The effect of hydroxychloroquine on thrombosis prevention and antiphospholipid antibody levels in primary antiphospholipid syndrome: A pilot open label randomized prospective study

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.

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

American Journal of Obstetrics and Gynecology ◽

10.1016/j.ajog.2017.01.026 ◽

2017 ◽

Vol 216 (5) ◽

pp. 525.e1-525.e12 ◽

Cited By ~ 58

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

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

Autoimmunity ◽

10.3109/08916939108997126 ◽

1991 ◽

Vol 9 (1) ◽

pp. 69-75 ◽

Cited By ~ 65

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

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Intensity of immune/clotting assays relate to multiple antiphospholipid antibody positivity in thrombotic primary antiphospholipid syndrome

International Journal of Hematology ◽

10.1007/s12185-020-03009-2 ◽

2020 ◽

Author(s):

Paul R. J. Ames ◽

Mira Merashli ◽

Bucci Tommaso ◽

Luigi Iannaccone ◽

Vincenzo Marottoli ◽

...

Keyword(s):

Antiphospholipid Syndrome ◽

Antiphospholipid Antibody ◽

Primary Antiphospholipid Syndrome ◽

Antibody Positivity

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Independent association of low IFNλ1 gene expression and type I IFN score/IFNλ1 ratio with obstetric manifestations and triple antiphospholipid antibody positivity in primary antiphospholipid syndrome

Clinical Immunology ◽

10.1016/j.clim.2019.108265 ◽

2019 ◽

Vol 209 ◽

pp. 108265 ◽

Cited By ~ 3

Author(s):

Christina-Maria Flessa ◽

Stelios Vlachiotis ◽

Adrianos Nezos ◽

Evangelos Andreakos ◽

Clio P. Mavragani ◽

...

Keyword(s):

Gene Expression ◽

Antiphospholipid Syndrome ◽

Antiphospholipid Antibody ◽

Type I ◽

Primary Antiphospholipid Syndrome ◽

Type I Ifn ◽

Antibody Positivity ◽

Independent Association

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Prospective study of perinatal outcome in pregnancies with primary antiphospholipid syndrome

Vojnosanitetski pregled ◽

10.2298/vsp1408742c ◽

2014 ◽

Vol 71 (8) ◽

pp. 742-745 ◽

Cited By ~ 1

Author(s):

Aleksandar Cetkovic ◽

Biljana Kastratovic ◽

Ivana Novakovic

Keyword(s):

Birth Weight ◽

Prospective Study ◽

Antiphospholipid Syndrome ◽

Perinatal Outcome ◽

Perinatal Outcomes ◽

Primary Antiphospholipid Syndrome ◽

Mortality And Morbidity ◽

Neonatal Complications ◽

Live Births ◽

Gestational Age At Delivery

Background/Aim. Pregnancies complicated with antiphospholipd syndrome are associated with the increased perinatal mortality and morbidity. The aim of this study was to assess perinatal outcome in pregnancies with primary antiphospholipd syndrome. Methods. This prospective study evaluated perinatal outcome in 25 pregnant women with antiphospholipid syndrome. After establishing vital pregnancy all the patients were treated with low-molecularweight heparin and aspirin. The perinatal outcome was measured by rates of miscarriages, preterm deliveries, live births and neonatal complications. Results. Of the 25 pregnancies, 20 (80%) resulted in live birth, 3 (12%) in spontaneous abortion and 2 (8%) were stillbirths. The mean gestational age at delivery was 37.2 ? 1.0 weeks, mean neonatal birth weight was 2,930.4 ? 428.0 g. Prematurity occurs in 4 (20%) live births, and there were 4 (20%) intrauterine growth restriction with mean birth weight 2,060 ? 210.6 g. Neonatal complications were present in 6 (30%) newborns. Adverse perinatal outcome was significantly associated with anticardiolipin IgG antibodies (p < 0.01) and development of hypertension during pregnancy (p < 0.01). Conclusion. Despite a high incidence of adverse perinatal outcomes in pregnancies with primary antiphospholipid syndrome, early treatment with aspirin and low-molecular-weight heparin, combined with meticulous fetomaternal monitoring could be associated with a relatively high probability of favorable perinatal outcome.

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Loss of antiphospholipid antibody positivity post-thrombosis in SLE

Lupus Science & Medicine ◽

10.1136/lupus-2020-000423 ◽

2020 ◽

Vol 7 (1) ◽

pp. e000423

Author(s):

Muznay Khawaja ◽

Laurence Magder ◽

Daniel Goldman ◽

Michelle A Petri

Keyword(s):

Clinical Practice ◽

Prospective Study ◽

Antiphospholipid Syndrome ◽

Antiphospholipid Antibodies ◽

Lupus Anticoagulant ◽

Complete Loss ◽

Antiphospholipid Antibody ◽

Confirmatory Tests ◽

Antibody Positivity

Background/PurposeLoss of positivity of antiphospholipid antibodies has been observed in clinical practice post-thrombosis in patients with SLE with secondary antiphospholipid syndrome (APS). Our study defined the frequency of this loss and the duration before positivity recurred.MethodsIn this prospective study, patients with SLE having at least two positive antiphospholipid markers prior to thrombosis and at least 1 year of follow-up after thrombosis were included. Antiphospholipid markers included lupus anticoagulant (dilute Russell viper venom test >45 s followed by mixing and confirmatory tests) and/or anticardiolipin titre (aCL IgG ≥20, aCL IgM ≥20 and/or aCL IgA ≥20). The percentage of visits with positive antiphospholipid markers after thrombosis was calculated. For patients with a negative antiphospholipid marker any time after thrombosis, survival estimates were performed to calculate the time to return of antiphospholipid positivity.ResultsIn APS due to SLE, complete loss of antiphospholipid positivity post-thrombosis was up to 41% for aCL IgG, 51% for IgM and 50% for IgA, but only 20% for those with lupus anticoagulant. Of those who at some point lost aCL IgG or became negative for lupus anticoagulant, the majority (60% and 76%, respectively) reacquired the antibody within 5 years. In contrast, of those who lost aCL IgM or IgA, fewer reacquired it within 5 years (37% and 17%, respectively).ConclusionIntermittent positivity of antiphospholipid antibodies is present in APS due to SLE. These fluctuations make it difficult to decide on length of anticoagulation. Lupus anticoagulant is more likely to persist post-thrombosis.

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