Microangiopathy and microvascular thrombosis with APS (MAPS)

What is microangiopathy and microvascular thrombosis with APS (MAPS)?

MAPS refers to microangiopathy and microvascular thrombosis that can occur in patients with APS. Patients with classic PAPS and SAPS typically have clots predominantly in medium or larger vessels. However, it has become increasingly recognized that small vessels in the visceral organs may develop endothelial proliferation with or without clot leading to clinical manifestations resulting from ischemia and microinfarcts (livedoid vasculopathy with skin necrosis, cerebral microinfarcts with cognitive dysfunction, autonomic dysfunction, GI/pancreatic/ hepatic/splenic microinfarcts, alveolar hemorrhage, chronic vaso-occlusive lesions with renal insufficiency, ischemic cardiomyopathy, pulmonary hypertension without major emboli, fibrosing alveolitis, hearing loss, bone marrow infarction, others). In some of these patients, profound endothelial injury occurs in these microvessels leading to a microangiopathy with microangiopathic hemolytic anemia (schistocytes, high lactate dehydrogenase levels), severe thrombocytopenia (<20,000), and thrombosis of multiple small vessels. In patients with catastrophic antiphospholipid syndrome (CAPS), MAPS predominates over the larger vessel thrombosis typical of classic APS.

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