Cutaneous manifestations of antiphospholipid syndrome

Lupus ◽  
2021 ◽  
pp. 096120332199010
Author(s):  
Minerva Gomez-Flores ◽  
Genesis Herrera-Argaez ◽  
Osvaldo Vazquez-Martinez ◽  
Maira Herz-Ruelas ◽  
Jorge Ocampo-Candiani ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Binding Proteins ◽  
Cutaneous Manifestations ◽  
Phospholipid Binding

Antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which autoantibodies are produced against a variety of phospholipids and phospholipid-binding proteins. The purpose of this article is to review cutaneous findings in patients with APS diagnosis. An overview regarding prevalence, description, pathogenesis and histopathology, are described for cutaneous manifestations of APS.

The Antiphospholipid Syndrome

Hematology ◽  
2007 ◽  
Vol 2007 (1) ◽  
pp. 136-142 ◽  
Author(s):  
Jacob H. Rand
Keyword(s):  
Antiphospholipid Syndrome ◽  
Pregnancy Complications ◽  
Binding Proteins ◽  
Clinical Manifestations ◽  
Therapeutic Approaches ◽  
Vascular Thrombosis ◽  
Phospholipid Binding ◽  
Thrombophilic Condition

Abstract The antiphospholipid syndrome (APS) is an autoimmune thrombophilic condition that is marked by the presence of antibodies that recognize phospholipid-binding proteins. The clinical manifestations of APS include vascular thrombosis and pregnancy complications, especially recurrent spontaneous miscarriages. This article provides an update on diagnostic and therapeutic approaches to this disorder.

scholarly journals Pathophysiology of the Antiphospholipid Antibody Syndrome

2021 ◽  
Author(s):  
David Green
Keyword(s):  
Antiphospholipid Syndrome ◽  
Binding Proteins ◽  
Membrane Receptors ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Clotting Factors ◽  
Anionic Phospholipid ◽  
Autoantibody Production ◽  
Phospholipid Binding ◽  
Glycoprotein I

The antiphospholipid syndrome is characterized by antibodies directed against phospholipid-binding proteins and phospholipids attached to cell membrane receptors, mitochondria, oxidized lipoproteins, and activated complement components. When antibodies bind to these complex antigens, cells are activated and the coagulation and complement cascades are triggered, culminating in thrombotic events and pregnancy morbidity that further define the syndrome. The phospholipid-binding proteins most often involved are annexins II and V, β2-glycoprotein I, prothrombin, and cardiolipin. A distinguishing feature of the antiphospholipid syndrome is the “lupus anticoagulant”. This is not a single entity but rather a family of antibodies directed against complex antigens consisting of β2-glycoprotein I and/or prothrombin bound to an anionic phospholipid. Although these antibodies prolong in vitro clotting times by competing with clotting factors for phospholipid binding sites, they are not associated with clinical bleeding. Rather, they are thrombogenic because they augment thrombin production in vivo by concentrating prothrombin on phospholipid surfaces. Other antiphospholipid antibodies decrease the clot-inhibitory properties of the endothelium and enhance platelet adherence and aggregation. Some are atherogenic because they increase lipid peroxidation by reducing paraoxonase activity, and others impair fetal nutrition by diminishing placental antithrombotic and fibrinolytic activity. This plethora of destructive autoantibodies is currently managed with immunomodulatory agents, but new approaches to treatment might include vaccines against specific autoantigens, blocking the antibodies generated by exposure to cytoplasmic DNA, and selective targeting of aberrant B-cells to reduce or eliminate autoantibody production.

scholarly journals Antiphospholipid Syndrome and Pregnancy-Diagnosis, Complications and Management: An Overview

2021 ◽  
Author(s):  
Panagiotis Tsikouras ◽  
Christina Tsiggalou ◽  
Anastasia Bothou ◽  
Aggeliki Gerede ◽  
Ifigenia Apostolou ◽  
...  
Keyword(s):  
Venous Thrombosis ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Pregnancy Complications ◽  
Binding Proteins ◽  
Phospholipid Binding ◽  
Blood Clots ◽  
Human Proteins ◽  
Specific Proteins ◽  
Normal Human

Antiphospholipid syndrome which is also known as APS is an autoimmune disease which represents an acquired form of thrombophilia. The etiology of APS remains unknown. This disorder occurs when the immune system mistakenly attacks some of the normal human proteins and manifests itself as recurrent arterial or venous thrombosis and it could emerge after abortions or in recurrent pregnancy loss. In APS, the body produces the wrong antibodies against phospholipid-binding proteins, that is present in the blood and plays an important role in coagulation. Antibodies are specific proteins that usually target and neutralize the body’s invaders, such as viruses and bacteria. When antibodies attack phospholipid-binding proteins, blood clots abnormally. Specifically, it could cause blood clots in veins or arteries leading to stroke and various pregnancy complications such as: endometrial death, miscarriage, preeclampsia, intrauterine growth restriction and prematurity. APS is divided into primary and secondary, which is associated with autoimmune diseases and more often with systemic lupus erythematosus (SLE), while antibodies against cardiolipin are detected in many other conditions (infections, malignancies, drugs, etc.). The symptoms of APS, in addition to arterial and/or venous thrombosis and pregnancy complications, are multisystemic and the differential diagnosis of the primary APS from the secondary, in the context of SLE, is of particular clinical interest and is subject of this literature review.

scholarly journals A calcyclin-associated protein is a newly identified member of the Ca2+/phospholipid-binding proteins, annexin family.

