scholarly journals Evaluation of the severe preeclampsia classification criterion for antiphospholipid syndrome in a study of 40 patients

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Maddalena Larosa ◽  
Véronique Le Guern ◽  
Nathalie Morel ◽  
Mériem Belhocine ◽  
Amelia Ruffatti ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Gestational Age ◽  
Lupus Erythematosus ◽  
Placental Abruption ◽  
Antibody Test ◽  
Severe Preeclampsia ◽  
First Episode ◽  
Foetal Death ◽  
Rheumatology Unit

Abstract Background The criteria for antiphospholipid syndrome (APS) include severe preeclampsia and/or placental insufficiency leading to preterm delivery before 34 weeks of gestation, but this APS manifestation has been rarely studied. Thus, we report a series of severe preeclampsia occurred in patients with APS. Methods We retrospectively analysed data of women with APS (Sydney criteria) who experienced severe preeclampsia with delivery before 34 weeks’ gestation between 2000 and 2017 at five French internal medicine departments and one Italian rheumatology unit. Results The 40 women had a mean age of 30.5 ± 4.6 years at their first episode of preeclampsia; 21 were nulligravid (52.5%), 12 (30%) had already been diagnosed with APS, and 21 (52.5%) had a triple-positive antiphospholipid (aPL) antibody test. Preeclampsia occurred at a median gestational age of 25.5 weeks (IQR 23-29). It was associated with HELLP in 18 cases (45%), eclampsia in 6 (15%), placental abruption in 3 (7.5%), catastrophic APS in 3 (7.5%), and foetal and neonatal death in 11 and 15 cases. Overall, 14 (35%) children survived, born at a median gestational age of 31 weeks. Among other APS criteria, 16 women (40%) experienced at least one thrombosis, 17 (42.5%) an intrauterine foetal death, and 19 (47.5%) at least one episode of HELLP during follow-up (median 5 years, IQR = 2-8). None had three or more consecutive miscarriages. Notably, 12 women (30%) had systemic lupus erythematosus. Conclusions Severe preeclampsia led to high mortality in the offspring. Almost half of these women experienced other APS features, but not three consecutive miscarriages.

scholarly journals Evaluation of the severe preeclampsia classification criterion for antiphospholipid syndrome in a study of 40 patients

2021 ◽  
Author(s):  
Maddalena Larosa ◽  
Veronique Le Guern ◽  
Nathalie Morel ◽  
Mériem Belhocine ◽  
Amelia Ruffatti ◽  
...  
Keyword(s):  
Internal Medicine ◽  
Preterm Delivery ◽  
Antiphospholipid Syndrome ◽  
Antibody Test ◽  
Placental Insufficiency ◽  
Large Series ◽  
Severe Preeclampsia ◽  
First Episode ◽  
Classification Criterion ◽  
Rheumatology Unit

Abstract Background The criteria for antiphospholipid syndrome (APS) include severe preeclampsia and/or placental insufficiency leading to preterm delivery before 34 weeks of gestation, but this APS manifestation has been rarely studied. Thus, we reported a large series of severe preeclampsia occurred in patients with APS. Methods We retrospectively analysed data of APS women (Sydney criteria) who experienced severe preeclampsia with delivery before 34 weeks' gestation between 2000 and 2017 at five French internal medicine departments and one Italian rheumatology unit. Results The 40 women had a mean age of 30.5 ± 4.6 years at their first episode of preeclampsia; 21 were nulligravid (52.5%), 12 (30%) had already been diagnosed with APS, and 21 (52.5%) had a triple-positive antiphospholipid (aPL) antibody test.

Are Patients Who Have Recovered From ADAMTS13-Deficient Thrombotic Thrombocytopenia Purpura (TTP) at Risk for Developing Systemic Lupus Erythematosus (SLE)?

