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Comparative Study
. 2018 Dec;38(6):517-527.
doi: 10.14639/0392-100X-1776.

Clinical characteristics of patients with granulomatosis with polyangiitis and microscopic polyangiitis in ENT practice: a comparative analysis

J Wojciechowska  1 , T KręCicki  1
Affiliations
Comparative Study

Clinical characteristics of patients with granulomatosis with polyangiitis and microscopic polyangiitis in ENT practice: a comparative analysis

J Wojciechowska et al. Acta Otorhinolaryngol Ital. 2018 Dec.

Abstract
in English, Italian

ENT manifestations are commonly observed in patients with small vessel vasculitis (SVV). The main aim of this study was to analyse and present the clinicopathological characteristics of individuals with SVV emphasising otorhinolaryngological symptoms. This study evaluated 64 patients, 41 with granulomatosis with polyangiitis (GPA) and 23 with microscopic polyangiitis (MPA). Herein, we compare the clinicopathologic features of GPA and MPA. The average age at diagnosis was 50.2 and 56.2 years, for GPA and MPA, respectively. 57 patients (89%) were antineutrophil cytoplasmic antibody (ANCA) positive, 34 (59.6%) for anti-proteinase 3 (PR3)-ANCA and 21 (36.8%) for myeloperoxidase (MPO)-ANCA. 7 patients (10.9%) were ANCA negative. The most commonly affected organs were lungs (76.56%), ear, nose, throat (ENT) (75%) and kidneys (73.44%). ENT disorders mainly appeared as chronic rhinosinusitis and epistaxis and preceded SVV diagnosis by an average 14.4 months. In the majority of patients, ENT disorders were the first symptoms of SVV and preceded its systemic transformation. Pulmonary, ENT and nervous manifestations were more common in GPA, whereas the prevalence of renal, gastrointestinal, cutaneous, cardiovascular and ocular disorders was higher in MPA. The results of our study emphasise the high prevalence of ENT symptoms in patients with SVV, especially in those with GPA. We highlight the significant role of the otorhinolaryngologist in early SVV diagnosis and management. Any patient with persistent ENT symptoms or ENT dysfunctions not responding to standard otorhinolaryngological treatment should be precisely and rapidly evaluated for the presence of systemic dysfunctions (especially renal and pulmonary). Realising the differences and similarities between GPA and MPA is crucial in undelayed SVV diagnosis and proper treatment.

Caratteristiche cliniche dei pazienti con granulomatosi con poliangioite e poliangioite microscopica nella pratica clinica ORL: analisi comparativa.

Riassunto: Nei pazienti con vasculiti dei piccoli vasi (SVV), si osservano frequentemente manifestazioni del distretto otorinolaringoiatrico (ORL). Lo scopo principale di questo studio è stato quello di analizzare e presentare le caratteristiche clinicopatologiche degli individui con SVV, enfatizzando i sintomi della sfera ORL. Sono stati valutati 64 pazienti, 41 con granulomatosi con poliangioite (GPA) e 23 con poliangioite microscopica (MPA). Abbiamo presentato e paragonato le manifestazioni clinicopatologiche di GPA e MPA. L’età media alla diagnosi è stata rispettivamente di 50,2 e 56,2 anni per GPA e MPA. 57 pazienti (89%) erano positivi per gli anticorpi anti citoplasma dei neutrofili (ANCA), 34 (59,6%) per gli anti proteinasi 3-ANCA e 21 (36,8%) per mieloperossidasi (MPO)-ANCA. 7 pazienti erano ANCA negativi. Gli organi più frequentemente colpiti sono stati polmone (76,5%), ORL (75%) e reni (73,4%). Le problematiche ORL si sono manifestate più frequentemente sotto forma di rinosinusite cronica ed epistassi ed hanno preceduto in media la diagnosi di SVV di circa 14,4 mesi. Nella maggior parte dei pazienti i disturbi ORL hanno rappresentato il primo sintomo di SVV, precedendone la trasformazione sistemica. Nella GPA, sono risultate più frequenti le manifestazioni polmonari, ORL e del sistema nervoso, mentre nella MPA quelle renali, gastrointestinali, cutanee, cardiovascolari ed oculari. I risultati del nostro studio hanno enfatizzato l’alta prevalenza di sintomi ORL nei pazienti con SVV, specialmente in quelli con GPA. Vogliamo quindi sottolineare il ruolo importante dello specialista ORL nella diagnosi precoce e nel conseguente precoce trattamento delle SVV. Ogni paziente con sintomi o disfunzioni ORL persistenti e non responsivi alle terapie standard, deve essere valutato per la presenza di manifestazioni sistemiche (specialmente renali e polmonari). Evidenziare le differenze e le somiglianze tra GPA e MPA è cruciale per una precoce diagnosi di SVV e per iniziare una terapia adeguata.

Keywords: Granulomatosis with polyangiitis; MPO-ANCA; Microscopic polyangiitis; PR3-ANCA; Vasculitis.

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Figures

Fig. 1.
Fig. 1.
Main organ involvement in various forms of AAV.
See this image and copyright information in PMC

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