A 34-year-old woman with a 7-ear history of deformity of the nose gets a diagnosis of granulomatosis with polyangiitis
A 34-year-old woman presented to the facial plastic surgery clinic with complaints of rhinorrhea, nasal crusting with blood, and progressive deformity of the nose over the past 7 years. The patient had a history of chronic rhinosinusitis and nasal polyposis However, she had no history of intranasal cocaine use.
Examination of the nose revealed a near-total loss of the nasal bones and septal cartilage, hence a completely collapsed nasal bridge and a retracted nasal tip.
A computed tomography scan of the sinuses showed severe, near-complete opacification of the paranasal sinuses. Moreover, it also showed a large nasal septal perforation (Panel B, arrow).
An enzyme-linked immunosorbent assay (ELISA) showed circulating antibodies to proteinase 3 (PR3). The antibodies present were at a level of 2432 units (≥20 units indicates positivity)
Therefore, with the history, physical examination, imaging, and ELISA, the doctor diagnosed her with granulomatosis with polyangiitis.
Granulomatosis with polyangiitis is an autoimmune disorder characterized by extravascular necrotizing granulomatous inflammation and necrotizing vasculitis. The disease has no sex predilection and mainly affects individuals in the 40s-50s. Although it has no sex predilection, males tend to have more severe disease, whereas females are more likely to have localised disease.
Although it can virtually affect any organ, it most commonly involves the respiratory tract and the kidneys. Patients may present with recurrent rhinosinusitis and bloody nasal crusting. Moreover, the disease can progress. The nasal involvement may progress to destroy the sinonasal cavities and perforate the septum. Therefore, it is imperative to diagnose and manage at the earliest to avoid complications.
She received rituximab, low-dose cyclophosphamide, and prednisone. At a 6-month follow-up visit, the patient’s bloody nasal discharge and crusting had resolved. However, the nasal deformity remained unchanged.
The patient continued to receive maintenance therapy with rituximab.