Incidence, pathophysiology, and clinical manifestations of antiphospholipid syndrome

Abstract

Antiphospholipid syndrome (APLS) is a complex systemic disease with a wide variety of clinical manifestations. In the obstetric population, recurrent early pregnancy loss, fetal loss, and thrombosis are hallmarks of the disease. Patients with APLS have developed one or more pathogenic auto-antibodies directed against plasma and cell surface proteins. These antibodies are characterized by their affinity for anionic phospholipids. Interactions between APLS antibodies and their protein targets influence a wide variety of biological systems and signaling pathways, including monocytes, platelets, the complement system, and endothelial cells. While much research is currently directed at understanding the mechanisms involved in this autoimmune disease, the key clinical presentation is the hypercoagulable state resulting in thrombosis occurring in essentially any arterial or venous location, as well as numerous obstetrical complications. Treatment of APLS is generally directed at preventing thrombosis and poor pregnancy outcomes by ameliorating the hypercoagulable state.

Proteins Involved in the Induction of Procoagulant Activity and Autoimmune Response in Patients With Primary Antiphospholipid Syndrome

“…The observed aPL profile (prevalence of aCL, IgG, and 7% triple positivity) was also compatible with the literature, according to which aCL is the most common antibody found in patients with APS. 4,6,[11][12][13] The plasma protein identified with proteomic techniques may be useful in early diagnosis, investigation of pathogenic mechanisms, and monitoring of treatment response and disease progression. 13 However, so far, few proteomic analyses of patients with APS have been conducted, and only 1 study has evaluated the protein expression in monocytes of patients with APS.…”

Progesterone receptor expression in fibromuscular dysplasia: A report of two unusual cases

“…She had no history of thrombosis and/or loss of pregnancy, which are the hallmarks of antiphospholipid syndrome. [20] Lower extremity arterial obstructions are known to be mainly caused by intrinsic vascular factors such as atherosclerosis, cystic adventitial disease, fibromuscular dysplasia, Buerger's disease, vasculitis, and idiopathic midaortic syndrome. [21] The presented case did not show any histopathological feature which can be attributed to atherosclerosis, such as intimal thickening with lipid pools and smooth muscle cells or fibroatheroma plaque.…”

Presumed antiphospholipid syndrome and thrombotic thrombocytopenic purpura: An infrequent association

“…Antiphospholipid syndrome autoantibodies are present in 41% of women with SLE, although 1%‐5% of all women are found positive for these antibodies without associated symptoms or pathology . APS contributes to a significant percentage of repeated miscarriages, as the antibodies are found in more than 15% of women who experience recurrent pregnancy loss .…”

“…Antiphospholipid syndrome autoantibodies are present in 41% of women with SLE, although 1%‐5% of all women are found positive for these antibodies without associated symptoms or pathology . APS contributes to a significant percentage of repeated miscarriages, as the antibodies are found in more than 15% of women who experience recurrent pregnancy loss . In addition to the pregnancy/birthing complications that are part of the diagnostic criteria, APS patients are at risk for severe thrombotic events, including venous thromboembolism and ischemic stroke, even if time has passed and they no longer qualify for the diagnosis …”

“…3 APS contributes to a significant percentage of repeated miscarriages, as the antibodies are found in more than 15% of women who experience recurrent pregnancy loss. 3,4 In addition to the pregnancy/birthing complications that are part of the diagnostic criteria, APS patients are at risk for severe thrombotic events, including venous thromboembolism and ischemic stroke, even if time has passed and they no longer qualify for the diagnosis. 5,6 In a differential diagnosis of APS, the diagnosis and development of TTP becomes challenging (Table 1).…”