Abstract:Descreve-se o caso de um paciente com Granulomatose de Wegener, em tratamento com esteróides e ciclofosfamida, que, apesar do tratamento, evoluiu com dispnéia progressiva e rouquidão. A investigação mostrou tratar-se de uma estenose subglótica, sendo a dispnéia prontamente aliviada com a traqueostomia. São discutidos diagnósticos diferenciais e modalidades de tratamentos de estenose subglótica em portadores de Wegener.
“…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.…”
“…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.…”
“…Two of the following criteria, accompanied by positive ANCA-c, confirm the diagnosis: oral or nasal inflammation; nodules or fixed opacities or cavitation on chest X-rays; microscopic hematuria or more than 5 erythrocytes per high-power microscopic field; and a biopsy finding of granulomatous inflammation (in the arterial wall or the perivascular/extravascular region of arteries and arterioles). (7,8) The presence of two or more criteria confers 88.2% sensitivity and 92.0% specificity. (7) The classical treatment for WG includes prednisone (1 mg/kg/day), accompanied by cyclophosphamide (2-3 mg/kg/day), for 4 to 6 weeks, the cyclophosphamide dose being adjusted according to the lymphocyte count (which should be maintained at approximately 1,000 cells/mm 3 ) and the prednisone being tapered over the subsequent 6 months.…”
Section: Introductionmentioning
confidence: 99%
“…(7,8) The presence of two or more criteria confers 88.2% sensitivity and 92.0% specificity. (7) The classical treatment for WG includes prednisone (1 mg/kg/day), accompanied by cyclophosphamide (2-3 mg/kg/day), for 4 to 6 weeks, the cyclophosphamide dose being adjusted according to the lymphocyte count (which should be maintained at approximately 1,000 cells/mm 3 ) and the prednisone being tapered over the subsequent 6 months. (1,8) We describe a case of WG with an uncommon initial presentation-a single tumor in the right lung.…”
A granulomatose de Wegener é uma doença inflamatória multissistêmica de etiologia desconhecida, que se caracteriza por vasculite de pequeno e médio calibre. As manifestações clínicas mais comuns envolvem o trato respiratório superior, pulmões e rins e as alterações encontradas nos exames de imagem são geralmente opacidades pulmonares ou nódulos múltiplos, bilaterais e, em 50% dos casos, cavitações. O tratamento é feito com corticoides e imunossupressores. Descreve-se um caso atípico de um homem de 61 anos cuja investigação inicial de um tumor pulmonar unilateral evidenciou, na verdade, Granulomatose de Wegener.
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