scholarly journals Longterm Outcome of Patients with Primary Antiphospholipid Syndrome: A Retrospective Multicenter Study

2017 ◽  
Vol 44 (8) ◽  
pp. 1165-1172 ◽  
Author(s):  
Mara Taraborelli ◽  
Rossella Reggia ◽  
Francesca Dall’Ara ◽  
Micaela Fredi ◽  
Laura Andreoli ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Oral Anticoagulants ◽  
Thrombotic Event ◽  
Organ Damage ◽  
Solid Cancer ◽  
Primary Antiphospholipid Syndrome ◽  
Severe Infections ◽  
Bleeding Episodes ◽  
Longterm Outcome ◽  
Retrospective Multicenter Study

Objective.To assess the longterm frequency of thrombotic recurrences, obstetrical complications, organ damage, severe comorbidities, and evolution toward connective tissue disease (CTD) in primary antiphospholipid syndrome (PAPS).Methods.Medical records of patients with PAPS followed in 6 centers for ≥ 15 years were retrospectively reviewed.Results.One hundred fifteen patients were studied: 88% women, followed between 1983 and 2014 with a mean (± SD) age at diagnosis of 33 (± 10) years. During a median followup of 18 years (range 15–30), 50 patients (44%) had at least a thrombotic event for a total of 75 events and an annual incidence of 3.5%. Thromboses were more frequent in patients with previous thrombotic history (p = 0.002). A catastrophic antiphospholipid syndrome occurred in 6 patients (5%). The use of oral anticoagulants in patients with thrombotic onset did not appear to be protective against recurrences (p = 0.26). Fifty-two women had 87 pregnancies, successful in 78%. Twenty-nine percent of patients accrued functional damage. Damage was significantly associated with a thrombotic history (p = 0.004) and with arterial events (p < 0.001), especially stroke, but not with demographics, serology, or treatment. Twenty-four major bleeding episodes were recorded in 18 patients, all receiving anticoagulants. Severe infections affected 6 patients (5%), with 1 fatality. A solid cancer was diagnosed in 8 patients (7%). Altogether, 16 patients (14%) developed an autoimmune disease and 13 (11%) a full-blown picture of CTD.Conclusion.Despite therapy, a high proportion of patients experienced new thrombotic events and organ damage, while evolution toward CTD was infrequent.

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.

Patients with longstanding primary antiphospholipid syndrome: retrospective analysis of organ damage and mortality

Lupus ◽  
2014 ◽  
Vol 23 (12) ◽  
pp. 1255-1258 ◽  
Author(s):  
F Dall’Ara ◽  
R Reggia ◽  
M Taraborelli ◽  
L Andreoli ◽  
M Taglietti ◽  
...  
Keyword(s):  
Retrospective Analysis ◽  
Antiphospholipid Syndrome ◽  
Organ Damage ◽  
Primary Antiphospholipid Syndrome

Antiphospholipid-associated thrombocytopenia or autoimmune hemolytic anemia in patients with or without definite primary antiphospholipid syndrome according to the Sapporo revised classification criteria: a 6-year follow-up study

Blood ◽  
2010 ◽  
Vol 116 (16) ◽  
pp. 3058-3063 ◽  
Author(s):  
Lucía Comellas-Kirkerup ◽  
Gabriela Hernández-Molina ◽  
Antonio R. Cabral
Keyword(s):  
Hemolytic Anemia ◽  
Antiphospholipid Syndrome ◽  
Autoimmune Hemolytic Anemia ◽  
Lupus Anticoagulant ◽  
Thrombotic Event ◽  
Classification Criteria ◽  
Response To Treatment ◽  
Primary Antiphospholipid Syndrome ◽  
Pregnancy Morbidity

