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Vol. 40. Issue 1.
Pages 41-44 (January 2004)
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Vol. 40. Issue 1.
Pages 41-44 (January 2004)
Case Reports
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Polyradiculitis and Wegener's Granulomatosis
Visits
5241
A. Casanovaa, E. Pérez Amora, R.M. Giróna,
Corresponding author
med002861@nacom.es

Correspondence: Dr. R.M. Girón Moreno. Servicio de Neumología. Hospital de la Princesa. Diego de León, 62. 28006 Madrid. España
, E. Zamoraa, M.D. Vélezb, J. Ancocheaa
a Servicio de Neumología, Hospital Universitario de la Princesa, Madrid, Spain
b Servicio de Anatomía Patológica, Hospital Universitario de la Princesa, Madrid, Spain
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Wegener's granulomatosis is a systemic vasculitis of unknown etiology, primarily affecting the upper and lower respiratory tract and the kidneys, although there is a form restricted to the lungs. Wegener's granulomatosis occurs infrequently, with an estimated annual incidence of 8.5 cases per million. The principal symptoms at onset usually involve the upper and lower airway. The central nervous system is involved in approximately 22% of cases, but neurological signs occur infrequently among the initial clinical manifestations. Proteinase 3-specific antineutrophil cytoplasmic antibodies are useful serological markers for establishing a diagnosis, which should, however, be confirmed by a tissue biopsy of the affected organ.

Key words:
Wegener's granulomatosis
Polyneuropathy
Pulmonary vasculitis

La enfermedad de Wegener es una vasculitis granulomatosa sistémica de etiología desconocida, que afecta sobre todo al tracto respiratorio superior e inferior y al riñón, aunque se distingue una forma limitada al pulmón. Es una entidad poco frecuente, con una incidencia estimada de 8,5 por 1.000.000 de habitantes/año. Los principales síntomas de comienzo suelen ser los derivados de la afectación de las vías aéreas superior e inferior. El sistema nervioso central se implica en alrededor de un 22% de los casos, pero es infrecuente que la clínica neurológica forme parte de la sintomatología inicial. Los anticuerpos anticitoplasma del neutrófilo específicos contra la proteinasa 3 constituyen marcadores serológicos de gran utilidad para el establecimiento del diag-nóstico, aunque éste debe confirmarse por la biopsia tisular del órgano afectado.

Palabras clave:
Enfermedad de Wegener
Polineuritis
Vasculitis pulmonares
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Copyright © 2004. Sociedad Española de Neumología y Cirugía Torácica (SEPAR)
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