A 58-year-old Caucasian male presented with left periorbital oedema extending to the nasal area for 1 year along with nasal discharge for 1 month. Lab work was significant for positive cytoplasmic antineutrophil cytoplasmic antibodies. CT scan showed solid mass along the nasal soft tissue with bony nasal destruction. A CT scan of the thorax was performed to rule out granulomatosis with polyangiitis and showed multiple pulmonary nodules. Biopsies of the nasal mass and lung nodule were performed which showed fragments of fibrosis with spindle cell proliferation consistent with eosinophilic angiocentric fibrosis (EAF). EAF is a very rare disease, recently described as a subtype of immunoglobulin G4-related disease. A few rare cases of EAF involving the structures of the orbit have been reported in the literature.

Relapsing polychondritis is a very rare autoimmune disease characterised by a relapsing inflammation of hyaline, elastic and fibrous cartilaginous tissues. The incidence is estimated to be between 3.5 and 4.5 per million people per year. Clinical signs and symptoms can be very subtle, and if left undiagnosed for a prolonged period, airway involvement can cause fibrosis of the tracheobronchial wall, leading to a fixed tracheobronchial stenosis. Eventually, this can progress to life-threatening tracheobronchomalacia due to irreversible damage and loss of tissue integrity. We report an elderly man who presented with recurrent bilateral ear inflammation and intermittent polyarthritis who was diagnosed with relapsing polychondritis with asymptomatic involvement of his large airways.