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 2519-2519
Author(s):  
Deirdra R. Terrell ◽  
Zayd Al-Nouri ◽  
Judith A. James ◽  
Johanna A. Kremer Hovinga Strebel ◽  
Bernhard Lämmle ◽  
...  
Keyword(s):  
At Risk ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Conflicts Of Interest ◽  
Population Data ◽  
First Episode ◽  
Adamts13 Activity ◽  
Acr Criteria ◽  
Adamts13 Deficiency

Abstract Abstract 2519 TTP associated with acquired, ADAMTS13-deficiency and SLE are both autoimmune disorders that occur preferentially in young, black women and they have many similar clinical features. TTP may occur in patients previously diagnosed with SLE, or patients may develop SLE following recovery from TTP. In addition, TTP may be quite difficult to distinguish from SLE patients with severe hematologic manifestations. We compared the prevalence of SLE-associated autoantibodies in TTP patients to published population data using 95% confidence intervals (CI). The Oklahoma TTP Registry enrolled 292 consecutive patients with their first episode of clinically diagnosed TTP from 11–13-1995 (date of our initial ADAMTS13 measurement) to 7–31-2009; ADAMTS13 activity was measured in 271 (93%) patients; 64 (24%) patients had ADAMTS13 activity <10%, 63 were evaluated for SLE-associated autoantibodies, including 2 patients with a previous diagnosis of SLE. Serum from the patient's acute initial episode was used for analysis. The prevalence of ANA, anti-dsDNA, anti-Ro, and aPL in TTP patients was significantly higher than published population data; prevalences of anti-nRNP, anti-Sm, and anti-La were not different. Autoantibody TTP (95% CI) Population % ANA  ≥1:40 89% (78%–95%) 0–27%  ≥1:120 56% (42%–68%) 0% Anti-dsDNA  ≥1:30 43% (30%–56%) 3% Anti-Ro  OD>0.350 17% (8%–29%) 3% aPL IgM  ≥20 PL units 15% (7%–26%) 2% Because of the increased prevalence of SLE-associated autoantibodies, we evaluated our TTP patients for the America College of Rheumatology (ACR) criteria for SLE (presence of ≥4 of 11 criteria suggests the diagnosis of lupus); abnormalities associated with any TTP episode were not counted in this evaluation of clinical criteria for SLE. By definition ACR criteria can be fulfilled serially or simultaneously over a lifetime. Evaluations were completed between 6-1-2007 and 5-1-2009 on 38/42 (90%) eligible patients (alive, non-institutionalized, no previous SLE diagnosis) consisting of physical examination, review of available lifetime medical records, and serial laboratory evaluations. Patients have been followed for a median of 8.3 years (range, 1–14 years). During this time, 3 (8%) developed clinically evident SLE requiring treatment 1, 5, and 70 months after their initial TTP episodes. Among the other 35 patients, 3 (8%) have ≥4 SLE classification criteria by medical record review (1 had pre-existing Sjögren's syndrome and receives treatment; 2 have minimal clinical features and are not actively treated for SLE); 9 (24%) have 3 criteria; 16 (42%) have 2 criteria; 6 (16%) have 1 criterion; and 1 (2%) patient has no ACR criteria for SLE. All patients continue to be followed and clinically evaluated for potential intervention. SLE diagnosis is a clinical designation and because of the lack of disease modifying drugs, routine follow-up is standard of care unless the patient is symptomatic. Conclusions: [1] A high prevalence of SLE-associated autoantibodies was present in a cohort of consecutive patients with TTP associated with acquired severe ADAMTS13 deficiency. [2] The presence of anti-dsDNA, anti-Ro, aPL and high titers of ANA suggest that patients with ADAMTS13-deficient TTP may be at risk for developing SLE. [3] During long-term follow-up, 6 (16%) of 38 patients have developed overt SLE or ACR criteria without an established diagnosis of SLE. Careful continuing evaluation following recovery from TTP is important. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Secondary autoimmune diseases occurring after HSCT for an autoimmune disease: a retrospective study of the EBMT Autoimmune Disease Working Party

Blood ◽  
2011 ◽  
Vol 118 (6) ◽  
pp. 1693-1698 ◽  
Author(s):  
Thomas Daikeler ◽  
Myriam Labopin ◽  
Massimo Di Gioia ◽  
Mario Abinun ◽  
Tobias Alexander ◽  
...  
Keyword(s):  
Risk Factors ◽  
Autoimmune Disease ◽  
Autoimmune Diseases ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Working Party ◽  
Thyroid Stimulating Hormone ◽  
European Multicenter Study ◽  
Autologous Hsct

