Progressive hearing loss as the leading sign of Wegener’s granulomatosis

Debski, Marcin; Życińska, Katarzyna; Czarkowski, Marek; Żukowska, Małgorzata; Wardyn, Kazimierz; Bar-Andziak, Ewa

doi:10.20452/pamw.150

Cited by 3 publications

(1 citation statement)

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“…In the literature, we identified only nine studies mentioning Wegener's granulomatosis and FUO together (5)(6)(7)(8)(9)(10)(11)(12)(13). In the study of Sipahi et al (3), this disease was identified in only 3 cases out of a total of 857.…”

Section: Discussionmentioning

confidence: 99%

A Case with Fever of Unknown Origin Diagnosed as Wegener Granulomatosis

et al. 2010

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Abst ractFever of unknown origin (FUO) was described in 1961 by Petersdorf and Beeson as a fever above 38.3°C that lasts more than three weeks and cannot be diagnosed despite a one-week hospitalized examination. In 1991, Durack and Street made a different FUO description for patients with nosocomial infections, human immunodeficiency virus (HIV) and neutropenia. Along with this reconsideration, the investigation period was limited to one week of investigation, three days of hospitalization or three clinical visits. In the literature, the most frequent cause of FUO is infections followed by neoplasms and connective vascular diseases, respectively. The etiology in 5-15% of patients can not be diagnosed despite detailed examination. Here, we present a 75-year-old patient admitted to the hospital with a two-week history of fever, fatigue and lack of appetite. He had been examined in the infection and lung diseases clinics over a three-month period without diagnosis. In our internal medicine clinic, we diagnosed the patient as Wegener granulomatosis and a satisfactory response to the therapy was observed. (Turk J Rheumatol 2010; 25: 159-61

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Section: Discussionmentioning

confidence: 99%

A Case with Fever of Unknown Origin Diagnosed as Wegener Granulomatosis

et al. 2010

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Facial nerve paresis in the course of masked mastoiditis as a revelator of GPA

Marszał

Bartochowska

et al. 2021

Eur Arch Otorhinolaryngol

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Purpose The aim of this study was to present a series of 6 patients with facial nerve palsy and masked mastoiditis which constituted as revelators of localized granulomatosis with polyangiitis (GPA) and to evaluate the utility of the ACR/EULAR 2017 provisional classification criteria for GPA in such cases. Methods Study group included 58 patients with GPA. Cases with facial nerve palsy and masked mastoiditis were thoroughly analyzed. Results The mean age of patients was 37 years. All manifested unilateral facial nerve palsy and hearing loss, while only 2 reported aural complaints suggesting inflammatory cause of the disease. All cases were qualified for surgical intervention. Intraoperative findings were similar: granulation tissue in tympanic cavity and/or pneumatic spaces of the mastoid process. Only 50% of histopathological results suggested vasculitis. In all cases, elevated levels of antineutrophil cytoplasmic antibodies (ANCA) against peroxidase 3 (PR3-ANCA) were determined. Two patients presented rapid progression of the disease and died within 1 week and 2 months, respectively. Four other patients manifested gradual improvement of hearing and facial nerve function after treatment. Conclusion GPA should be included into differential diagnosis in all cases of persistent facial nerve palsy especially when otological symptoms coexist. Even localized GPA could be very aggressive, revelating generalized form of the disease. Rapid systemic treatment of GPA can protect hearing and facial nerve from permanent severe dysfunction. The ACR/EULAR 2017 provisional classification criteria for GPA seem to be valuable tool in diagnosing ENT patients with localized otological form of the disease.

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