Hemorragia alveolar masiva en la granulomatosis de Wegener / Massive alveolar haemorrhage in Wegener’s granulomatosis

La granulomatosis de Wegener es una vasculitis sistémica primaria granulomatosa con afectación del tracto respiratorio superior e inferior, glomerulonefritis y vasculitis de vasos de pequeño calibre. La afección pulmonar abarca desde nódulos pulmonares asintomáticos a infiltrados pulmonares y hemorragias alveolares fulminantes. El pronóstico es malo debido a insuficiencia renal o respiratoria, aunque las cifras han cambiado gracias a los nuevos tratamientos con glucocorticoides y ciclofosfamida. Se presenta un caso con afectación pulmonar severa que tras adecuada anamnesis, múltiples pruebas y un óptimo proceso secuencial de actuación se diagnostica de la enfermedad descrita previamente. Es una enfermedad con baja incidencia en urgencias, donde la anamnesis del paciente nos ofrece una sospecha diagnóstica apoyada por las pruebas complementarias precisas. Es importante conocer y dominar el manejo de la estabilidad de este tipo de pacientes que permita una resolución de los síntomas por los que acuden a urgencias (AU)

Wegener’s granulomatosis is a systemic vasculitis with involvement of primary granulomatous upper and lower respiratory tract, glomerulonephritis and vasculitis of small vessels. The lung disease ranges from asymptomatic pulmonary nodules to pulmonary infiltrates and fulminant alveolar haemorrhage. The prognosis is poor due to kidney and respiratory failure, although the data are changing due to new treatments with glucocorticoids and cyclophosphamide. We report a case with severe lung disease, which after appropriate anamnesis, multiple tests, and optimal sequential action, the patient was diagnosed with Wegener’s granulomatosis. This disease has a low incidence in the Emergency Department, where the patient history supported by the appropriate additional provides a diagnostic suspicion. It is important that the Emergency Department has the skills to manage the stability in these patients in order toresolve their symptoms (AU)

[Massive alveolar haemorrhage in Wegener's granulomatosis]. / Hemorragia alveolar masiva en la granulomatosis de Wegener.

Wegener's granulomatosis is a systemic vasculitis with involvement of primary granulomatous upper and lower respiratory tract, glomerulonephritis and vasculitis of small vessels. The lung disease ranges from asymptomatic pulmonary nodules to pulmonary infiltrates and fulminant alveolar haemorrhage. The prognosis is poor due to kidney and respiratory failure, although the data are changing due to new treatments with glucocorticoids and cyclophosphamide. We report a case with severe lung disease, which after appropriate anamnesis, multiple tests, and optimal sequential action, the patient was diagnosed with Wegener's granulomatosis. This disease has a low incidence in the Emergency Department, where the patient history supported by the appropriate additional provides a diagnostic suspicion. It is important that the Emergency Department has the skills to manage the stability in these patients in order to resolve their symptoms.