Abstract The updated Sapporo classification criteria for antiphospholipid syndrome (APS) only include thrombosis or pregnancy morbidity as clinical criteria. To test this notion, we studied 55 patients (80% women) with hematologic manifestations. All fulfilled the laboratory criteria for primary APS. Thirty-five patients (64%) had thrombocytopenia, 14 (25%) had autoimmune hemolytic anemia, and 6 (11%) had both. Twenty-five patients (22 women, 88%) also fulfilled one clinical criterion for APS after a median follow-up of 13.2 years (range, 1.45-37 years), whereas the remaining 30 patients (22 women, 73%) have not had any thrombotic event nor pregnancy morbidity after a median follow-up of 5.4 years (range, 0.12-24 years). No patient developed systemic lupus erythematosus during follow-up. The hematologic manifestation was asynchronous with the APS onset in 84% of patients. The response to treatment was similar regardless of the APS status. Patients with definite APS were more frequently positive for the lupus anticoagulant (63%) than lupus anticoagulant-positive patients without APS (30%; odds ratio, 3.5; 95% confidence interval, 1.07-11.4; P < .02). Anticardiolipin or anti–β2-glycoprotein-I antibodies were highly prevalent among the study groups. Our study suggests that, depending upon their antiphospholipid profile, patients with hemocytopenias appear to comprise a peculiar subset of patients with APS; some develop thrombotic and/or obstetric APS whereas others continue with hematologic APS.

scholarly journals P168 Non-bacterial endocarditis in patients with antiphospholipid syndrome treated with direct oral anticoagulants

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
I Munoz Pousa ◽  
F E Calvo Iglesias ◽  
M Cespon Fernandez ◽  
E Lopez Rodriguez ◽  
G Pradas Montilla ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Vitamin K Antagonists ◽  
Oral Anticoagulants ◽  
Direct Oral Anticoagulants ◽  
High Dose ◽  
Primary Antiphospholipid Syndrome ◽  
Poor Control ◽  
Mechanical Prosthesis ◽  
Coronary Syndrome ◽  
Dose Warfarin

Abstract Introduction Primary antiphospholipid syndrome (APS) is a hypercoagulability state of autoimmune origin. Vitamin K- antagonists remain the mainstay therapy though the difficulties in maintaining target therapeutic range contributed to prescriptions of direct oral anticoagulants (ACOD). A recent randomized trial reported an excess in thromboembolic events in patients under rivaroxaban therapy compared with warfarin. Purpose Description of two cases of Libman-Sacks endocarditis in APS patients on therapy with rivaroxaban due to poor control with coumadin. Methods and results. Case 1 is a 47-year-old woman and case 2 a 69-yo man with APS with high antibody titers of 3 classes and previous thrombotic events (pulmonary embolism in case 1 and acute coronary syndrome in case 2). In both cases, coumadin was switched to rivaroxaban because of poor control. During follow-up the diagnosis of blood culture-negative endocarditis was stablished in both cases. Echo examinations in case 1 revealed a 12 mm mobile vegetation in the ventricular face of the non-coronary aortic cusp, which resolved after 2 months high-dose warfarin therapy. Case 2 was admitted to hospital because of heart failure. TTE and TOE revealed a 26 mm mass attached to the atrial face of posterior mitral leaflet and moderate mitral regurgitation. He underwent mitral valve replacement with a mechanical prosthesis and the final pathologic diagnosis was Libman-Sacks endocarditis. Conclusions Libman-Sacks endocarditis as a thrombotic feature of high-risk APS patients can occur under rivaroxaban therapy. In one of our 2 cases, high-dose coumadin therapy resolved this complication Abstract P168 Figure. Cases

Damage index for antiphospholipid syndrome during long term follow-up: Correlation between organ damage accrual and quality of life

Lupus ◽  
2020 ◽  
Vol 30 (1) ◽  
pp. 96-102
Author(s):  
Gabriela Medina ◽  
Erik Antonio Cimé Aké ◽  
Olga Vera-Lastra ◽  
Miguel Ángel Saavedra ◽  
María del Pilar Cruz-Domínguez ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Antiphospholipid Syndrome ◽  
Damage Index ◽  
Organ Damage ◽  
Second Phase ◽  
Primary Antiphospholipid Syndrome ◽  
Sf 36 ◽  
Damage Accrual

