keywords: Immunoglobulin G4-related disease; Review; Rituximab; Subglottic stenosis.

Fig. 1

Cervical computed tomography images showing a thickening at the level of the first tracheal ring (arrows), with no apparent origin. a Axial plane. b Sagittal plane. c 3D reconstruction

Fig. 2

Rigid bronchoscopy image of larynx after dilation of the lumen

Fig. 3

Histopathological examination of the tracheal biopsy. a Note a submucosal inflammatory lymphoid nodular tissue destroying seromucous glands (Hematoxylin and Eosin [H&E] Stain, original magnification × 25). b The lymphoid infiltrate affects small arteries in an endarteritis obliterans pattern (arrow) (H&E Stain, original magnification × 100). c and d Immunohistochemistry performed with monoclonal antibodies against IgG4 (c) and IgG (d) demonstrates nodular aggregates of IgG4 + plasma cells in more than 25% of total IgG + plasma cells (Original magnification × 100)

Fig. 4

Timeline of diagnostic and therapeutic events of our patient. CT computed tomography, IgG4-RD Immunoglobulin G4-related disease, TB tuberculosis, RTX rituximab, RX simple radiography, w week(s)