Head and Neck Manifestations of Eosinophilic Granulomatosis with Polyangiitis

Goldfarb, Jared; Rabinowitz, Mindy; Basnyat, Shristi; Nyquist, Gurston; Rosen, Marc

doi:10.1177/0194599816657044

Cited by 15 publications

(15 citation statements)

References 37 publications

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“…41 In established EGPA, flares in upper and lower respiratory tract features are increasingly being thought of as distinct from systemic flares. 41 Nasal polyposis with adult-onset asthma should be an indication to check ANCA and blood eosinophil levels, 42 particularly in the presence of refractory disease. Recurrent, persistent OME with highly viscous effusions, in the presence of polyps and asthma, should further raise suspicion of EGPA.…”

Section: Eosinophilic Granulomatosis With Polyangiitismentioning

confidence: 99%

“…Nasal polyposis with adult‐onset asthma should be an indication to check ANCA and blood eosinophil levels, 42 particularly in the presence of refractory disease. Recurrent, persistent OME with highly viscous effusions, in the presence of polyps and asthma, should further raise suspicion of EGPA 43 …”

Section: When Should Anca‐associated Vasculitis Be Suspected In the Ementioning

confidence: 99%

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

Coates

Pero

2020

Clinical Otolaryngology

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ENT involvement is common in ANCA-associated vasculitis (AAV), particularly in GPA and EGPA. Early recognition and treatment is important for good outcomes, yet evidence suggests that UK ENT surgeons may not consistently recognise the early features of AAV, despite a similar incidence to vestibular schwannoma. AAV is a rapidly advancing field, with significant developments in the understanding of its pathogenesis, classification and treatment over the past decade. Relevant vasculitis mimics are also discussed with a particular focus on the increasing prevalence of vasculitis mimics driven by an increase in recreational cocaine use, as well as the emergence and reclassification of several other vasculitis mimics in the head and neck. This article reviews key recent updates in the vasculitis literature, with a particular focus on those relevant to recognition and diagnosis of AAV for the ENT surgeon. Strengths and limitations of relevant diagnostic testing are discussed, and a method of evaluation of patients with features of AAV presenting to ENT services is outlined. | 317

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Section: Eosinophilic Granulomatosis With Polyangiitismentioning

confidence: 99%

Section: When Should Anca‐associated Vasculitis Be Suspected In the Ementioning

confidence: 99%

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

Coates

Pero

2020

Clinical Otolaryngology

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“…Next, the use of imaging has been limited to detecting otomastoiditis and granulomatous lesions secondary to vasculitis due to the small size of vessels affected 6 . Lastly, biopsy specimens from the ear never provide histological confirmation of AAV, except for those taken from the mastoid cavity via a surgical approach 7,8 . Given the lack of clinicopathologic correlation, the diagnosis and monitoring of otologic involvement of AAV is occasionally difficult, especially in ear‐limited variants or ANCA‐negative AAV.…”

Section: Introductionmentioning

confidence: 99%

Enhanced fallopian canal as a potential marker for temporal bone vasculitis

Fujikawa

Honda

Ito

et al. 2020

Laryngoscope Investig Oto

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Objectives: This study aimed to test the hypothesis that contrast-enhanced 3D MRI with gradient-echo sequences (CE-3D-GRE) can detect signs of vasculitis in the fallopian canal, which may cause otologic involvement, in four patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods: CE-3D-GRE acquired at 3.0 Tesla was performed on four patients diagnosed with granulomatosis with polyangiitis or eosinophilic granulomatosis with polyangiitis, at onset or relapse of the disease, and in remission. Clinical correlations between otologic symptoms and radiological findings were examined for each patient. Furthermore, signal intensity of the mastoid segment of the fallopian canal was compared between the ears with active disease (n = 3) and those in remission or without vasculitis (n = 3). Results: Intense enhancement in the tympanic and mastoid segments of the fallopian canal was associated with development of external otitis, otitis media, and sensorineural hearing loss, and was unrelated to the presence of facial paresis. Maximal intensity projection images visualized the close relationship between the enhanced fallopian canal and middle ear inflammation. The findings were absent in remission. Signal intensity of the mastoid segment of the fallopian canal was higher in ears with active disease than in normal ears (P < .001) and decreased to normal levels during remission (P = .597). Conclusion: CE-3D-GRE can demonstrate vasculitis in the temporal bone, reflecting disease activity and the severity of otologic manifestations, including cochlear involvement, in AAV patients. Intense enhancement of the fallopian canal on CE-3D-GRE can be a potential marker for vasculitis of the temporal bone.

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“…Nevertheless, patients with MEE had to temporarily increase the maintenance steroid dose. Local administration of steroids is reported to be effective for localized symptoms in MEE [ 28 ]. In the present study, however, intratympanic injection combined with external drainage via myringotomy/VT insertion showed temporal effectiveness, and the maintenance steroid eventually had to be increased in most patients (> 70%) to control MEE.…”

Section: Discussionmentioning

confidence: 99%

Intractable middle ear effusion in EGPA patients might cause permanent hearing loss: a case–control study

Kang

Shin

Cho

et al. 2022

Allergy Asthma Clin Immunol

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Background Ear, nose, and throat involvement are common in eosinophilic granulomatosis with polyangiitis (EGPA). Among otologic manifestation, middle ear effusion (MEE) is less recognized but a problematic condition as it may progress to hearing impairment when left untreated. This study aimed to evaluate the characteristics, risk factors and clinical outcomes of MEE in EGPA patients. Methods This is a case–control study of patients who were diagnosed and treated for EGPA from January 1995 to November 2018. Patients with ear symptoms (ear fullness, ear discharge, tinnitus or hearing loss) were assessed by otologists and were included in the case group (n = 23) if clinically relevant. The other patients without MEE were included in the control group (n = 52). Risk of MEE was calculated using the Cox proportional-hazard model. Results During median follow-up of 9.9 years, 23 (30.7%) out of 75 patients had MEE. In MEE group, 12 (52.2%) patients had hearing loss; conductive type in 10 (10/12, 83.3%) and mixed type in two (2/12, 16.7%). In multivariable regression analysis, major organ involvement at diagnosis (adjusted hazard ratio [aHR] 65.4; 95% confidence interval [CI], 1.50—2838.39; P = 0.030] , early onset of ear symptom after systemic therapy (< 6 months) (aHR 40.0; 95% CI, 1.35—1183.43; P = 0.033) and continuing the maintenance steroid without cessation (aHR 8.59; 95% CI, 1.13—65.42; P = 0.038) were independently associated with a risk of MEE. To control MEE, 16 (69.6%) patients had to increase maintenance steroid dose and 9 (39.1%) patients experienced recurrent MEE whenever maintenance dose was tapered. Conclusions MEE is a common but frequently neglected condition in EGPA which is often intractable. The maintenance steroid dose should be adequately adjusted to control MEE and to prevent from progressive hearing loss. Novel biologic agents possibly have a role in controlling MEE in EGPA.

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Head and Neck Manifestations of Eosinophilic Granulomatosis with Polyangiitis

Cited by 15 publications

References 37 publications

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

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

Enhanced fallopian canal as a potential marker for temporal bone vasculitis

Intractable middle ear effusion in EGPA patients might cause permanent hearing loss: a case–control study

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