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

Granulomatosis with polyangiitis (GPA) frequently affects ear, nose and throat (ENT) at disease onset. Our aim was to report on our experience with the ENT manifestations in GPA patients, therapy and outcome. A retrospective study of GPA patients was performed who followed up in Rheumatology clinics at King Khalid University hospital, Riyadh during the period 1990-2016. Demographics, different ENT manifestations, laboratory and diagnostic features, therapy and outcome of GPA patients were retrieved from their medical charts. ENT involvement was observed in 15 (65.2%) of the 23 GPA cases identified. Otologic symptoms were noted in 3 (13.0%), nose and sinus symptoms in 13 (56.5%) and throat symptoms in 3 (13.0%) GPA patients. Epistaxis (39.1%) was the most prevalent nose and sinus symptom followed by sinusitis (30.4%), otitis media and hearing loss were the frequent otologic symptoms, oral ulcers and hoarseness of voice constituted frequent throat symptoms in GPA patients. Of the 15 ENT-GPA patients, 9 were males and 6 were females (male: female; 1.5: 1). Their mean age at disease onset was 33.8 ± 18.3 (range 11-57) years and mean duration of disease was 10.1 ± 5.9 (range 1-20) years. ANCA was positive in 93.3% ENT-GPA patients, 73.3% had c-ANCA and 20.0% had p-ANCA. Infections were noted in 33.3% ENT-GPA patients that included pneumonia, septicemia, esophageal candidiasis, bacterial meningitis and herpes zoster. All patients received oral prednisolone, 60.0% received intravenous cyclophosphamide, 20.0% refractory cases received rituximab doses, and the disease outcome was good. Comparison of ENT- GPA with non- ENT GPA cohort showed that 26.7% ENT-GPA patients had renal involvement compared to 87.5% non ENT-GPA patients (p = 0.009). Our study showed that the frequency of ENT symptoms in our GPA patients was less compared to other studies, and the disease outcome was good. Renal involvement was significantly less frequent in ENT-GPA cohort compared to non ENT-GPA cohort