Catastrophic APS — A small subset of patients with APS has widespread thrombotic disease
with visceral damage, referred to as the "catastrophic" APS . Preliminary classification criteria for this disorder have been proposed .
The prognosis for such patients without treatment is poor. The prognosis may be better with intensive treatment. The combination of anticoagulation, glucocorticoids, and plasma exchange with or without intravenous immune globulin has been associated with recovery rates ranging from 50 to 80 percent .
A majority of patients with catastrophic APS who survive their initial illness remain free of further thromboembolic events when treated long-term with warfarin
. This was illustrated in an observational study of 58 survivors followed for an average of 67 months . Two-thirds had no recurrent clotting or emboli. Approximately 20 percent had recurrent APS-related events, but none had another episode of multiorgan failure. Among the recurrent thromboembolic events, 40 percent occurred in a perioperative period.
Recommended management for patients with the catastrophic APS consists of the following:
Treatment of any identifiable disorder that may have precipitated the catastrophic APS (eg, infection).
Anticoagulation with heparin in the acute setting, followed by long-term warfarin.
High-dose glucocorticoids (eg, methylprednisolone 1 g intravenously daily for three days) followed by oral or parenteral therapy with the equivalent of 1 to 2 mg/kg of prednisone
If there are features of microangiopathy
, microangiopathic hemolytic anemia
), plasma exchange (see below) with or without IVIG (eg, 400 mg/kg per day for five days) are added to the above regimen.
so you donot have catastrophic aps...and secondly you had heamorrhagic infarcts in bRAIN WHICH APS DOESNT DO...IT CAUSES ISCHEMIC INFARSCTS....u are having vasculitis
of brain and then rupture due to
do an mra with mrv and you need high does immunosuppresants