The ‘Primary’ Antiphospholipid Syndrome: Antiphospholipid Antibody Pattern and Clinical Features of a Series of 23 Patients

Autoimmunity ◽  
1991 ◽  
Vol 9 (1) ◽  
pp. 69-75 ◽  
Author(s):  
Josep Font ◽  
Alfons Loapez-Soto ◽  
Ricard Cervera ◽  
Joan Balasch ◽  
Lucio Pallarés ◽  
...  
Keyword(s):  
Clinical Features ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibody ◽  
Primary Antiphospholipid Syndrome ◽  
Antibody Pattern

scholarly journals Case of an unusual diagnosis of primary antiphospholipid syndrome with multiple clinical complications

2020 ◽  
Vol 2020 (12) ◽  
Author(s):  
Stathis Tsiakas ◽  
Chrysanthi Skalioti ◽  
Paraskevi Kotsi ◽  
Ioannis Boletis ◽  
Smaragdi Marinaki
Keyword(s):  
Antiphospholipid Syndrome ◽  
Thrombotic Event ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Young Male ◽  
Multiple Organ ◽  
Systemic Autoimmune Disease ◽  
Antiphospholipid Antibody ◽  
Primary Antiphospholipid Syndrome ◽  
Wide Range

ABSTRACT Antiphospholipid syndrome (APS) is a systemic autoimmune disease defined by the presence of antiphospholipid antibodies in association with thrombotic events and/or obstetric complications. Renal involvement is not infrequent in both primary and secondary APS. Kidney manifestations comprise a wide range of clinical features, including hypertension, major renal vessel thrombosis or microvascular endothelial injury, also described as APS nephropathy. In the absence of a thrombotic event, clinical manifestations of APS are often non-specific. We recently encountered a case of primary APS in a young male with newly diagnosed hypertension and renal impairment. The diagnosis of APS was initially suspected by his kidney biopsy findings, when electron microscopy examination showed the features of chronic microangiopathy, and was later confirmed by a triple positive antiphospholipid antibody profile and multiple organ involvement.

Antiphospholipid antibody profile based obstetric outcomes of primary antiphospholipid syndrome: the PREGNANTS study

2017 ◽  
Vol 216 (5) ◽  
pp. 525.e1-525.e12 ◽  
Author(s):  
Gabriele Saccone ◽  
Vincenzo Berghella ◽  
Giuseppe Maria Maruotti ◽  
Tullio Ghi ◽  
Giuseppe Rizzo ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Obstetric Outcomes ◽  
Antiphospholipid Antibody ◽  
Primary Antiphospholipid Syndrome ◽  
Antibody Profile

The role of antiphospholipid antibodies in reproductive failure

1997 ◽  
Vol 6 (3) ◽  
pp. 133-143
Author(s):  
D Ware Branch ◽  
Harry H Hatasaka
Keyword(s):  
Pregnancy Outcome ◽  
Clinical Features ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Fetal Loss ◽  
Placental Insufficiency ◽  
Antiphospholipid Antibody ◽  
The Relationship

The relationship between antiphospholipid antibodies and the clinical features of placental insufficiency, pre-eclampsia, and fetal loss has emerged as one of the most exciting new observations in obstetrics in the last 15 years. Antiphospholipid syndrome is the only convincing ‘immunologic’ disturbance of pregnancy affecting the fetus other than anti-erythrocyte or antiplatelet alloimmunization disorders, and it is now routine to test patients with fetal loss for the two best characterized antiphospholipid antibodies, lupus anticoagulant and anticardiolipin. Although there is no proven mechanism for fetal loss, treatment of antiphospholipid antibody-positive mothers during pregnancy with heparin improves pregnancy outcome.

scholarly journals Primary antiphospholipid syndrome in a hemodialysis patient with recurrent thrombosis of arteriovenous fistulas

2020 ◽  
Vol 42 (2) ◽  
pp. 259-263
Author(s):  
Nikola Gjorgjievski ◽  
Pavlina Dzekova-Vidimliski
Keyword(s):  
Antiphospholipid Syndrome ◽  
Vascular Access ◽  
Lupus Anticoagulant ◽  
Hemodialysis Patient ◽  
Systemic Autoimmune Disease ◽  
Antiphospholipid Antibody ◽  
Primary Antiphospholipid Syndrome ◽  
Case Presentation ◽  
Arteriovenous Fistulas ◽  
Thrombophilia Screening

