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Diffuse alveolar hemorrhage resulting from pauci-immune pulmonary capillaritis

Hemorragia alveolar difusa por capilarite pulmonar pauci-imune

Andreia Salarini Monteiro, Gabriela Addor, David Henrique Nigri, Wilhermo Torres, Carlos Alberto de Barros Franco

ABSTRACT

A 27 year-old female patient, cocaine user, presenting hemoptysis and progressive dyspnea with onset 48 hours prior to hospital admission, without any other signs or symptoms. Serum tests for infectious diseases, collagen disorders and vasculitis were negative. Urinalysis was type I. Computed tomography of the chest showed diffuse alveolar infiltrate, affecting mainly the lower left lobe. A thoracoscopic lung biopsy was performed to clarify the diagnosis. The histopathological findings showed capillaritis and diffuse intra-alveolar hemorrhage. Treated with steroid and cyclophosphamide pulse therapy, a good clinical and radiographical response was obtained. The recently described pauci-immune pulmonary capillaritis is characterized by the presence of isolated pulmonary capillaritis and negative serum testing for auto-immune diseases.

Keywords: Hemorrhage. Pulmonary alveoli/physiopathology.

RESUMO

Paciente de 27 anos, usuária de cocaína, apresentando hemoptise e dispnéia progressiva de início 48 horas antes da internação, sem outros sinais e sintomas. Sorologias para doenças infecciosas, doenças do colágeno e vasculites negativas. Exame de urina tipo I. Tomografia computadorizada de tórax com infiltrado alveolar difuso, principalmente em lobo inferior esquerdo. Submetida a biópsia pulmonar por toracoscopia para esclarecimento diagnóstico. Histopatológico mostrou capilarite e hemorragia alveolar difusa. Tratada com pulsoterapia com esteróide e ciclofosfamida, obteve boa resposta clínica e radiológica. Recentemente descrita, a capilarite pulmonar pauci-imune é caracterizada pela presença de capilarite pulmonar isolada e sorologias para doenças auto-imunes negativas.

Palavras-chave: Hemorragia. Alvéolos pulmonares/fisiopatologia.


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