Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by a combination of arterial or venous thrombosis and recurrent fetal loss. It may be primary or in association with an underlying systemic disease, particularly systemic lupus erythemathosus.
The diagnostic criteria include vascular thrombosis, complications during pregnancy with recurrent losses, positive titers of anticardiolipin and lupus anticoagulant antibodies.
Vascular thrombosis can occur anywhere in the body, including lung vessels. The association of APS with pulmonary arterial hypertension has been estimated between 1.8% and 3.5%.
Methods:
Case Presentation: We present an 18 month old male boy, who was admitted to our hospital with a history of 3 previous episodes of pneumonia, at arrival with pulse saturation of 69% without oxygen, improving to 100% with oxygen. Pulmonary arterial hypertension was documented with echocardiogram resulting in 100mmHg without structural abnormalities. An autoimmune etiology was suspected, resulting with high titers of anticardiolipin (IgG 34.6 mg/dl) and anti-b2 glicoprotein (IgG >200mg/dl, IgA 53 mg/dl).
Results:
Treatment with acenocumarine, hydroxichloroquine and prednisone was initiated. Nowadays he has evolved with clinical improvement, on its last echocardiogram the pulmonary arterial tension resulted in 65mmHg, without evidence of thrombosis.
Conclusions:
We report an infant with pulmonary arterial hypertension as the only manifestation of antiphospholipid syndrome, with clinical improvement with anticoagulant and steroid therapy.