Jaimes CYC, Leonardi CFL, Bracamonte W, Lesseur C

Language: Spanish
References: 12
Page: 40-44
PDF size: 57.40 Kb.

ABSTRACT

Wegener's granulomatosis (GW) is a systemic vasculitis of small vessels and apparent autoimmune etiology characterized by formation of granulomas in small and medium vessels, compromising mainly respiratory system and kidney. The forward case refers to a twelve year old female patient who affirms beginning of current disease on October 2007, with the appearance bipalpebral bilateral edema left predominant, wich increase gradually, exophthalmos and impossibility of the eyelid closure and then diplopia with lateralization of gaze to the left, without disturbances in visual acuity. Concomitantly, intermittent fever 39°C without chills or sweating. Since April 2008, dry cough associated to cianosis and emesis and also selflimited dyspnea episodes at rest. In June 2008, she was ingressed to Hospital Universitario de Caracas, where imaging studies show that retroorbital bilateral tumor, being refered to resection, with transient improvement and subsequent recurrence of the edema. Chest imaging study showed bilateral parahilar nodules. Laboratory studies showed progressive increase in urea and creatinine, renal biopsy was performed and reported vasculitis of small vessels consistent with GW. ANCAc test were negative. Treatment with steroids and cyclophosphamide was started, achieving normalization of renal function, total resolution of respiratory symptoms and significant reduction of exophthalmos.

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