Wegener's granulomatosis presenting as subglottic stenosis

Area of Science:

• Rheumatology
• Pulmonology
• Otolaryngology

Background:

• Wegener's granulomatosis (WG), a necrotizing vasculitis, typically affects the lungs, kidneys, and upper airways.
• Subglottic stenosis (SGS) occurs in up to 20% of diagnosed WG patients but is an uncommon initial manifestation.

Purpose of the Study:

• To report a case of WG where subglottic stenosis was the predominant initial presentation.
• To discuss the diagnostic challenges and management of WG presenting atypically without lung or kidney involvement.

Main Methods:

• Case report detailing a patient with initial symptoms of subglottic stenosis.
• Diagnostic workup included initial non-specific biopsies.
• Antineutrophil cytoplasmic antibodies (ANCA) testing and a second biopsy confirmed vasculitis.

Main Results:

• The patient presented with subglottic stenosis as the primary symptom, with no initial signs of pulmonary or renal disease.
• Elevated cytoplasmic ANCA titers were crucial in guiding the diagnosis.
• Second biopsy confirmed vasculitis, leading to effective treatment.

Conclusions:

• Subglottic stenosis can be the predominant initial manifestation of Wegener's granulomatosis, even without typical organ involvement.
• Diagnostic uncertainty can arise, underscoring the importance of ANCA testing and repeat biopsies.
• Multimodal treatment, including immunosuppressants and surgical intervention, may be necessary for optimal outcomes.