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Table 2 Anti-phospholipid syndrome clinical manifestations not yet considered as classification criteria

From: Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers

Clinical manifestations PAPS APS-SLE Notes
 Thrombocytopenia 20-25% 30-40% Usually mild
No protective effect on thrombotic risk
 Heart valve disease 12-33% 40% Possibly an additional risk for secondary thromboembolism
 Livedo reticularis 20-25% 35% Original association with arterial thrombosis not confirmed in prospective studies
 Ulcers 33% 7-10% Pre-tibial area
Frequently observed in catastrophic APS
 Superficial thrombophlebitis 9% Reported in aPL-positive patients but their value still debated
 Renal artery stenosis 26% of aPL + patients with uncontrolled hypertension Resulting in severe renovascular hypertension, renal infarcts
 APS nephropathy (renal small artery vasculopathy, involving both arterioles and glomerular capillaries) 35%a 39-67% a Association with pregnancy complications, extra-renal vascular thrombosis and higher risk of chronic renal failure among SLE patients
Central nervous system
 Migraine/headache 20% 25% Controversial association with aPLs because of the high prevalence in the general population
 Epilepsy 6-7% 14% In many but not all cases secondary to ischemic events
Conflicting data on relationship between aPLs and seizure in SLE
 MS-like disease    No definite data regarding prevalence because of the difficult differential diagnosis
 Cognitive impairment 38% 48% Mostly involving attention and verbal fluency
 Dementia 2.5-56% Resulting from chronic or recurrent ischemic events
 Ocular manifestations 15-88% Amaurosis fugax as potential first sign of cerebral ischemia
Retinal artery thrombosis vessels (arteries and veins) are relatively frequent and can lead to significant visual loss
 Transverse myelopathy 1% Strong correlation with aPLs in SLE patients
 Pulmonary alveolar hemorrhage <1% Very poor prognosis
  1. aData from small series, with hypertension or signs suggestive of nephropathy. aPL, anti-phospholipid antibody; APS, anti-phospholipid syndrome; MS, multiple sclerosis; PAPS, primary anti-phospholipid syndrome; SLE, systemic lupus erythematosus.