Laryngeal IgG4-Related Disease: A Systematic Review of Clinical Features and Management.

In 30 seconds

This systematic review synthesized clinical features, diagnostic approaches, and treatment outcomes in laryngeal IgG4-related disease. Searches identified 27 publications describing 32 patients with histopathologically or clinicopathologically confirmed laryngeal involvement. Patients commonly presented with hoarseness, dyspnea, or stridor, and disease often affected the supraglottis or subglottis. The review found two recurring patterns: circumferential fibrostenosis and mass-like or polypoid lesions. Serum IgG4 was frequently normal despite biopsy-confirmed disease; deep or repeat biopsy was often needed. Relapse and progression to fixed fibrostenosis were common after treatment.

Key findings

• The review included 27 publications and 32 patients with confirmed laryngeal IgG4-related disease.
• Supraglottic and subglottic involvement predominated; multilevel airway disease occurred in about one third of cases.
• Serum IgG4 levels were frequently normal, and superficial biopsies could be nondiagnostic.
• Relapse and progression to fixed fibrostenosis were common despite treatment, which often combined glucocorticoids with other therapies or airway procedures.

Why it matters

Laryngeal IgG4-related disease can resemble idiopathic stenosis or neoplasia, while normal serum IgG4 and nondiagnostic superficial sampling may delay recognition. Awareness of these diagnostic patterns may help clinicians consider the disease in unexplained laryngeal narrowing or submucosal masses and pursue tissue confirmation adequate to assess deeper involvement.

What to keep in mind

Evidence came from 27 publications describing only 32 patients, limiting the strength and generalizability of conclusions about presentation and treatment outcomes.