DIFFUSE ALVEOLAR HEMORRHAGE SYNDROM Katarina Osolnik University Clinic of Respiratory and Allergic...

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.