Research On Non-criteria Anti-phospholipid Antibodies And Clinical Manifestations Of The Anti-phospholipid Syndrome

Antiphospholipid Syndrome(APS)is a set of autoimmune disease induced by antiphospholipid antibodies(aPLs),it is characterized by recurrent arterial/venous thrombosis and pregnancy loss.According to the 2006 Sydney classification criteria,which is most widely used nowadays,a patients can be diagnosed as APS only if he/she satisfies both a clinical criteria(vascular thrombosis or pregnancy morbidity)and a laboratory criteria(including the positivity of lupus anticoagulant,anti-cardiolipin or anti β2-glycoprotein Ⅰ)simultaneously.But as a systemic autoimmune disease,there are still a set of extra-criteria manifestations of APS that cannot be completely explained by the pre-thrombotic status,including thrombocytopenia,valvular heart disease,livedo reticularis,nephropathy,skin ulcer and so on.These clinical manifestations are positively correlated to the presence of aPLs and thrombotic/obstetric clinical events.Fully recognizing these extra-criteria clinical manifestations that happen to APS patients commonly may help to distinguish patients with elevated risk for thrombosis or pregnancy morbidities,and may influence the therapeutic decisions of the physicians and eliminate the occurrence of adverse events of these potential APS patients,thus improving the prognosis.On the other hand,patients exist with clinical manifestations strongly suggest APS but with persistent negative or transient positive aPL,however as the classical APS patients,these patients may also experience recurrent thrombosis and PM,or even catastrophic antiphospholipid syndrome(CAPS).These patients are categorized into seronegative antiphospholipid syndrome(SNAPS)patients.Thus recognizing them and exerting risk management is an important challenge in clinical practice.Notably,with the development to base research upon the pathogenesis of APS,more and more studies have demonstrated the correlation between different antigens targeting different antigens(including various proteins involved in the coagulation cascade and the anti coagulation cascade,cytoskeletal protein,platelet related factors,etc)and thrombotic/obstetric events.Presence of these non-criterial aPLs can help us to distinguish the SNAPS patients,thus bringing chance for efficacious intervention and prophylactic treatment.Hence,this article reviewed the progression on the extra-criteria manifestation and the non-criteria aPLs of APS.Meanwhile,we chose 282 APS serum samples from the APS dataset established by this research group in Shanghai,retested the aPL profiles and performed the analysis upon the diagnosis value and the odds ratio of the adverse events of the single and combined antibodies disease controls and healthy controls were included in this process,demonstrating that non-criterial antiphospholipid antibodies can provide additional value for the diagnosis and evaluation of APS.In addition,this article also analyzed the clinical characteristics of 14 CAPS patients in the dataset,which may help to provide experience for the diagnosis and treatment of CAPS.