Background Consequences of organ damage in primary antiphospholipid syndrome (PAPS) are diverse, our aim was to determine organ damage over time and the correlation of organ damage accrual with health-related quality of life (HRQoL) in PAPS. Methods First phase: retrospective cohort applying Damage Index for Antiphospholipid Syndrome (DIAPS) at 1, 5, 10, 20 years, or longer since diagnosis. Second phase: cross-sectional study, assessing HRQoL by the Medical Outcomes Study Short Form 36 (SF-36), and organ damage accrual. Descriptive statistics and Spearman correlation coefficient were used. Results Sixty-seven patients were included, mean follow-up:15 years. Deep vein thrombosis prevailed (71.6%), pulmonary embolism (35.8%) and stroke (32.8%). Organ damage was found in 98.5%, with a cumulative DIAPS value of 3, with greater involvement in the neuropsychiatric and peripheral vascular domains. Regarding HRQoL, deterioration in the physical component summary (PCS) was found in 89.6%. Organ damage accrual correlated inversely and significantly with all the SF-36 domains, mainly with the total score and PCS. Body pain and PCS correlated the most (rho = −0.503, rho = −0.475). Conclusions Organ damage accrual impaired HRQoL in PAPS. Secondary thromboprophylxis through adequate systemic management and control of cardiovascular risk factors are necessary to prevent further impairment.

scholarly journals Clinical diagnosis in patients with positive antiphospholipid antibodies

2020 ◽  
Vol 105 (105(810)) ◽  
pp. 90-95
Author(s):  
P. Herreros Fernández-Arroyo ◽  
J. M. Urra-Ardanaz
Keyword(s):  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Thrombotic Event ◽  
Primary Antiphospholipid Syndrome ◽  
Clinical Environment ◽  
Systemic Lupus ◽  
Cross Sectional ◽  
Igm Antibodies ◽  
Coagulation Tests

Objective: To know the relationship between the presence of three antiphospholipid antibodies: lupus anticoagulant and the anticardiolipin of isotypes IgM and IgG with the development of thrombotic events and alterations in coagulation and also, study the clinical environment in which those antibodies appear. Material and methods: Cross-sectional descriptive study in which we have analyzed retrospectively, in 123 patients with positive results for at least one of the antiphospholipid antibodies under study, their alterations in coagulation, if they suffer or have suffered any thrombotic event and the clinical environment in which these antibodies appear and. Results: 52,1% of patients with positive lupus anticoagulant have some type of abnormality in coagulation tests, compared with 43,75% of patients with anticardiolipin of isotype IgG and 24,64% of patients with anticardiolipin of isotypes IgM. The most frequent antibody in patients with primary antiphospholipid syndrome is anticardiolipin of isotypes IgM, which appears in 75%, while in the case of patients with secondary antiphospholipid syndrome due to erythematosus systemic lupus, the most frequent antibody is anticardiolipin of isotypes IgG, which is detected in 46,7%. Among the patients who suffered thrombotic event, in 45,94% anticardiolipin of isotypes IgM was detected, compared with 43,24% with lupus anticoagulant, and only 16,22% with anticardiolipin of isotype IgG. Conclusions: The antiphospholipid antibodies that alters coagulation tests to a greater extent is the lupus anticoagulant. Anticardiolipin of isotype IgM antibodies are the most frequent in primary antiphospholipid syndrome while anticardiolipin of isotype IgG are associated in a greater degree with secondary antiphospholipid syndrome, especially in patients with erythematosus systemic lupus. Anticardiolipin of isotype IgM antibodies represent a higher risk of thrombotic events in patients with positive antiphospholipid antibodies.

scholarly journals Immunogenicity, Safety and Antiphospholipid Antibodies After SARS-CoV-2 Vaccine in Patients With Primary Antiphospholipid Syndrome

2021 ◽  
Author(s):  
Flavio Signorelli ◽  
Gustavo Guimarães Moreira Balbi ◽  
Nadia Emi Aikawa ◽  
Clovis Artur Almeida Silva ◽  
Léonard de Vinci Kanda Kupa ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Neutralizing Antibodies ◽  
Thrombotic Event ◽  
Control Group ◽  
Blood Collection ◽  
Primary Antiphospholipid Syndrome ◽  
Increased Risk ◽  
Excellent Safety Profile ◽  
Induced Changes