ABSTRACT Introduction: The antiphospholipid syndrome is a systemic autoimmune disease defined by recurrent vascular and/or obstetrical morbidity that occurs in patients with persistent antiphospholipid antibodies. Case presentation: A patient on hemodialysis with a primary antiphospholipid syndrome presented with recurrent vascular access thrombosis, obstetrical complications, and positive lupus anticoagulant. The patient had multiple arteriovenous fistulas that failed due to thrombosis. The obstetrical morbidity was defined by one miscarriage at the 7th week of gestation and a pregnancy complicated by pre-eclampsia with preterm delivery at the 28th week of gestation. A thorough thrombophilia screening confirmed the presence of antiphospholipid antibody. Lupus anticoagulant was present in plasma, measured on two occasions 12 weeks apart. Conclusion: Thrombophilias are inherited or acquired predispositions to vascular thrombosis and have been associated with thrombosis of the arteriovenous fistula. Patients on hemodialysis with recurrent vascular access thrombosis and presence of thrombophilia should be evaluated about the need for anticoagulant therapy with a vitamin K antagonist.

Antiphosphatidylserine/prothrombin antibodies as biomarkers to identify severe primary antiphospholipid syndrome

2017 ◽  
Vol 55 (6) ◽  
pp. 890-898 ◽  
Author(s):  
Ariela Hoxha ◽  
Elena Mattia ◽  
Marta Tonello ◽  
Chiara Grava ◽  
Vittorio Pengo ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Clinical Laboratory ◽  
Antiphospholipid Antibody ◽  
Primary Antiphospholipid Syndrome ◽  
Serum Samples ◽  
Elisa Kit ◽  
Pregnancy Morbidity ◽  
Life Threatening ◽  
The Relationship

Abstract Background: Anti-phosphatidylserine/prothrombin (aPS/PT) antibodies have begun to be considered potentional biomarkers for antiphospholipid syndrome (APS). This cohort study investigate the role of aPS/PT antibodies as a risk factor for severe APS by evaluating the association between those antibodies and clinical/laboratory profiles of APS. Methods: Plasma/serum samples from 197 APS patients, 100 healthy subjects and 106 patients with autoimmune diseases were collected. IgG/IgM aPS/PT antibodies were assayed using commercial ELISA kit. Results: Prevalences of IgG and IgM aPS/PT (p<0.0001 and p=0.0009, respectively) and their titres (p<0.0001 and p=0.0002, respectively) were significantly higher in thrombosis/pregnancy group with respect to pregnancy morbidity alone. Prevalences of IgG and IgM aPS/PT (p<0.0001 and p=0.0004, respectively) and their mean levels (p=0.0001 for both) were significantly higher in the prematurity linked to life-threatening obstetric complications group with respect to miscarriage group. There was a significant relationship between IgG and IgM aPS/PT (p=0.001 and p=0.0002) and their mean levels were higher (p=0.0004 and p=0.0002, respectively) in the thrombotic microangiopathy group, considered a milestone manifestation of catastrophic APS. The relationship between IgG and IgM aPS/PT was significant and mean levels were higher in triple positive antiphospholipid antibody patients than in double and single positivity ones (p<0.0001 for all). Conclusions: APS/PT antibodies were associated to severe thrombosis, severe pregnancy complications inducing prematurity, and vascular microangiopathy, all generally associated to high risk APS forms requiring strong therapy.

scholarly journals Primary antiphospholipid syndrome in children: experience from two tertiary centres in South India

2019 ◽  
Vol 6 (2) ◽  
pp. 243
Author(s):  
Mahesh Janarthanan ◽  
Dhaarani Jayaraman ◽  
Julius Scott ◽  
M. S. Latha ◽  
Saravanan Margabandhu ◽  
...  
Keyword(s):  
Clinical Features ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Clinical Manifestations ◽  
Fetal Loss ◽  
Primary Antiphospholipid Syndrome ◽  
Retrospective Case ◽  
Case Record ◽  
Vascular Thrombosis ◽  
Immunologic Profile

Background: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by the presence of episodes of vascular thrombosis, recurrent fetal loss and other clinical features in the presence of antiphospholipid antibodies. The aim of the study was to analyze the clinical manifestations and immunologic profile of children presenting with APS.Methods: Authors did a retrospective case record study of patients admitted with thrombotic events between September 2013 and August 2018 and identified patients with positive antiphospholipid antibodies. Children who had clinical features of active lupus were not included.Results: The clinical and immunologic profile of 7 pediatric patients presenting with APS over 5 years from 2013 to 2018 were analysed. Symptoms secondary to vascular thrombosis were limb swelling, stroke, gangrene of toes and Budd Chiari syndrome.Conclusions:APS though rare should be considered in the differential diagnosis of children presenting with thrombotic events. They need long term anticoagulants to prevent further episodes. 

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