DIFFUSE ALVEOLAR HEMORRHAGE SYNDROM

Katarina OsolnikUniversity 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

• collagen deposition in small airways

• fibrosis

DIFFUSE ALVEOLAR HEMORRHAGE

• Wegener granulomatosis

• microscopic polyangiitis

• Goodpasture syndrome

• connective tissue disorders

• antiphospholipid antibody sy

• infectious or toxic exposures

• neoplastic conditions

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

CLINICAL MANIFESTATIONSAcute 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.

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

DIAGNOSTIC EVALUATION

HRCT• better evaluate the

extent of disease • more sensitive in

identifying ground-glass opacities, but not more specific

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

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!

BAL

• is the method of choice

• by showing free red blood cells and hemosiderin-laden, iron-positive macrophages

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%

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

BAL WITH IRON +AM GOLNIK 2004-2009

alveolar bleeding associated with another process or condition:

• 48% infections

• 32% sarcoidosis

• 20% malignant conditions

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

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.

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.

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.