Vasculitis of the upper airways.

Area of Science:

• Rheumatology
• Otolaryngology
• Immunology

Background:

• Systemic vasculitides are rare, severe diseases affecting blood vessels.
• Granulomatosis with polyangiitis (GPA) and Eosinophilic granulomatosis with polyangiitis (EGPA) are key types involving the upper respiratory tract.
• Initial symptoms like sinusitis or allergic rhinitis can mimic other conditions, posing diagnostic challenges.

Purpose of the Study:

• To highlight the diagnostic challenges of upper airway manifestations in GPA and EGPA.
• To differentiate between GPA and EGPA based on specific upper airway signs.
• To emphasize the importance of local treatment for refractory upper airway symptoms, particularly subglottic stenosis (SGS).

Main Methods:

• Review of clinical presentations of GPA and EGPA focusing on upper airway involvement.
• Analysis of symptom specificity for differentiating between GPA and EGPA.
• Evaluation of treatment strategies for upper airway manifestations.

Main Results:

• Chronic rhinitis/polyposis suggests EGPA, while recurrent sinusitis/otitis can occur in both.
• Nasal septum perforation, saddle nose deformity, and subglottic stenosis (SGS) are more indicative of GPA.
• Upper airway symptoms can be treatment-refractory, necessitating local management.

Conclusions:

• Upper airway symptoms in GPA and EGPA present diagnostic difficulties, especially when initial manifestations.
• Certain signs like SGS are more suggestive of GPA, aiding differential diagnosis.
• Local treatment is a critical component in managing persistent upper airway issues in systemic vasculitis, particularly SGS.