Supraglottic Localization of IgG4-Related Disease—Rare and Challenging Equity

Abstract

Supraglottic stenosis is a rare symptom, particularly in fibroinflammatory multifocal diseases, such as IgG4-related disease (IgG4-RD). There is still an inconsistency in the diagnosis of less-common locations of IgG4-RD, which causes a delay in the diagnosis and treatment. Our paper aims to analyze different aspects of IgG4-RD presenting as supraglottic stenosis, including the possible overlap with ANCA-associated vasculitis. We compare the usefulness of the recently revised ACR/EULAR and Comprehensive criteria and discuss treatment options. The review was performed according to PRISMA guidelines using the MEDLINE Pubmed and Scopus databases. The analysis includes nine papers describing supraglottic laryngeal stenosis in 13 patients. Furthermore, we present a case of a woman with ongoing supraglottic stenosis presenting with cough, temporary dyspnea and stridor as the symptoms of localized IgG4-RD. At the time of writing, the patient remains in remission while receiving treatment with cyclophosphamide and methylprednisolone. The symptoms of supraglottic localization of IgG4-RD may be severe; however, at that point, clinicians should suspect autoimmune etiology and attempt to modulate the autoimmune response instead of performing dilatation surgery—the effects of which may not result in extended intervals between interventions. The ACR/EULAR criteria show great specificity; however, when IgG4-RD is presumed, the specific treatment should be implemented.