Sinonasal Imaging Findings in Granulomatosis with Polyangiitis (Wegener Granulomatosis): A Systematic Review

D’Anza, Brian; Langford, Carol A.; Sindwani, Raj

doi:10.2500/ajra.2017.31.4408

Cited by 38 publications

(35 citation statements)

References 31 publications

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“…Only one in ten patients with GPA has been reported to have normal imaging findings. The spectrum of typical sinonasal findings in GPA includes mucosal thickening (~88% of cases), most commonly involving the maxillary sinuses, to osseous erosion (~60% of cases) and osteoneogenesis (~56% of cases) 9 . Although these changes can occur in garden-variety chronic rhinosinusitis also, there are certain subtle indicators which indicate a possibility of underlying GPA.…”

Section: Discussionmentioning

confidence: 99%

Evolution of sinonasal radiological findings in a case of granulomatosis with polyangiitis

Handa

Singh

Sharma

2019

Romanian Journal of Rhinology

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Wegener's granulomatosis (Granulomatosis with Polyangiitis) is an autoimmune granulomatous disorder of unknown etiology. The basic pathology is necrotizing granulomatous inflammation with necrotizing vasculitis involving medium-sized blood vessels. The typical triad of involvement of the upper and lower respiratory tracts and the kidneys is observed in varying proportions in individual patients. The sinonasal involvement is observed in the majority of patients and it can be involved in isolation in up to a quarter of affected individuals. The sinonasal changes involve both the soft tissue and bony architecture, with refractory chronic sinusitis associated with erosion of the cartilaginous and bony skeleton and may lead to saddle nose and other facial deformities in advanced cases. The radiology has a contributory role as diagnostic and diseases monitoring aid. The various radiological findings associated with the disease (bone erosion with osteoneogenesis) have been mentioned in the literature. We present the progression of a radiological abnormality in a patient with a localized form of Wegener's Granulomatosis and highlight some peculiar appearances observed on radiology in the patient.

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Section: Discussionmentioning

confidence: 99%

Evolution of sinonasal radiological findings in a case of granulomatosis with polyangiitis

Handa

Singh

Sharma

2019

Romanian Journal of Rhinology

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“…In accordance with this sinonasal biopsies aided diagnosis in our GPA patients. Some studies have shown that sinus imaging with CT and MRI reveals a spectrum of non-specific findings [26,27]. Radiographic sinonasal pathological features including mucosal thickening is seen in 87.7%, bony destruction in 59.9% and nasal septal erosion in 59.4% patients [26].…”

Section: Discussionmentioning

confidence: 99%

“…Some studies have shown that sinus imaging with CT and MRI reveals a spectrum of non-specific findings [26,27]. Radiographic sinonasal pathological features including mucosal thickening is seen in 87.7%, bony destruction in 59.9% and nasal septal erosion in 59.4% patients [26]. Maxillary sinuses were found to be the most frequently affected paranasal sinuses by CT imaging [27].…”

Section: Discussionmentioning

confidence: 99%

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

Arfaj¹

2019

AJIM

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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.

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“…A GPA é rara em negros e em crianças 1,2,6 . A etiologia permanece incerta e provavelmente é multifatorial 1,8,9 . Agentes infecciosos podem ter participação como gatilho de ativação a doença, através de mimetismos moleculares.…”

Section: Introductionunclassified

“…Agentes infecciosos podem ter participação como gatilho de ativação a doença, através de mimetismos moleculares. Há relatos de ocorrência familiar, com predomínio de associação ao HLA-DP e ao SERPINA 1,[8][9][10] , porém não se sabe exatamente esta relação com a doença 1,[8][9][10] . Nos últimos 5 anos, o tratamento tem se consolidado com base em terapia imunossupressora em duas fases indução e manutenção -com melhora substancial da sobrevida e das complicações da GPA.…”

Section: Introductionunclassified

Granulomatose com poliangiite

Santana

Rodrigues

Silva

et al. 2019

Rev. Med. (São Paulo)

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Introdução: Granulomatose com poliangiite (GPA) é uma vasculite granulomatosa necrosante pauci-imune que afeta predominantemente pequenos vasos. É uma doença rara, de etiologia multifatorial, e constitui importante diagnóstico diferencial das síndromes pulmão-rim. Objetivo: atualizar os principais tópicos acerca da granulomatose com poliangiite, além de trazer perspectivas futuras e avanços no manejo clínico dessa vasculite. Metodologia: revisão de literatura realizada em 2018 a partir das publicações dos últimos 5 anos nas base de dados do PubMed Central® e da Scielo®. Resultados: 29 referências bibliográficas selecionadas, das quais: 16 revisões de leteratura; 2 revisões sistemáticas; 1 estudo transversal; 2 estudos caso-controle; 6 coortes; 2 consensos atualizados (1 brasileiro de 2017 e 1 internacional de 2017). Conclusão: a granulomatose com poliangiite é uma entidade de elevada morbimortalidade, e teve seu algoritmo de tratamento recentemente atualizado, conforme abordado nesta revisão. Novos estudos ainda em estão em andamento para avaliar a eficácia e segurança de novas abordagens terapêuticas.

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Sinonasal Imaging Findings in Granulomatosis with Polyangiitis (Wegener Granulomatosis): A Systematic Review

Cited by 38 publications

References 31 publications

Evolution of sinonasal radiological findings in a case of granulomatosis with polyangiitis

Evolution of sinonasal radiological findings in a case of granulomatosis with polyangiitis

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

Granulomatose com poliangiite

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