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. DIAGNOSTIC EVALUATION
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 function test
increased diffusing capacity
restrictive changes
obstructive changes

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 %
pulmonary haemorrhage without capillaritis or vasculitis (»bland« pulmonary haemorrhage) %
alveolar bleeding associated with another process or condition %
pneumoconiosis %

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

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.