Laryngeal Frame Involvement as The First Sign of Wegener's Granulomatosis.

Abstract

Introduction: Granulomatosis with Polyangiitis (GPA), also known as Wegener's Granulomatosis, is an ANCA-associated vasculitis that primarily affects small vessels, leading to necrotizing granulomatous reactions in the airways and small vessels. The etiology remains uncertain.

Case report: We report the case of a woman in her 70s, who was previously tracheostomized at another facility and was presented to our attention with glottic-subglottic stenosis. We performed a lysis of glottic synechia and subglottic debulking via transoral laser microsurgery, yielding satisfactory results over the short term. However, a relapse occurred within two months, along with ulcerative lesions on the nasal septum. Biopsies revealed multinucleated giant cells and inflammation suggestive of vasculitis. Based on the histological and clinical features, a diagnosis of vasculitis was considered. Anti-Neutrophil Cytoplasmic Antibodies testing was positive. A rheumatological examination confirmed the hypothesis of Granulomatosis with Polyangiitis. The lack of typical symptoms was the main reason for the delayed diagnosis.

Conclusion: Involvement of the subglottic region and the upper portion of the trachea is a rare but severe complication of GPA. The current literature reports only few cases of laryngeal stenosis, with poor prognosis. Histological examinations of biopsied laryngeal tissue showed significant but non-specific inflammation, contributing to the delay in diagnosis. There are still no precise guidelines for the surgical treatment of subglottic stenosis. This case underscores the importance of considering laryngeal involvement in GPA for early diagnosis and timely intervention to prevent serious complications in order to improve patient outcomes.