INTRODUCTION We report the case of a 58-year-old Caucasian Greek man who presented with dry cough, fever, bilateral alveolar infiltrates and acute hepatitis. CASE PRESENTATION After a lung biopsy, the patient was diagnosed with Wegener's granulomatosis. The diagnosis was supported by the presence of anti-proteinase-3 anti-neutrophil cytoplasmic antibodies. A liver biopsy demonstrated the pres...

OBJECTIVE Wegener's granulomatosis is a systemic vasculitis characterized by necrotizing granulomatous lesions mostly involving the upper and lower respiratory tract. The disease rarely causes parotid gland involvement and pneumothorax. We report a case of Wegener's granulomatosis involving parotid gland, and complicated with a pneumothorax. CLINICAL PRESENTATION A 45-year-old man admitted wi...

BACKGROUND Fabry's disease is a rare X-linked, hereditary lysosomal storage disease caused by a deficiency of the enzyme α-galactosidase A. Granulomatosis with polyangiitis is characterized by the involvement of the respiratory tract and kidneys. Here, we report the first case of the coexistence of these diseases. CASE PRESENTATION We describe a 29-year-old man suffering from fever with maxil...

BACKGROUND Pulmonary histoplasmosis is a fungal infection caused by histoplasma capsulatum, rarely diagnosed in non endemic areas and/or immunocompromised patients. Complication of pulmonary histoplasmosis with bronchocentric granulomatosis is extremely rare. CASE REPORT A 48-year-old man with prolonged fever and nausea was admitted to our hospital. Clinical examination revealed pathological ...

1. Wong CF. Rituximab in refractory antineutrophil cytoplasmic antibody associated vasculitis: what is the current evidence? Nephrol Dial Transplant 2007; [Epub ahead of print]. 2. Aries PM, Hellmich B, Voswinkel J et al. Lack of efficacy of rituximab in Wegener’s granulomatosis with refractory granulomatous manifestations. Ann Rheum Dis 2006; 65: 853–858 3. Omdal R, Wildhagen K, Hansen T, Gunn...

UNLABELLED Wegeners Granulomatosis is a condition associated with systemic vasculitis which can present with upper respiratory tract symptoms initially. On September 2001, a 15-year-old girl presented with symptoms of nasal block for 3 weeks. She later developed joint pains and worsening renal status requiring dialysis. A renal biopsy was performed which showed pauci-immune cresentric glomerulo...

The recent case of necrotising sarcoid granulomatosis described by ALIBERTI et al. [1] provides clinicians with an interesting insight into this rare condition. We would like to supplement the paucity of literature surrounding necrotising sarcoid granulomatosis and illustrate a case of our own. This was also in a young patient but did require treatment with oral corticosteroids at initial prese...

Upper airway disease, especially nasal and paranasal sinus involvement, is the most common manifestation of Wegener's granulomatosis. Chronic otitis media and facial palsy are rare but well known presenting features of Wegener's granulomatosis. We report a 40-year-old woman who presented with complaints of ear discharge, deep-seated ear pain and loss of hearing in her right ear. Early diagnosis...

Introduction Granulomatosis with polyangiitis (GPA) (Wegener’s granulomatosis) is a systemic necrotizing disease that involves the main upper and lower respiratory tracts and results in pauci-immune glomerulonephritis in the kidneys and areas supplied by smalland medium-sized arteries (1). If the disease is localized only to the upper respiratory tract or lungs, it is defined as a limited form ...

Ophthalmic involvement is frequent, between 30% and 70% of patient’s present ophthalmic symptoms during the course of Wegner’s granulomatosis. Orbital disease may present initially before the onset of systemic manifestations in only 8 to 16% of patients and it could delay final diagnosis. We report a case illustrates the diagnosis of Wegner’s granulomatosis presenting with proptosis (exophthalm...