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

 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

Skin

 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

Kidney

 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