Abstract To specify the incidence and risk factors for secondary autoimmune diseases (ADs) after HSCT for a primary AD, we retrospectively analyzed AD patients treated by HSCT reported to EBMT from 1995 to 2009 with at least 1 secondary AD (cases) and those without (controls). After autologous HSCT, 29 of 347 patients developed at least 1 secondary AD within 21.9 (0.6-49) months and after allogeneic HSCT, 3 of 16 patients. The observed secondary ADs included: autoimmune hemolytic anemia (n = 3), acquired hemophilia (n = 3), autoimmune thrombocytopenia (n = 3), antiphospholipid syndrome (n = 2), thyroiditis (n = 12), blocking thyroid-stimulating hormone receptor antibody (n = 1), Graves disease (n = 2), myasthenia gravis (n = 1), rheumatoid arthritis (n = 2), sarcoidosis (n = 2), vasculitis (n = 1), psoriasis (n = 1), and psoriatic arthritis (n = 1). After autologous HSCT for primary AD, the cumulative incidence of secondary AD was 9.8% ± 2% at 5 years. Lupus erythematosus as primary AD, and antithymocyte globulin use plus CD34+ graft selection were important risk factors for secondary AD by multivariate analysis. With a median follow-up of 6.2 (0.54-11) years after autologous HSCT, 26 of 29 patients with secondary AD were alive, 2 died during their secondary AD (antiphospholipid syndrome, hemophilia), and 1 death was HSCT-related. This European multicenter study underlines the need for careful management and follow-up for secondary AD after HSCT.

scholarly journals Primary antiphospholipid syndrome progressing to systemic lupus erythematosus: a case report

2013 ◽  
pp. 116-121
Author(s):  
Rocco Manganelli ◽  
Salvatore Iannaccone ◽  
Walter De Simone
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Primary Antiphospholipid Syndrome ◽  
Systemic Lupus ◽  
Anionic Phospholipids ◽  
Serological Studies ◽  
Homogeneous Pattern

Introduction: Primary antiphospholipid syndrome (APS) is a thrombophilic disease that should be suspected in the presence of thrombotic events associated with hematologic abnormalities such as thrombocytopenia and prolongation of the activated partial thromboplastin time. The diagnosis must be confirmed by the demonstration of autoantibodies directed against anionic phospholipids and/or phospholipid-binding proteins. The disease can cause arterial thrombosis in any vascular district, including those of the kidney and central nervous system. Case report: In 2006 a 29-year-old male presented with kidney and brain involvement that was attributed to primary APS. The clinical diagnosis was confirmed by the results of a renal biopsy, which excluded the presence of systemic lupus erythematosus (SLE). The patient remained stable through 32 months of follow-up and then developed a malar rash with deteriorating renal function, decreasing platelet count, and reduced complement levels. Serological studies revealed positivity for ANA (homogeneous pattern), dsDNA, ACA, and beta-2-glycoprotein-1 antibodies. The diagnosis was revised to APS secondary to SLE. Conclusions: A diagnosis of primary APS should not be considered permanent: progression to SLE can occur, in some cases years after the original diagnosis. This case highlights the importance of ongoing follow-up of patients diagnosed with primary APS to detect changes that herald the emergence of SLE.

Antiphospholipid syndrome damage index (DIAPS): distinct long-term kinetic in primary antiphospholipid syndrome and antiphospholipid syndrome related to systemic lupus erythematosus

Lupus ◽  
2020 ◽  
Vol 29 (3) ◽  
pp. 256-262 ◽  
Author(s):  
A Kuhl Torricelli ◽  
M Remião Ugolini-Lopes ◽  
E Bonfá ◽  
D Andrade
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Damage Index ◽  
Diagnosis Delay ◽  
Primary Antiphospholipid Syndrome ◽  
Systemic Lupus ◽  
Tertiary Center

