Patterns of presentation and diagnosis of patients with Wegener's granulomatosis: ENT aspects

Srouji, Ibrahim Albert; Andrews, Peter; Edwards, Chris; Lund, Valerie J.

doi:10.1017/s0022215106005032

Cited by 76 publications

(78 citation statements)

References 5 publications

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“…In contrast to our case, a recent study found that patients older than 60 years when diagnosed with GPA initially presented with renal, pulmonary and other manifestations more so than younger patients [7]. This study also found that the nose and paranasal sinuses in particular, were the sites of presenting symptoms in 50 per cent of patients diagnosed before the age of 60 years, compared with only 27 per cent of those diagnosed when older than 60 years.…”

Section: Discussioncontrasting

confidence: 97%

Myeloperoxidase Positive Wegener’s Granulomatosis with Isolated Otorhinological

Rs¹,

Kl²,

Baxter³

et al. 2013

JCR

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Abstract:Introduction: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract and kidney. Case Report: We present here the uncommon case of a 71year old Caucasian man with newly diagnosed GPA presenting with isolated otorhinological involvement. The patient described 3 weeks of right-sided otalgia, mastoid pain and right hemifacial pain with associated rhinorrhoea, particularly at night, fevers and sweats. On examination, the right mastoid was tender to palpation but neither erythematous nor swollen. A Computed Tomography (CT) brain scan showed right-sided sinusitis and mastoiditis and right-sided acute on chronic otitis media without bony erosion. A biopsy of the right maxillary sinus found suppurative granulomatous changes, mild active vasculitis with neutrophil polymorphs in vessel walls and healing vasculitis with sclerosed vessels in keeping with a diagnosis GPA. Serology showed that he was p-anti-neutrophil cytoplasmic antibody (p-ANCA) positive, myeloperoxidase (MPO) positive and serine proteinase 3 antigen (PR3) negative. His GPA was treated with prednisolone in the acute phase and azathioprine was later commenced for maintenance therapy. Conclusion: This case highlights the need to consider GPA in a patient presenting with isolated otorhinological involvement without organ involvement.

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Section: Discussioncontrasting

confidence: 97%

Myeloperoxidase Positive Wegener’s Granulomatosis with Isolated Otorhinological

Rs¹,

Kl²,

Baxter³

et al. 2013

JCR

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“…Therefore ENT doctors should be more careful in diagnosis. [1] As in our case, when the patient consults with limited symptoms of WG and rare organ involvement (like the parotid gland), it is important to have a high index of suspicion. [16] For this reason, ENT doctors should not overlook WG in the differential diagnosis of patients with chronic inflammation and head and neck region involvement that is non responsive to medical therapy.…”

Section: Discussionmentioning

confidence: 74%

“…Sirouji et al [1] showed that time from consultation with presenting symptoms to the definite diagnosis was >1 month in 92% of the patients. Early diagnosis and appropriate treatment can prevent disease progression, as the disease can convert into a systemic, irreversible form with lung and kidney involvement.…”

Section: Discussionmentioning

confidence: 99%

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Wegener granulomatosis in parotid fistula and bilateral nasal mass

Demir¹

2017

Kulak Burun Bogaz Ihtis Derg

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“…10 Recognising patients with secondary chronic rhinosinusitis, who have normal sinuses despite being convinced otherwise, is sometimes as difficult as managing those patients mentioned above. Valerie Lund's experience of vasculitis in the nose and sinuses is unparalleled; the manifestations of Wegener's in nearly 200 patients are described in a paper by Srouji et al 11 A top tip, straight from Professor Lund, is to ask all patients if they 'feel unwell' -a positive response should prompt consideration of systemic disease.…”

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confidence: 99%

Editorial

Hettige

Hopkins

2017

J. Laryngol. Otol.

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It has been a great pleasure to browse The Journal of Laryngology & Otology Digital Archive in order to compile a freely accessible special 'rhinology anthology' ('rhinolanth' for short) issue centred around sinus disease and the evolution of endoscopic sinus surgery. We would like to dedicate it to Professor Nick Jones; our long list had at least another 10 of his original articles, and we were reminded of his enormous contribution to rhinology. He has already inspired a generation of rhinologists, but we hope that some trainees who did not have the fortune to work with him will have the opportunity to learn from the selected papers. His paper on septal correction, 1 while not strictly linked to our theme, is essential reading for any ENT surgeon, and could not be overlooked.We start with some excellent papers on the microbiology of acute sinusitis. We have failed to heed the warnings of antibiotic overuse, as described in a 2005 paper by Brook, 2 but fortunately the recent paper by Miah et al. 3 shows that in most cases of complicated allergic rhinitis, the isolated organisms are still sensitive to the majority of common antibiotics. An updated study conducted 20 years from now is less likely to reveal the same results. Thunberg and colleagues' study,4 simple yet elegant, shows that anterior rhinoscopy and culture is comparable to sinus puncture, and therefore may allow more selective use of antibiotics in primary care. In chronic sinus disease, attention has turned to the immunomodulatory effects of antibiotics, as highlighted in Fan and colleagues' study of clarithromycin. 5 The efficacy, dosage and indications for low-dose clarithromycin have yet to be firmly established, and we suspect that there is probably significant overuse at present. It is also unclear whether biofilms are causative in the pathophysiology of chronic rhinosinusitis, or simply opportunistic bystanders in the setting of epithelial dysbiosis. 6 Any rhinologist will know the perils of diagnosis, particularly in patients with facial pain and post-nasal drip. Rigid endoscopy is an essential part of the diagnostic investigation; McCluney et al. 7 suggest there is little benefit in topically anaesthetising the nose before this procedure. A simple change in practice could reduce costs and morbidity associated with using the nasal spray, which is as relevant in today's healthcare economy as when this article was first published. Computed tomography (CT) is often helpful and mandatory before surgery. Mason and colleagues' review of CT evaluation remains of great value nearly 20 years after publication. 8 It is cases where chronic rhinosinusitis has been excluded that are often the most challenging, and where Professor Jones' wisdom and experience come to the fore. His paper on the use of ice-cold carbonated water 9 has helped enormously in our clinical practice, and we would recommend it to anyone struggling to treat a patient with catarrh. Patients with facial pain needn't become your own headache, and with careful assessment and treatment, n...

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Patterns of presentation and diagnosis of patients with Wegener's granulomatosis: ENT aspects

Cited by 76 publications

References 5 publications

Myeloperoxidase Positive Wegener’s Granulomatosis with Isolated Otorhinological

Myeloperoxidase Positive Wegener’s Granulomatosis with Isolated Otorhinological

Wegener granulomatosis in parotid fistula and bilateral nasal mass

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