Unusual Presentation of Granulomatosis With Polyangitis (Wegener's Granulomatosis)
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Abstract
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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References
Macias JD, Wackym PA, McCabe BF. Early diagnosis of otologic Wegener’s granulomatosis using serological marker c-ANCA. Ann Otol Rhinol Laryngol. 1993; 102:337-41
Hern JD, Hollis LJ, Mochloulis G, et al. Early diagnosis of Wegener’s granulomatosis presenting with facial nerve palsy. J Laryngol Otol. 1996; 110: 459-61
Atula T, Honkanen V, Tarkkanen J, et al. Otits media as a sign of Wegener’s granulomatosis in childhood. Acta Otolaryngol. 2000 543(Suppl):48-50
De Remee RA, Mcdonald TJ, Harrison EG, Coles DT. Wegener’s granulomatosis. Anatomic correlates, A proposed classification. Mayo clinic proceedings 1976; 52:777-81
D’Cruz DP, Baguley E, Asherson RA, Hughes GRV. Ear, nose and throat symptoms in subacute Wegener’s granulomatosis. British Med J. 1989; 299:419-22
McCaffey TV, McDonald TJ, Facer GW, et al. Otologic manifestations of Wegener’s granulomatosis. Otolaryngol Head Neck Surg. 1980; 88:586-93
Friedmann I, Bauer F. Wegener’s granulomatosis causing deafness. J Laryngol Otol. 1973; 87:449-64
Chegar BE, Kelley RT. Wegener's granulomatosis presenting as unilateral parotid enlargement. Laryngoscope 2004; 114(10):1730-3
Jones GL et al. Wegener's granulomatosis mimicking a parotid abscess. J Laryngol Otol. 2005; 119(9):746-9
Drinias V, Florentzson R. Facial palsy and Wegener’s granulomatosis. Am J Otolaryngol. 2004; 25(3):208-212
Bibas A, Fahy C, Sneddon L, et al. Facial paralysis in Wegener’s granulomatosis of the middle ear. J Laryngol Otol. 2001; 115:304-6
Illum P, Thorling K. Wegener’s granulomatosis. Long term results of treatment. Ann Otol Rhinol Laryngol. 1981; 90:231-5
Banerjee A, Armas JM, Dempster JH. Wegener’s granulomatosis: Diagnostic dilemma. J Laryngol Otol. 2001; 115: 46-7
Tamura N, Matsudaira R, Hirashima M, Ikeda M, Tajima M, Nawata M, et al. Two cases of refractory Wegener's granulomatosis successfully treated with rituximab. Intern Med. 2007; 46(7):409-14