1992 ◽  
Vol 267 (13) ◽  
pp. 8919-8924
Author(s):  
H Tokumitsu ◽  
A Mizutani ◽  
H Minami ◽  
R Kobayashi ◽  
H Hidaka
Keyword(s):  
Binding Proteins ◽  
Phospholipid Binding

Some Patients with Antiphospholipid Syndrome Express hitherto Undescribed Antibodies to Cardiolipin-binding Proteins

2001 ◽  
Vol 85 (01) ◽  
pp. 57-62 ◽  
Author(s):  
P. Rampazzo ◽  
A. Biasiolo ◽  
J. Garin ◽  
A. Rosato ◽  
C. Betterle ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Binding Proteins ◽  
Anticardiolipin Antibodies ◽  
Factor H ◽  
Complement Factor H ◽  
Macromolecular Complex ◽  
Complement Factor ◽  
Close Relationship

SummaryContrary to infective anticardiolipin (aCL) antibodies, autoimmune aCL antibodies react with phospholipids (PL) mainly via binding to the plasma glycoprotein cofactor β2-Glycoprotein I (β2GPI). While there is a well-documented link between the risk of thrombosis and the presence of β2GPI-dependent anticardiolipin antibodies, the pathological impact of other antiphospholipid antibodies is less clear. By means of cardiolipin affinity-chromatography, we isolated and identified 3 CL-binding proteins, complement component C4, complement factor H and a kallikrein-sensitive glycoprotein, and tested for the presence of autoantibodies against these proteins in patients with antiphospholipid syndrome (APS), systemic lupus erythematosus (SLE) and other autoimmune diseases. High titers of autoantibodies to C4 as compared to age- and sex-matched healthy controls were present in 3 of 26 patients with APS, and weak titers were found in 2 of 26 patients with SLE and in none of 26 patients with other autoimmune diseases. Autoantibodies to complement factor H were found in 4 APS, 3 SLE and none of the other autoimmune patients. Autoantibodies to kallikrein-sensitive glycoprotein were detected in 6 APS patients, 1 SLE patient, and 1 patient with another autoimmune disease. A close relationship between these antibodies was found, suggesting their origin from a common macromolecular complex. However, no relationship with anti-β2GPI antibodies was found, with the three patients with higher levels of autoantibodies having a low titer of anti-β2GPI antibodies. In conclusion, some patients with APS harbor circulating antibodies to other CL-binding proteins which might be useful to further characterize these patients.

Pregnancy loss and autoantibodies against phospholipid-binding proteins

1997 ◽  
Vol 89 (6) ◽  
pp. 975-980 ◽  
Author(s):  
C FALCON ◽  
M MARTINUZZO ◽  
R FORASTIERO ◽  
G CERRATO ◽  
L CARRERAS
Keyword(s):  
Pregnancy Loss ◽  
Binding Proteins ◽  
Phospholipid Binding

Annexin 1 and neutrophil apoptosis

2004 ◽  
Vol 32 (3) ◽  
pp. 507-510 ◽  
Author(s):  
M. Perretti ◽  
E. Solito
Keyword(s):  
Inflammatory Response ◽  
Binding Proteins ◽  
Neutrophil Apoptosis ◽  
The Novel ◽  
Inhibitory Effects ◽  
Phospholipid Binding ◽  
Annexin 1 ◽  
Anti Inflammatory ◽  
Leucocyte Recruitment ◽  
Host Inflammatory Response

ANXA1 (annexin 1), a member of the ‘annexin’ family of calcium- and phospholipid-binding proteins, was originally identified as an endogenous mediator of the anti-inflammatory actions of glucocorticoids. However, this protein exerts multiple inhibitory effects on the host inflammatory response, including a preferential regulation of the adhesion step of blood-borne neutrophil within the microenvironment of an inflamed vasculature. It is now emerging that ANXA1 is endowed with other roles, since the protein is abundant in inflammatory exudates as it is produced and released by the extravasated neutrophil. In the present paper, we review the novel proapoptotic effect of ANXA1 and discuss its potential with respect to the pathophysiology of inflammation and leucocyte recruitment.

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