Ear, nose and throat involvement in granulomatosis with polyangiitis: how it presents and how it determines disease severity and long-term outcomes

Abstract

Ear, nose and throat (ENT) manifestations in granulomatosis with polyangiitis (GPA) represent the most frequent symptoms at disease onset. The aim of the study was to analyse ENT involvement at diagnosis, as well as how it could influence relapse rate, mortality and disease severity. A retrospective non-controlled cohort study was performed including all consecutive diagnosed GPA from 1996 to 2016 in two rheumatology centres of Northern Italy, focusing particularly on ENT presenting signs and symptoms at baseline. Eighty-nine patients (48.3% females) with new onset GPA were evaluated. They were mostly Caucasian (97.7%), middle aged (mean 54.5 years) and more frequently anti-neutrophil cytoplasmic antibodies (ANCA) positive (78.6%) with PR3 specificity (81.4%). At diagnosis, ENT involvement was reported in 71.9% patients, second only to systemic symptoms. These patients were significantly younger at disease onset (0.013), with less frequent renal involvement (0.014) irrespectively to ANCA status, but with significantly higher Vasculitis Damage Index (VDI) (0.001). The most frequent ENT manifestation was sinonasal involvement (58.4%, 73% of which with nasal inflammation/chronic sinusitis and 48% with nasal crusting), while otologic involvement (mainly otitis media/otomastoiditis) was observed in 34.8%. ENT-GPA patients presented a higher survival rate at 5 years (98.1 vs 77.7%, 0.049), and ENT involvement resulted to be an independent predictor of better outcome (OR 0.37, 95% CI 0.2-0.8, 0.019). Our data confirms that ENT involvement is not only one of the key clinical features of GPA, but also could point out a milder GPA subset with lower renal involvement and lower mortality rate, irrespectively to ANCA status.

Ear Nose and Throat (ENT) Manifestations in Granulomatosis with Polyangiitis in Patients from Saudi Arabia

“…Sinusitis and nasal inflammation were found in 30.4% of our patients. These figures are lower than those reported elsewhere [10,15,16]. Likewise all other sino nasal symptoms occurred less frequently in our patients compared to other studies [15,16].…”

“…A study from Tunisia has reported initial ENT involvement of 56% and 83% at follow up [17]. Nose and sinus are reported to be affected by GPA in 55-90% of patients, which is comparable to our finding of 56.5% [8,9,16,18]. Nasal crusting and obstruction, rhinorrhea, nasal discharge, epistaxis, sinusitis, nasal septum deviation and perforation, edema of nasal mucosa and turbinates, and saddle nose deformity are frequently reported sino nasal symptoms [19,20].…”

“…ENT involvement is often the first manifestation of GPA [6]. ENT involvement was found in 65.2% of our GPA patients, which is less than what is found in previous studies reporting 72%-99% [1,5,[13][14][15][16]. A study from Tunisia has reported initial ENT involvement of 56% and 83% at follow up [17].…”

Updates in antineutrophil cytoplasmic antibody (ANCA)‐associated vasculitis for the ENT surgeon

“…PR3 ANCA positive 56%-83% 8,14 ANCA Negative 11%-31% 14,25 ENT involvement 70%-99% 13,26 Rhinologic Sinus involvement/Rhinitis 47%-72% 8,25 Recurrent epistaxis/nasal crusting 28%-83% 25,27 Nasal cavity adhesions 19%-26% 18,28 Rhinorrhoea 35%-54% 25,28 Mucosal ulcerations/granulations 10%-27% 18,28 Nasal septal perforation 8%-33% 27,29 External nasal/Saddle deformity 2%-22% 27,29 Epiphora 13% 29…”

“…Cranial nerve palsy 17% 33 Subglottic stenosis 1%-23% 18,27 Abbreviations: CHL, conductive hearing loss; OME, otitis media with effusion; SNHL, sensorineural hearing loss; VC: vocal cord.…”