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

Published on Apr 8, 2021in Rheumatology International1.984
· DOI :10.1007/S00296-021-04816-4
Belén Atienza-Mateo7
Estimated H-index: 7
Teresa Díaz de Terán-López + 5 AuthorsMiguel A. González-Gay85
Estimated H-index: 85
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.
#1Azfar Syed (Madigan Army Medical Center)H-Index: 1
#2Rhonda E. Colombo (Madigan Army Medical Center)H-Index: 1
Last. Peter M Henning (Madigan Army Medical Center)H-Index: 1
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IgG4-related autoimmune diseases (IgG4 RD) are a relatively recently recognised group of disease processes that can affect multiple organ systems and result in protean symptoms. Here, we present a rare case of a 69-year-old man with a history of IgG4 RD affecting his lacrimal gland and pancreas who developed symptoms of severe laryngitis not responsive to usual therapy. He presented with non-productive cough, hoarseness and dyspnoea. Imaging findings suggestive of aortitis and laryngeal inflamma...
1 CitationsSource
1 CitationsSource
Last. Luis Caminal-MonteroH-Index: 13
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Resumen La enfermedad relacionada con IgG4 (ER-IgG4) se caracteriza por un infiltrado linfoplasmocitico rico en celulas plasmaticas IgG4 positivas, fibrosis estoriforme y flebitis obliterativa. Se puede presentar como seudotumor orbitario, parotidomegalia, nefritis tubulo intersticial, fibrosis retroperitoneal o pancreatitis, aunque practicamente cualquier organo puede verse afectado. Presentamos el caso de una mujer de 37 anos, que presenta un cuadro de disfonia severa y aftosis oral dolorosa r...
#2Faustino Núñez-Batalla (University of Oviedo)H-Index: 2
Last. Luis Caminal-MonteroH-Index: 13
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Abstract IgG4-related disease is characterized by a lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. It can present as parotid gland enlargement, tubulointerstitial nephritis, retroperitoneal fibrosis or pancreatitis, although nearly any organ can be affected. We report the case of a 37-year-old woman who presented with severe dysphonia and recurrent painful aphthous ulcers, with histopathological findings at the level of the larynx ...
3 CitationsSource
#1Hua Zhong (SYSU: Sun Yat-sen University)H-Index: 2
#2Li Y (SYSU: Sun Yat-sen University)H-Index: 3
Last. Wenbin Lei (SYSU: Sun Yat-sen University)H-Index: 14
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1 CitationsSource
#1Koji Matsushima (Toho University)H-Index: 5
#2Shinya Ohira (Toho University)H-Index: 2
Last. Kentaro Matsuura (Toho University)H-Index: 3
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Abstract Mikulicz’s disease, an immunoglobulin G4-related disease (IgG4-RD) occurs frequently in the head and neck region but rarely in the larynx. We report a case of IgG4-RD with pseudotumor formation in the larynx. A 50-year-old man presented at our facility for a complete physical examination and diagnostic testing of a left arytenoid tumor. On examination, a large supraglottic mass was noted with signs of dyspnea. Movement disorder of the vocal fold was absent. The patient underwent surgery...
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#1Zachary S. Wallace (Harvard University)H-Index: 27
#2Ray Naden (McMaster University)H-Index: 7
Last. John H. Stone (Harvard University)H-Index: 94
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IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serological, radiological and pathological data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exe...
109 CitationsSource
#1Opriţă RH-Index: 1
#2Opriţă BH-Index: 1
Last. Diaconescu IbH-Index: 1
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: Rationale (hypothesis): IgG4-related disease (IgG4-RD) is a pathological entity recently recognized by the medical world that can affect any organ or system. However, there is insufficient data about this disease in medical literature. Aim (objective): A more extensive clarification of the IgG4 molecule, the diversified aspects of IgG4-related disease, and the response of this disease to treatment, will provide a crucial understanding of the immune system and other diseases now known to be ass...
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IgG4-related sclerosing disease of the larynx (IgG4-SD) is a recently described immunodependent systemic pathology characterized by diffusive or focal inflammatory infiltration of the affected organs and tissues by plasma cells expressing IgG4; it is accompanied by the subsequent development of obliterative phlebitis and fibrosclerosis associated with the increase of the serum IgG4 level. According to the recently published materials, the disease can also develop in the respiratory system. The p...
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