A patient with antiphospholipid antibodies may rarely develop diffuse alveolar hemorrhage (DAH).
Antiphospholipid antibody associated disorders include:
(1) lupus anticoagulant
(2) anticardiolipin antibody
(3) anti-beta-2 glycoprotein I antibodies
Clinical features:
(1) venous and/or arterial thrombosis
(2) thrombocytopenia
(3) cognitive impairment
(4) seizures
(5) migraine headaches
(6) chorea
(7) cardiac valve disease
(8) skin ulcers
(9) nephropathy
Pulmonary manifestations:
(1) pulmonary embolism
(2) pulmonary hypertension
(3) pulmonary microvascular thrombosis
(4) fibrosing alveolitis
(5) diffuse alveolar hemorrhage (DAH)
(6) ARDS
Imaging studies in patients with DAH show bilateral alveolar infiltrates on chest X-ray and ground-glass opacities on CT.
Bronchoalveolar lavage shows blood and hemosiderin-laden macrophages.
Lung biopsy in a patient with DAH may show:
(1) bland hemorrhage
(2) capillaritis
Diagnosis:
(1) evidence of diffuse alveolar hemorrhage
(2) clinical features of antiphospholipid antibody syndrome
(3) serologic evidence of antiphospholipid antibodies
(4) exclusion of other causes of diffuse alveolar hemorrhage
Therapy may include: corticosteroids, plasmapheresis, rituximab, methotrexate, azathioprine, cyclophosphamide, hydroxychloroquine.
