Acometimento pulmonar na doença de Behçet: uma boa experiência com o uso de imunossupressores / Pulmonary involvement in Behcet's disease: a positive single-center experience with the use of immunosuppressive therapy

OBJETIVO: A doença de Behçet (DB) representa uma patologia sistêmica, cuja sobrevida se relaciona com a presença de acometimento pulmonar. Entretanto, sugere-se que pacientes com diferentes tratamentos podem apresentar diferentes prognósticos. O objetivo deste estudo foi avaliar a evolução clínica e tomográfica, bem como a sobrevida deste pacientes com acometimento pulmonar relacionado à DB acompanhados em nosso serviço. MÉTODOS: Uma análise retrospectiva de nossa experiência com pacientes com acometimento pulmonar relaionado a DB acompanhados de 1 de Janeiro de 1988 a 30 de Abril de 2006. Os dados clínicos, radiológicos, terapêuticos e de sobrevida foram obtidos dos prontuários médicos. RESULTADOS: Foram identificados 9 pacientes, com idade média de 34 ± 11,5 anos, sendo 7 deles do sexo masculino. Os achados radiológicos foram aneurisma de artéria pulmonar (AAP) em 8 pacientes, embolia pulmonar em 3 (resultando em uma incidencia de 5,11 casos/100 paciente-anos), hemorragia alveolar em 1 e hipertensão pulmonar em 1 de 9 doentes. O tratamento consistiu-se de prednisona mais clorambucil (ou ciclofosfamida ou micofenolato de mofetil) em todos os 9 pacientes, com resolução total ou parcial dos AAP. O paciente com AAP e hipertensão pulmonar também recebeu sildenafil e warfarina, com boa resposta clínica e tomográfica. A sobrevida de nossos pacientes foi de 88,8 por cento em 3 e 5 anos, com acompanhamento médio de 6,52 anos. CONCLUSÕES: Pacientes com acometimento pulmonar relacionado à DB podem apresentar boa sobrevida com tratamento imunossupressor, e a DB deve ser lembrada como uma possível causa de hipertensão pulmonar e hemorragia alveolar.

OBJECTIVE: Behcet's syndrome, or Behcet's disease (BD), is a multisystem pathology, and survival is related to pulmonary involvement. However, it appears that different treatments correlate with different prognoses. The aim of this study was to evaluate clinical and tomographic evolution, as well as the survival, of patients with BD-related pulmonary involvement. METHODS: A retrospective review of our experience with pulmonary manifestations in patients with BD treated at our institution between January 1, 1988 and April 30, 2006. The clinical, radiological, treatment and survival data were obtained from medical charts. RESULTS: We identified 9 patients with BD-related pulmonary involvement. The mean age was 34 ± 11.5 years, and 7 of the patients were male. The radiological findings were as follows: pulmonary artery aneurysm (PAA) in 8 patients; pulmonary embolism in 3 (translating to an incidence of 5.11 cases/100 patient-years); alveolar hemorrhage in one; and pulmonary hypertension in one. The treatment consisted of immunosuppression with prednisone plus chlorambucil (or cyclophosphamide or mycophenolate mofetil) in all patients, with partial or complete resolution of the PAAs. One patient with a PAA and pulmonary hypertension also received sildenafil and warfarin, with good clinical and tomographic response (the first report in the English literature). In our sample, the mean duration of the follow-up period was 6.52 years. The three-year survival rate was 88.8 percent, as was the five-year survival rate. CONCLUSIONS: Patients with BD-related pulmonary involvement can present good survival with immunosuppressive therapy, and BD should be borne in mind as a possible cause of pulmonary hypertension and alveolar hemorrhage.

Estudo da camada elástica das arteríolas pulmonares em portadores de granulomatose de Wegener / Study of the elastic fibers of the pulmonary arteriolas of patients with Wegener granulomatosis

A Granulomatose de Wegener é uma síndrome clínico-patológica caracterizada por vasculite granulomatosa necrozante que acomete o trato respiratório superior, pulmões e rins. A vasculite granulomatosa afeta os vasos pulmonares com destruição das camadas elásticas das artérias.. O objetivo do presente estudo foi quantificar o sistema elástico da parede das artérias pulmonares de pacientes portadores de Granulomatose de Wegener comparado com o de pacientes normais. Foram estudadas 36 artérias de pequeno calibre ( diâmetro < 62 micra) e 36 artérias de médio calibre ( diâmetro entre 62 e 213 micra) obtidas de tecido pulmonar de 6 pacientes portadores de Granulomatose de Wegener comparadas com 36 artérias de pequeno e 36 artérias de médio calibre obtidas de 6 pacientes normais...

Wegener Granulomatosis is a clinico-pathologic syndrome characterized by necrotizing granulomatous vasculitis that affects the upper respiratory tract, the lungs and the kidneys. The granulomatous vasculitis affects the pulmonary vessels with destruction of the elstic system fibers of the vessels walls that can be visualized with the Verhoff staining technique . The objective of the present study was to quantify the area of the elastic system fibers of the walls of the pulmonary arteries of patients with the diagnosis of Wegener Granulomatosis compared to the area of the elastic system fibers of normal patients. We studied 36 small size pulmonary arteries ( diameter < 62 µ) and 36 medium size pulmonary arteries ( diameter between 62 and 213µ) taken from lung tissue of 6 patients with the diagnosis of Wegener Granulomatosis compared to 36 small size and 36 medium size pulmonary arteries taken from lung tissue of normal patients. The elastic system fibers of the pulmonary arteries walls was atained by resorcine-fucsine technique e the área of the elastic system was quantified through a special softer for área calculation of the Image analyser FOX pro of the Faculty of Medicine of the University of São Paulo. The medium area of the elastic system fibers of the small size pulmonary arteries of Wegener Granulomatosis was 73,6 ± 20,9 µ² ( 29,76 to 109,05 µ²) compared to 680,8 ± 256,1 µ² ( 263,34 to 1380,75 µ²) of the normal patients ( p< 0,0001- ANOVA -2 factors)...

Síndrome de Churg-Strauss / Churg-Strauss syndrome

A síndrome de Churg-Strauss caracteriza-se por asma, eosinofilia e graus variados de vasculite sistêmica. As formas mais graves com acometimento cardíaco, gastrintestinal, sistema nervoso central e renal requerem ciclofosfamida para seu tratamento.

Outras vasculites pulmonares / Other forms of pulmonary vasculitis

A poliangeíte microscópica, a arterite de Takayasu, a síndrome de Behçet, a púrpura de Henoch-Schõnlein e as vasculites associadas às doenças do colágeno incluem-se entre as vasculites pulmonares. Seu diagnóstico é realizado associando-se as manifestações clínicas aos achados radiológicos e anatomopatológicos.