Abstract
Introduction
Wegener granulomatosis is a systemic vasculitis affecting small and medium-sized vessels of the upper and lower respiratory tract and kidneys.
Objective
To describe a case of Wegener disease with atypical manifestation.
Resumed Report
We describe the case of a 50-year-old woman with chronic otitis media and sensorineural hearing loss as the primary symptoms, without other manifestations.
Conclusion
In cases of acute ear manifestations with or without hearing loss and with poor response to usual treatments,Wegener granulomatosis should be included among the possible etiologies. After adequate diagnoses and treatment of this rare disease, there was favorable evolution.
Wegener granulomatosis; otitis media; hearing loss
Introduction
Wegener granulomatosis (WG) is a rare multisystem autoimmune disease of unknown etiology.11 Rossini BAA, Bogaz EA, Yonamine FK, Testa JRG, Penido N de O. Refractory otitis media as the first manifestation of Wegener's granulomatosis. Braz J Otorhinolaryngol 2010;76(4):541 22 Scalcon MRR, Pereira IA, Rachid Filho A, Paiva ES. Manifestação otológica localizada em paciente com granulomatose deWegener. Rev Bras Reumatol 2008;48(4):253-255 It is a systemic vasculitis, affecting small and medium-sized vessels of the upper and lower respiratory tract and kidneys, including necrotizing granulomatous inflammation.22 Scalcon MRR, Pereira IA, Rachid Filho A, Paiva ES. Manifestação otológica localizada em paciente com granulomatose deWegener. Rev Bras Reumatol 2008;48(4):253-255 33 Antunes T, Barbas CSV. Granulomatose de Wegener. J Bras Pneumol 2005;31(Suppl 1):S21-S26 WG has a spectrum of clinical presentations that includes recurrent respiratory infection, renal manifestations, and nonspecific systemic symptoms. Otologic manifestations are occasionally the first to appear in 20 to 60% of cases of WG, and serous otitis media is the most common presentation in ear disease.44 Pires APBA, Sousa NJA, Sousa RCA, et al.Wegener's granulomatosis presenting with bilateral facial nerve palsy. Acta ORL 2008;26(4): 209-259 55 Rezende CEB, Rodrigues REC, Yoshimura R, Uvo IP, Rapoport PB. Wegener's granulomatosis: a case report. Rev Bras Otorrinolaringol 2003;69:261-265 66 Cahali S, Souza MMA, Silveira MC, Cabali MB, Cahali RB.Wegener's granulomatosis-case report with otological manifestation as first symptom. Braz J Otorhinolaryngol 1997;63(1):72-74 However, WG is not a common diagnosis in ear, nose, and throat (ENT) practice. We describe a case of a 50-year-old woman with severe bilateral hearing loss as the first symptom of the disease.
Differential Diagnosis
The differential diagnosis of WG with otologic manifestations may include tuberculous otitis media, cholesteatoma, Langerhans cell histiocytosis, neoplastic diseases, and other forms of vasculitis, sarcoidosis, and systemic lupus erythematosus.
Tuberculous otitis media is characterized by painless otorrhea that fails to respond to the usual antimicrobial treatment in a patient with evidence of tubercle infection elsewhere, followed by multiple tympanic membrane perforations, abundant granulation tissue, bone necrosis, and preauricular lymphadenopathy. Deafness is out of proportion with the apparent degree of development of disease seen in the otoscopy.
An ear exam may show a pocket or perforation in the eardrum often with drainage when cholesteatoma occurs.
Sarcoidosis is an idiopathic disease that presents in anatomic areas of concern to otorhinolaryngologists. It can cause dysfunction of both auditory and vestibular systems. In patients known previously to have sarcoidosis, this disease should be seriously considered. In patients presenting with otologic disorders and associated facial nerve paralysis or other neuropathies, uveitis, granulomatous meningitis, or diabetes insipidus, sarcoidosis should be suspected. An examination of the eyes as well as a chest X-ray is imperative. Sudden and fluctuating neurosensory hearing loss has been reported.
