Clinical-biological and radiological study of Wegener's granulomatosis in a university hospital
Ríos Blanco, J.J.; Gómez Cerezo, J.; Vázquez Muñoz, E.; Suárez García, I.; López Rodríguez, M.; Yébenes Gregorio, L.; Torres Sánchez, I.; Barbado Hernández, F.J.
Revista Clinica Espanola 205(8): 367-373
2005
ISSN/ISBN: 0014-2565 PMID: 16143083 Document Number: 586317
Wegener's granulomatosis is a rare systemic vasculitis that fundamentally affects the kidney and respiratory tract. Although the histological findings are very characteristic, they do not occur in all cases, which makes diagnosis difficult. The main clinical, biological and radiological characteristics of 13 cases of Wegener's granulomatosis diagnosed in a university hospital of the center of Spain are collected in this present paper. The majority of the patients were men in their 50's. Fever and general syndrome were usual presentations of the disease. Upper respiratory tract (10 cases) and lung involvement (9), followed by renal involvement (8), were the most common. Alveolar infiltrates and bilateral nodules were the most common findings in the X-ray and thoracic CT scan, although the latter permits a greater definition of the lesions. Measurement of antibodies against cytoplasma of the neutrophils (ANCA) was positive in 8 patients (6 cANCA and 2 pANCA). Renal biopsy was used most for the histologic diagnosis, where focal and segmental glomerulonephritis was the most common finding. The majority of the patients received steroids plus cyclophosphamide as induction treatment. Wegener's disease is a rare systemic vasculitis. The existence of hematuria or respiratory tract involvement in a patient with fever suggests the diagnosis. Conducting a CT scan and ANCAs measurement are useful tools for its diagnosis, since the histology is not definitive.