Abstract Background: Coronavirus disease 19 (COVID-19) has an increased risk of coagulopathy with high frequency of antiphospholipid antibodies (aPL). The recent reports of thrombosis associated with the adenovirus-based vaccines raises concern that SARS-CoV-2 immunization in primary antiphospholipid syndrome (PAPS) patients may trigger a dysregulated immune response with possible clotting complications. Therefore, the objectives of this study were to assess immunogenicity, aPL production and safety of Sinovac-Coronavac in PAPS patients.Methods: This prospective controlled phase 4 study of SARS-CoV-2-naïve PAPS patients and a control group (CG) consisted of a two-dose Sinovac-CoronaVac (D0/D28) and blood collection before vaccination (D0), at D28 and 6 weeks after second dose (D69) for immunogenicity/aPL levels. Outcomes were seroconversion (SC) rates of anti-SARS-CoV-2 S1/S2 IgG and/or neutralizing antibodies (NAb) at D28/D69. Safety and aPL production were also assessed. Results: Forty-four PAPS patients and 132 CG had comparable age (p=0.982) and sex (p>0.999). At D69, both groups had high and comparable SC (83.9% vs. 93.5%, p=0.092) and GMT [50.2(95%CI 34.5-73.2) vs. 61.7 (95%CI 52.8-72.3), p=0.249] as well as NAb positivity (77.4% vs. 78.7%, p=0.440), and NAb-activity [64.3% (49.0-77.0) vs. 60.9% (45.6-81.3), p=0.689]. Of note, for D28, the antibody response was very low and also similar in both groups SC (25.8% vs. 30.6% p=0.609). Antiphospholipid levels remained stable throughout the study at D0 vs D28 vs D69 (IgG aCL, p=0.058; IgM aCL, p=0.091; IgG aβ2GPI, p=0.513 and IgM aβ2GPI, p=0.468). Thrombotic event up to 6 months or other moderate/severe side effects were not observed.Conclusions: We provide novel evidence that Sinovac-CoronaVac vaccine has a high immunogenicity and excellent safety profile in PAPS. We further demonstrated that this vaccine did not trigger thrombosis or induced changes in aPL-related antibodies production. Our findings strongly support the recommendation of SARS-CoV-2 vaccination for PAPS patients.Trial registration: ClinicalTrials.gov - Identifier: NCT04754698 first registered on February 8th, 2021.

Catastrophic antiphospholipid syndrome following the introduction of rivaroxaban

Lupus ◽  
2020 ◽  
Vol 29 (7) ◽  
pp. 787-790 ◽  
Author(s):  
Romain Stammler ◽  
Paul Legendre ◽  
Patrice Cacoub ◽  
Philippe Blanche ◽  
Jean -Charles Piette ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Vitamin K ◽  
Antiphospholipid Antibodies ◽  
Multiorgan Failure ◽  
Vitamin K Antagonists ◽  
Oral Anticoagulants ◽  
Direct Oral Anticoagulants ◽  
Thrombotic Event ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Thrombotic Complications

Summary Catastrophic antiphospholipid syndrome is the most severe complication of antiphospholipid syndrome. Vitamin K antagonists are the reference treatment for preventing relapsing thrombotic complications in patients with antiphospholipid syndrome. Direct oral anticoagulants are nonetheless sometimes used in this setting. We report two cases of women who were triple-positive for antiphospholipid antibodies and developed catastrophic antiphospholipid syndrome in the week after the introduction of rivaroxaban. The first patient, who had had a previous thrombotic event, had multiorgan failure 3 days after vitamin K antagonists was replaced by rivaroxaban, and the second developed a similar clinical presentation 7 days after introduction of the same treatment. Both catastrophic antiphospholipid syndrome episodes were successfully treated with heparin followed by vitamin K antagonists, corticosteroids, and plasmapheresis. These two cases highlight for the inefficacy of rivaroxaban preventing severe thrombotic events such as catastrophic antiphospholipid syndrome and thus provide further support for recommendations that vitamin K antagonists must remain the reference anticoagulant in patients with triple-positive antiphospholipid antibodies.

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