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Abstract

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Article in Japanese

Case Report

A case of ANCA negative limited form of granulomatosis with polyangitis having facial paralysis

Chihiro Nishioa  Norio Kokanb  Eiji Katsuyamac  Hiromi Tomiokaa 

aDepartment of Respiratory Medicine, Kobe City Medical Center West Hospital
bDepartment of Otorhinolaryngology, Kobe City Medical Center West Hospital
cDepartment of Pathology, Kobe City Medical Center West Hospital

ABSTRACT

An 85-year-old woman presented with rapidly progressive hearing loss, dyspnea, and multiple nodular shadows on her chest X-ray. On examination, she had bilateral exudative otitis media, sore throat, hemoptysis, fever, and polyarthralgia. Bronchoscopic examinations showed edematous airway mucosa, and transbronchial lung biopsy revealed necrotic granulomatous inflammation surrounded by epitheloid cells with multinucleated giant cells. The diagnosis of limited type of granulomatosis with polyangitis (Wegener's granulomatosis) was confirmed despite the absence of ANCA. She developed facial paralysis just before the diagnosis. Immunosuppressive therapy with cyclophosphamide, prednisolone, and sulfamethoxazole-trimethoprim was initiated. All of the above-described clinical symptoms improved rapidly. Facial nerve paralysis in association with granulomatosis with polyangitis is rare, but clinicians should consider this disease in the list of differential diagnoses even in the case of ANCA negativity.

KEYWORDS

Limited form of granulomatosis with polyangitis  ANCA negative  Facial paralysis  Mediastinal lymphadenopathy  Tracheobronchial involvement 

Received 23 Jan 2013 / Accepted 3 Jun 2013

AJRS, 2(5): 584-587, 2013

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