Table 1.

Major microvascular and nonthrombotic manifestations of APS

Microvascular manifestations

Renal (aPL nephropathy)

Acute—thrombotic microangiopathy

Chronic (ie, fibrous intimal hyperplasia, focal cortical atrophy, tubular thyroidization, glomerular ischemia, interstitial fibrosis, tubular atrophy, organized thrombi with or without recanalization)

Pulmonary (diffuse alveolar hemorrhage)

Cardiac (microvascular disease)

Dermatologic (livedo with/without skin ulcers)

Nonthrombotic manifestations

Thrombocytopenia

Immune mediated

Thrombotic microangiopathy related

Hemolytic anemia

Immune mediated

With schistocytes and thrombotic microangiopathy

Cardiac valve vegetations or thickening

Neurologic*

Cognitive dysfunction in the absence of stroke

Subcortical white matter changes

^*Due to multiple mechanisms, including small vessel ischemic events and the direct pathogenic role of aPL.