Case Report
The patient presented with complaints of severe and progressive bilateral hearing loss with onset 6 months previously. Symptoms evolved with otorrhea and pain refractory to broad-spectrum antibiotics and nonsteroidal anti-inflammatory drugs. During ENT examination, otoscopy showed bilateral thickening and retraction of the tympanic membrane and fluid in the middle ear, with no other clinical findings. After carrying out the audiometry (Fig. 1) and emittanciometry, ventilation tubes were placed bilaterally.
After 1 month, the patient complained of weight loss (7 kg/mo), lack of appetite, fevers, night sweats, asthenia, and dry cough. Laboratory findings revealed mild normochromic normocytic anemia, Westergren erythrocyte sedimentation elevated rate, elevated C-reactive protein, nonreactive Mantoux tuberculin skin test, negative sputum smear, and negative HIV antibody.
Computed tomography of the temporal bone (Fig. 2) and audiometry were performed. Chest computed tomography showed the presence of pulmonary nodules and masses with excavation areas in the left upper and lower lobes, with moderate pericardial effusion (Fig. 3). Bronchoscopy with transbronchial biopsy showed inconclusive pathologic result, with the presence of discrete fibrous thickening and septal fibrosis nodular focus.
Chest computed tomography showed the presence of pulmonary nodules and masses with excavation areas in the left upper and lower lobes and moderate pericardial effusion.
The patient was also referred to the Department of Rheumatology for antineutrophil cytoplasmic antibodies testing, which was positive, and then antiproteinase 3 antibody testing, which was also positive, confirming the diagnosis.
With the onset of steroid (prednisone 60 mg/d), the patient's symptoms improved and hearing thresholds recovered significantly after 1 week of treatment (Fig. 4). The patient currently uses cyclophosphamide monthly and corticosteroids daily with good clinical and laboratory control.
Discussion
WG is a rare idiopathic disease, immunologically mediated, which affects the small arteries of the upper and lower respiratory tracts and the kidneys, causing inflammation with necrosis, granuloma formation, and vasculitis in these organs.11 Rossini BAA, Bogaz EA, Yonamine FK, Testa JRG, Penido N de O. Refractory otitis media as the first manifestation of Wegener's granulomatosis. Braz J Otorhinolaryngol 2010;76(4):541 22 Scalcon MRR, Pereira IA, Rachid Filho A, Paiva ES. Manifestação otológica localizada em paciente com granulomatose deWegener. Rev Bras Reumatol 2008;48(4):253-255 33 Antunes T, Barbas CSV. Granulomatose de Wegener. J Bras Pneumol 2005;31(Suppl 1):S21-S26 44 Pires APBA, Sousa NJA, Sousa RCA, et al.Wegener's granulomatosis presenting with bilateral facial nerve palsy. Acta ORL 2008;26(4): 209-259 The average age of diagnosis is usually between 20 and 40 years, and males are affected more than females (1.5:1.0).55 Rezende CEB, Rodrigues REC, Yoshimura R, Uvo IP, Rapoport PB. Wegener's granulomatosis: a case report. Rev Bras Otorrinolaringol 2003;69:261-265 Otologic manifestation varies widely, occurring in ∼20 to 60% of the affected patients, and includes serous otitis media, chronic otitis media, sensorineural hearing loss, vertigo, tinnitus, and facial palsy.44 Pires APBA, Sousa NJA, Sousa RCA, et al.Wegener's granulomatosis presenting with bilateral facial nerve palsy. Acta ORL 2008;26(4): 209-259 66 Cahali S, Souza MMA, Silveira MC, Cabali MB, Cahali RB.Wegener's granulomatosis-case report with otological manifestation as first symptom. Braz J Otorhinolaryngol 1997;63(1):72-74 Treatment should be started