41 - The catastrophic antiphospholipid syndrome

Abstract

A widespread coagulopathy, affecting predominantly small vessels supplying organs, rather than the usual large vessel occlusions of larger veins and arteries seen in the classic antiphospholipid syndrome (APS), in association with elevations of anti-phospholipid antibodies (aPL) (often high levels), was defined as the catastrophic APS. The majority of patients suffering from catastrophic APS have been found to be suffering from defined systemic lupus erythematosus (SLE) or the primary APS, while a minority have had primary Sjögren's syndrome, rheumatoid arthritis, systemic sclerosis, ulcerative colitis, or relapsing polychondritis. The catastrophic APS may occur in two situations where an underlying hypercoagulable state already exists or develops, that is, carcinoma and the puerperium. A high index of clinical suspicion is necessary for the diagnosis of catastrophic APS in patients with evidence of both large and small vessel occlusion, affecting multiple organs and systems. A careful differential diagnosis is necessary in the presence of multiorgan thrombotic events in a particular patient and this should include catastrophic APS and other microangiopathic syndromes, which have in common thrombotic microangiopathy, hemolytic anemia, thrombocytopenia, and involvement of central nervous, and renal systems. The optimal management of catastrophic APS is not known but must have three clear aims: to treat any precipitating factors, to prevent and to treat the ongoing thrombotic events, and to suppress the excessive cytokine storm.