Large pulmonary vessel thrombosis causing severe pulmonary hypertension insystemic lupus erythematosus.

We describe a 34 year old male patient with systemic lupus erythematosus (S LE) who developed pulmonary hypertension (PHT) associated with anticardioli pin antibodies. Ten years after diagnosis, an helical CT scan revealed thro mbosis of the major pulmonary arteries, which was confirmed by digital pulm onary angiography. An inferior caval vein filter was placed and oral antico agulation was begun. The patient refused pulmonary thromboendarterectomy an d died soon thereafter. Even though PHT is widely described associated with SLE, proximal thrombosis of pulmonary arteries is exceptional. We describe such a patient review the different etiologies, incidence and diagnostic a pproach of PHT in SLE, focusing on those patients with an effective treatme nt.