Unusual Presentation of Granulomatosis With Polyangitis (Wegener's Granulomatosis)

Objective: To present a case of unusual presentations of granulomatosis with polyangitis (Wegener’s granulomatosis) with parotid swelling and intractable otitis externa. Material and method: A 22-year-old male patient presented with left sided otitis externa with left sided parotid swelling and bilateral nasal obstruction for last two weeks. CT scan of paranasal sinuses showed homogenous mass in both maxillary antra and nasal cavities. FNAC from the parotid swelling was suggestive of granulomatous disease and endoscopic biopsy from the nasal mass showed features of granulomatosis with polyangitis (Wegener’s granulomatosis). CT scan of chest revealed multiple cysts within the lung parenchyma; urine examination showed RBC and pus cells. Renal biopsy showed focal segmental glomerulonephritis. c-ANCA was highly positive and thus the diagnosis of Wegener’s granulomatosis was made. Result: Patient received cyclophosphamide and prednisolone immunsuppressive therapy for one year, which showed marked clinical improvement. Conclusion: Granulomatosis with polyangitis (Wegener’s granulomatosis) is not an uncommon entity for otolarygologists. Its usual presentation mimics chronic rhinosinusitis; but presentations like otitis externa and parotid swelling are rare for this disease.

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An Unusual Case of Otitis Media

Journal of the American Academy of Audiology ◽

10.3766/jaaa.16.8.8 ◽

2005 ◽

Vol 16 (08) ◽

pp. 596-599

Author(s):

Raymond H. Hull

Keyword(s):

Otitis Media ◽

Middle Ear ◽

Wegener’S Granulomatosis ◽

Systemic Vasculitis ◽

Wegener's Granulomatosis ◽

Respiratory Involvement ◽

Life Threatening ◽

Middle Ear Disease ◽

One Year ◽

Nose And Throat

This case report of bilateral otitis media in a 39-year-old woman secondary to Wegener's Granulomatosis highlights, for the audiologist, the importance of being aware of some of the less common etiologies of middle ear disease. Bilateral otitis media that resists usual forms of medical treatment may represent one of the earliest signs of Wegener's Granulomatosis, a potentially life-threatening disease. According to recent literature, involvement of the ears, nose, and throat can in many instances be the only early manifestation of the disease. It is important that audiologists be aware of the early signs in order to make appropriate referral for treatment, particularly since they are usually among the first to see these patients. Early diagnosis and referral is critical since the mean survival of untreated WG is five months, with 82% of patients dying within one year, and more than 90% dying within two years. The disease involves a systemic vasculitis that may involve any organ system: however, pulmonary or renal disease appear to be among the later signs, while both middle ear and upper respiratory involvement are frequently among the earliest.

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Parotid Swelling In Wegener's Granulomatosis

10.1164/ajrccm-conference.2012.185.1_meetingabstracts.a6166 ◽

2012 ◽

Author(s):

Hamdi G. Amar

Keyword(s):

Wegener’S Granulomatosis ◽

Wegener's Granulomatosis ◽

Parotid Swelling

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Wegener’s granulomatosis and subglottic stenosis: management of the airway

The Journal of Laryngology & Otology ◽

10.1258/0022215042450670 ◽

2004 ◽

Vol 118 (10) ◽

pp. 786-790 ◽

Cited By ~ 24

Author(s):

A. Alaani ◽

R.P. Hogg ◽

A.B. Drake Lee

Keyword(s):

Wegener’S Granulomatosis ◽

Wegener's Granulomatosis ◽

Subglottic Stenosis ◽

Necrotizing Vasculitis ◽

Respiratory Obstruction ◽

First Line ◽

Multisystemic Disease ◽

One Year ◽

Surgical Treatments ◽

Active Disease

Wegener’s granulomatosis is a multisystemic disease characterized by foci of necrotizing vasculitis and granuloma formation. Subglottic stenosis may occur either asa presenting feature or a late-stage manifestation of the disease, but will occur in approximately 10–20 per cent of cases.We present a series of seven cases of Wegener’s granulomatosis with subglottic stenosis and discuss our management of this condition. Where there is active disease, tracheostomy is the first-line surgical treatment of respiratory obstruction, as an adjunct to full medical therapy.More aggressive or elaborate surgical treatments should be reserved for non-active cases in which patients have not required medical treatment for one year.

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Wegener's Granulomatosis: A Rare Cause of Hydronephrosis

Case Reports in Medicine ◽

10.1155/2011/814794 ◽

2011 ◽

Vol 2011 ◽

pp. 1-3 ◽

Cited By ~ 5

Author(s):

Julien Lillaz ◽

Stéphane Bernardini ◽

Marie-Paule Algros ◽

Hugues Bittard ◽

François Kleinclauss

Keyword(s):

Urinary Diversion ◽

Wegener’S Granulomatosis ◽

Renal Colic ◽

Surgical Excision ◽

Wegener's Granulomatosis ◽

Neoplastic Process ◽

Clinical Description ◽

One Year ◽

Ureteral Lesion

A seventy-one-year-old woman was hospitalized at our institution for a right-sided “renal colic” associated with an infectious background. Alithiasic ureterohydronephrosis was diagnosed by imaging. A urinary diversion was thus performed using a double J endoureteral stent. The etiologic assessment of the hydronephrosis showed the presence of a periureteral mass that caused extrinsic ureteral compression. After surgical excision of the ureteral lesion, the Wegener's granulomatosis diagnosis was established. This report is the clinical description of a case of “atypical” Wegener's granulomatosis revealed by the onset of a ureteral disease mimicking a neoplastic process.

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