Journals / Toraks Dergisi / 2002 / Cilt: 3 - Sayı: 1
Alveolar hemorrhage syndromes; Clinical, pathological and imaging features: Analysis of eleven patients
- Journal
- Toraks Dergisi
- Pages
- 52–58
- DOI
- —
Abstract
Alveolar hemorrhage syndromes (AHS), caused by a group of heterogenous disorders, are characterized by the presence of widespread hemorrhage into alveolar spaces from damaged alveolar septa, leading to dyspnea, hemoptysis anemia to evaluate the diagnostic and therapeutic, anemia and bilateral alveoler infiltrates on chest radiograph. This study was aimed to evaluate the diagnostic and therapeutic methods and underlying etiological factors of these rare syndromes. Eleven patients (8 male, 3 female, mean age 40±17 years) diagnosed as AHS were investigated retrospectively. In addition to the clinical, serological and radiological investigation, the patients were diagnosed by bronchoalveolar lavage (BAL), transbronchial lung biopsy (TBLB), post-mortem lung biopsy (PMLB) and sputum examination. The underlying diseases were Good-Pasteur (G-P) syndrome, microscopic polyangiitis (MP), Behçet's syndrome, systemic vasculitis, idiopathic pulmonary hemosiderosis (IPH), warfarin overdose and pulmonary hypertension due to mitral stenosis and chronic renal failure (CRF). The patients with Behçet's disease and systemic vasculitis were treated with pulse steroid, the patients with G-P and MP had pulse cyclosphosphamide, oral steroid and plasmapheresis was performed, the patient with warfarin overdose was treated with steroid, fresh plasma and K vitamin, the patient with IPH had oral steroid, the patient with chronic renal failure was placed on hemodialysis and had desmopressin treatment. Six patients presented with acute respiratory failure, four of them requiring mechanical ventilation. Seven patients (64%) were died in spite of treatment. As a result, diffuse alveolar hemorrhage (DAH) is a catastrophic complication of a multitude immune and nonimmune disorders, requiring early diagnosis and investigations before respiratory failure develops.
Özet
Alveoler hemorajik sendromlar (AHS), alveoler septalardaki harabiyete bağlı, alveoler boşluklar içine oluşan yaygın kanama sonucu, dispne, hemoptizi, anemi ve akciğer grafisinde bilateral alveoler konsolidasyonlara yol açan bir grup heterojen hastalıktır. Bu çalışma sık görülmeyen bu sendromların, altta yatan etiyolojik faktörleri, tanı ve tedavi yöntemlerini değerlendirmek amacıyla planlandı. Alveoler hemoraji tanısıyla izlenen 11 hasta (8E, 3 K, ortalama yaş 40±17) geriye dönük olarak incelendi. Klinik, radyolojik ve serolojik incelemelerin yanı sıra, bronkoalveoler lavaj (BAL), transbronşiyal akciğer biyopsisi (TBAB), postmortem akciğer biyopsisi (PMAB) ve balgam incelemesiyle tanı koyuldu. Altta yatan hastalıklar Good-Pasteur sendromu (G-P), mikroskopik polianjit (MP), sistemik lupus eritamatoz (SLE), sistemik vaskülit, Behçet hastalığı, idiyopatik pulmoner hemosiderosis (İPH), varfarin aşırı dozuyla birlikte mitral darlığı (MD) ve kronik böbrek yetersizliğiydi (KBY). Sistemik vaskülit ve Behçet hastalığı olan hastaya pulse steroid, G-P sendromlu ve MP'li olgularda pulse siklofosfamid, oral steroid ve plazmaferez, varfarin aşırı dozu ve MD olan hastaya steroid, taze plazma ve K vitamini, İPH'li olguya oral steroid ve kan transfüzyonu, KBY'li hastaya tekrarlayan hemodiyaliz ve desmopressin tedavisi yapıldı. Altı hastada akut solunum yetersizliği vardı, dördü mekanik ventilatöre gereksinim gösterdi. Yedi hasta (%64) tedaviye rağmen yaşamını yitirdi. Sonuç olarak diffüz alveoler hemoraji (DAH), solunum yetersizliği gelişmeden önce erken tanı ve araştırmalara gereksinim gösteren çok sayıdaki immün ve nonimmün hastalığın kötü sonuçlanan bir komplikasyonudur.