early for a better hearing outcome.77 Takagi D, Nakamaru Y, Maguchi S, Furuta Y, Fukuda S. Otologic manifestations ofWegener's granulomatosis. Laryngoscope 2002; 112(9):1684-1690 Pulmonary manifestations occur in 45% of cases at initial presentation and 87% during the course of the disease.88 Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983;98(1):76-85 99 Cordier JF, Valeyre D, Guillevin L, Loire R, Brechot JM. Pulmonary Wegener's granulomatosis. A clinical and imaging study of 77 cases. Chest 1990;97(4):906-912 Ocular involvement in WG can be part of the initial presentation of the disease in 8 to 16% of cases. The majority of studies have reported initial manifestations in ENT, followed by lung, skin, and kidneys.33 Antunes T, Barbas CSV. Granulomatose de Wegener. J Bras Pneumol 2005;31(Suppl 1):S21-S26 1010 Correa JC, Azevedo AG, Rubens J, Rocha G. Granulomatose de Wegener: análise de dois casos. J Bras Med 1985;48(6): 34-38 Immunosuppressive drugs are the first-line therapy.11 Rossini BAA, Bogaz EA, Yonamine FK, Testa JRG, Penido N de O. Refractory otitis media as the first manifestation of Wegener's granulomatosis. Braz J Otorhinolaryngol 2010;76(4):541 22 Scalcon MRR, Pereira IA, Rachid Filho A, Paiva ES. Manifestação otológica localizada em paciente com granulomatose deWegener. Rev Bras Reumatol 2008;48(4):253-255 55 Rezende CEB, Rodrigues REC, Yoshimura R, Uvo IP, Rapoport PB. Wegener's granulomatosis: a case report. Rev Bras Otorrinolaringol 2003;69:261-265
Final Comments
In cases of acute ear manifestations with or without hearing loss and with poor response to usual treatments, WG should be included among the possible etiologies. As the prognosis depends on early diagnosis and appropriate treatment, the otolaryngologist plays a decisive role in reducing the morbidity and mortality rate in this disease.
References
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1Rossini BAA, Bogaz EA, Yonamine FK, Testa JRG, Penido N de O. Refractory otitis media as the first manifestation of Wegener's granulomatosis. Braz J Otorhinolaryngol 2010;76(4):541
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2Scalcon MRR, Pereira IA, Rachid Filho A, Paiva ES. Manifestação otológica localizada em paciente com granulomatose deWegener. Rev Bras Reumatol 2008;48(4):253-255
-
3Antunes T, Barbas CSV. Granulomatose de Wegener. J Bras Pneumol 2005;31(Suppl 1):S21-S26
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4Pires APBA, Sousa NJA, Sousa RCA, et al.Wegener's granulomatosis presenting with bilateral facial nerve palsy. Acta ORL 2008;26(4): 209-259
-
5Rezende CEB, Rodrigues REC, Yoshimura R, Uvo IP, Rapoport PB. Wegener's granulomatosis: a case report. Rev Bras Otorrinolaringol 2003;69:261-265
-
6Cahali S, Souza MMA, Silveira MC, Cabali MB, Cahali RB.Wegener's granulomatosis-case report with otological manifestation as first symptom. Braz J Otorhinolaryngol 1997;63(1):72-74
-
7Takagi D, Nakamaru Y, Maguchi S, Furuta Y, Fukuda S. Otologic manifestations ofWegener's granulomatosis. Laryngoscope 2002; 112(9):1684-1690
-
8Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983;98(1):76-85
-
9Cordier JF, Valeyre D, Guillevin L, Loire R, Brechot JM. Pulmonary Wegener's granulomatosis. A clinical and imaging study of 77 cases. Chest 1990;97(4):906-912
-
10Correa JC, Azevedo AG, Rubens J, Rocha G. Granulomatose de Wegener: análise de dois casos. J Bras Med 1985;48(6): 34-38
Publication Dates
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Publication in this collection
July-Sept 2015
History
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Received
09 May 2014 -
Accepted
01 July 2014