Granulomatose de Wegener

Abstract

A granulomatose de Wegener caracteriza-se por vasculite necrosante granulomatosa que acomete preferencialmente vias aéreas superiores, inferiores e rins. Seu diagnóstico é feito associando-se as manifestações clínicas, radiológicas (multiplos nódulos escavados) e os achados anatomopatológicos e o anticorpo anticitoplasma de neutrófilos positivo. O tratamento com corticosteróides e ciclofosfamida leva a 90% de remissão da doença em 1 ano.

Prevalência das manifestações clínicas iniciais da granulomatose de Wegener no Brasil: relato de seis casos e revisão da literatura

“…Table 4 shows the initial presentation of WG in adults by organs and systems, comparing two Brazilian studies and an international reference. 2,[19][20][21][22][23][24][25][26][27][28][29][30]32,35,[38][39][40]42,44,49,50 Some particularities of the patients seen at HGF should be mentioned: 1) the first patient developed paresthesia in the lower limbs with electroneuromyography showing axonal mononeuropathy of the posterior tibial nerve, a rare symptom in the initial phase of WG.…”

Wegener Granulomatosis: Otologic Manifestation as First Symptom

“…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. 1 2 3 4 The average age of diagnosis is usually between 20 and 40 years, and males are affected more than females (1.5:1.0). 5 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.…”

“…1 2 It is a systemic vasculitis, affecting small and medium-sized vessels of the upper and lower respiratory tract and kidneys, including necrotizing granulomatous inflammation. 2 3 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.…”

Wegener's Granulomatosis with Pulmonary Fungal Infection: A Case Report and Brief Review

“…11 In particular, the severity of the condition in antiproteinase-3 antibody-positive patients progresses rapidly without treatment. 35 It is generally accepted that patients with WG who are of older age, with renal involvement or infection, have worse outcomes and higher mortality rates compared with other patients who develop WG. 36 -40 Infection is the most frequent cause of death.…”