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DIFFUSE ALVEOLAR HEMORRHAGE SYNDROM

DIFFUSE ALVEOLAR HEMORRHAGE SYNDROM. Katarina Osolnik University Clinic of Respiratory and Allergic Diseases, Golnik, Slovenia Portorož, May 8th 2009. DIFFUSE ALVEOLAR HEMORRHAGE. acute, life-threatening event repeated episodes can lead to: organizing pneumonia

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DIFFUSE ALVEOLAR HEMORRHAGE SYNDROM

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  1. DIFFUSE ALVEOLAR HEMORRHAGE SYNDROM Katarina Osolnik University Clinic of Respiratory and Allergic Diseases, Golnik, Slovenia Portorož, May 8th 2009

  2. DIFFUSE ALVEOLAR HEMORRHAGE • acute, life-threatening event • repeated episodes can lead to: • organizing pneumonia • collagen deposition in small airways • fibrosis

  3. DIFFUSE ALVEOLAR HEMORRHAGE • Wegener granulomatosis • microscopic polyangiitis • Goodpasture syndrome • connective tissue disorders • antiphospholipid antibody sy • infectious or toxic exposures • neoplastic conditions

  4. CAUSES OF DIFFUSE ALVEOLAR HEMORRHAGE • vasculitis or capillaritis • pulmonary haemorrhage without capillaritis or vasculitis (»bland« pulmonary haemorrhage) • alveolar bleeding associated with another process or condition

  5. CLINICAL MANIFESTATIONS Acute or subacute (present for less than a week) • dyspnea, • cough, • fever, • haemoptysis are the most common clinical manifestations of DAH. *Haemoptysis may be absent at time of presentation in up to a third of patients.

  6. DIAGNOSTIC EVALUATION Chest X-ray • diffuse, bilateral consolidation or ground-glass opacities due to alveolar filling • distributed in the perihilar regions, sparing the apices and costophrenic angels

  7. DIAGNOSTIC EVALUATION HRCT • better evaluate the extent of disease • more sensitive in identifying ground-glass opacities, but not more specific

  8. Laboratory tests anemia, leukocytosis  ESR,  CRP  blood urea and serum creatinine, abnormal findings of urin analysis in pulmonary-renal sy anti-GBM, ANCA, C3 and C4, anti-ds-DNA, antiphospholipid Ab Pulmonary functiontest increased diffusing capacity restrictive changes obstructive changes DIAGNOSTIC EVALUATION

  9. DIAGNOSTIC EVALUATION Bronchoscopy • to document alveolar hemorrhage by BAL • to exclude airway sources of bleeding • to exclude an associated infection Within the first 48 hours of symptoms the diagnostic yield is higher!

  10. BAL • is the method of choice • by showing free red blood cells and hemosiderin-laden, iron-positive macrophages

  11. BAL WITH IRON +AM GOLNIK 2004-2009(64+/84staining samples) • vasculitis or capillaritis 29% • pulmonary haemorrhage without capillaritis or vasculitis (»bland« pulmonary haemorrhage) 18% • alveolar bleeding associated with another process or condition 39% ................................................................... • pneumoconiosis 14%

  12. vasculitis or capillaritis: 58% sistemic vasculitis 42% connective tissue disorders pulmonary haemorrhage without capillaritis or vasculitis: 66% drugs 17% infective endocarditis BAL WITH IRON +AM GOLNIK 2004-2009

  13. BAL WITH IRON +AM GOLNIK 2004-2009 alveolar bleeding associated with another process or condition: • 48% infections • 32% sarcoidosis • 20% malignant conditions

  14. TREATMENT OF DAH • combination of treatment autoimmune destruction of the alveolare capillary membrane and the underlaying condition • immunosupresive agents are the mainstay of therapy, especially if DAH is associated with systemic or pulmonary vasculitis, Goodpasture syndrome or conective tissue disorders • treatment of small vessel vasculitis of the lung is largely the same, regardless of aetiology or whether it is isolated to the lung or a component of a systemic disease

  15. TREATMENT OF DAH Immunosupresive agents • Methylprednisolone and • Cyclophosphamide are the mainstay of therapy. • Plasmapheresis - clinical benefit in Goodpasture syndrome • Recombinant activated human factor VII- successful in several case reports of treating alveolar hemorrhage due to allogenic hematopoietic stem cell transplantation, ANCA associated vascullitis, SLE or antiphospholipid syndrome.

  16. TREATMENT OF DAH-other possible management measures: • supplemental oxygen, • bronchodilators, • reversal of any coagulopathy, • intubation with bronchial tamponade, • protective strategies for the less involved lung, • mechanical ventilation should be done in the course of the disease if they are needed.

  17. CONCLUSION • DAH can be a catastrophic illness if recognition and treatment are delayed. • Diagnosis is often aided by other systemic findings, associated illnes and serological results. • Patients with unexplained isolated DAH should undergo a lung biopsy with immunofluorescent studies and routine histological tests. • During therapy close monitoring, due to potential complications of treatment and the possibility to relapses, is needed.

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