NOTE: A patient who meets the clinical criteria (vascular thromboses and/or pregnancy morbidity) for the antiphospholipid syndrome is excluded from the definition.
Features:
(1) laboratory features of the antiphospholipid syndrome, with the presence of:
(1a) lupus anticoagulant
(1b) anticardiolipin antibody
(1c) anti-beta-glycoprotein I antibody
(1d) mixture of 2 or more types
(2) presence of one or more of the following on renal biopsy:
(2a) thrombotic microangiopathy involving both glomerular capillaries and arterioles
(2b) fibrous intimal hyperplasia involving organized thrombi
(2c) fibrous and/or fibrocellular occlusions of arteries and arterioles
(2d) focal cortical atrophy
(2e) areas of atrophic tubules containing eosinophilic casts ("tubular thyroidization")
(3) exclusion another explanation for the renal disease including:
(3a) vasculitis
(3b) thrombotic thrombocytopenic purpura (TTP)
(3c) hemolytic uremic syndrome (HUS)
(3d) malignant hypertension
where:
• Focal cortical atrophy can be seen in many adult kidneys, so by itself is probably not a good indication of a nephropathy.
If the patient has SLE, then the lesions should be distinguished from those of lupus nephropathy (both conditions may co-exist).
