Granulomatose de Wegener: relato de caso

Abstract

A Granulomatose de Wegener é uma doença sistêmica, idiopática e rara, com acometimento das pequenas artérias dos tratos respiratórios superior e inferior e rins, levando à necrose e granuloma estes órgãos. Neste trabalho relatamos e discutimos o caso de uma adolescente, do sexo feminino, de apresentação inicial atípica e com evolução favorável após o diagnóstico precoce e tratamento adequados.

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.…”

Pseudotumor pulmonar como apresentação inicial de granulomatose de Wegener

“…What is expected in this type of vasculitis is pulmonary infiltration or bilateral multiple nodules, which can be accompanied by cavitations (in 50% of the cases). (8) The next step was then to resort to tomography-guided percutaneous biopsy, a procedure that has high sensitivity, specificity and accuracy, the last ranging from 84.5% to 90%, depending directly on the size of the lesion. The principal reason for requesting the procedure is to determine malignancy in solid pulmonary lesions.…”

“…(7) A finding of ANCA-c positivity has a sensitivity of 90-95% and a specificity of 90% for WG, whereas up to 60% of cases can test positive for rheumatoid factor (RF). (8) Inflammatory activity is typically elevated.…”

“…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.…”

“…(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.…”