Background Antiphospholipid syndrome (APS) is an acquired thrombophilia that affects young productive individuals, with permanent damage and negative impact on quality of life. Recently, a damage index specific for APS (DIAPS) was developed. There are, however, no data regarding the comparison of its performance and long-term damage in primary antiphospholipid syndrome (PAPS) and APS related to systemic lupus erythematosus (SLE; APS + SLE). The primary purpose of this study was therefore to compare the long-term damage in patients with these conditions. Methods This is a retrospective analysis of a single tertiary center cohort followed for approximately 10 years using a standardized prospective electronic chart database. Fifty consecutive PAPS patients age matched with 50 APS+SLE patients were consecutively selected for the study, and DIAPS was calculated once a year during follow-up. Long-term damage and damage kinetics in both groups were compared. Results PAPS and APS + SLE had comparable age (47.10 ± 12.4 vs. 44.04 ± 10.80 years; p = 0.19) and time of follow-up (9.40 ± 3.60 vs. 10.94 ± 4.50 years; p = 0.06). At diagnosis, PAPS had higher DIAPS than APS + SLE (1.72 ± 1.17 vs. 0.82 ± 0.96; p < 0.001). At the end of the 10-year follow-up, both groups presented comparable mean damage scores (2.04 ± 1.50 vs. 2.24 ± 1.61; p = 0.52). The damage increment throughout the observation period for PAPS was solely 35%, whereas for APS + SLE it was gradual, persistent and reached 139% at the end of follow-up, with a total damage increment for PAPS lower than APS + SLE (0.43 ± 0.30 vs. 1.22 ± 1.24; p < 0.001). Of note, the frequency of individuals who acquired damage was lower in PAPS than in APS + SLE (32% vs. 71%; p < 0.001). PAPS also had a longer delay in diagnosis than APS + SLE (4.00 ± 4.20 vs. 2.54 ± 3.05 years; p = 0.04). This delay was positively correlated with a higher damage score at diagnosis ( r = 0.36, p < 0.001) in all groups. Conclusion We identified a distinct pattern of damage in PAPS and APS related to SLE. Damage in PAPS is an early event, while APS+SLE is associated with higher long-term damage, with a striking increment of damage along the follow-up. A diagnosis delay is correlated with higher damage scores. Damage surveillance therefore requires different approaches for these two conditions.

Catastrophic antiphospholipid syndrome associated with systemic lupus erythematosus: a case-based review

2020 ◽  
Author(s):  
Jesus Garcia-Diaz ◽  
Mara Escudero-Salamanca ◽  
Ricardo Alvarez-Santana ◽  
Nilda Espinola-Zavaleta
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Underlying Disease ◽  
Rapid Progression ◽  
Systemic Lupus ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Good Clinical Condition ◽  
History Of

Antiphospholipid syndrome (APS) can occur as a primary disease or secondary to an underlying disease, such as systemic lupus erythematosus, or other systemic autoimmune diseases. Catastrophic APS refers to a rapid progression of the disease with the development of thrombotic events that affect three or more organs. This is the case of a 22-year-old woman without history of pregnancy. She developed a catastrophic APS associated with systemic lupus erythematosus, with kidney damage (focal lupus nephritis III), pulmonary embolism, and Libman–Sacks mitral valve endocarditis. Accurate diagnosis and optimal medical treatment (anticoagulants, corticosteroids, antimalarials, diuretics) improved her disease, and the patient was discharged in good clinical condition and continues her multidisciplinary follow-up in the outpatient clinic of our institution.

Hemoptysis and a cardiac murmur: is it primary or secondary antiphospholipid syndrome?

Lupus ◽  
2019 ◽  
Vol 28 (14) ◽  
pp. 1712-1715
Author(s):  
T Kolitz ◽  
O Fruchter ◽  
L Sasson ◽  
Y Geva ◽  
O Moreh-Rahav ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Infectious Agent ◽  
Intravenous Immunoglobulins ◽  
Clinical Findings ◽  
High Dose ◽  
Cardiac Murmur ◽  
Elective Procedure ◽  
Work Up

Endocarditis is most frequently infective in origin, and thus, when a patient presents with a clinical picture suggestive of endocarditis, an extensive work up aimed at finding the infectious agent is warranted. Among systemic lupus erythematosus (SLE) patients, cardiovascular disease is prevalent in more than 50% of patients including valvular disease and non-infective endocarditis, known as Libman–Sacks (LS) endocarditis. The prevalence of LS syndrome among SLE patients with secondary antiphospholipid syndrome (APS) is higher than in SLE without APS. Here, we present a case of a patient diagnosed with primary APS who presented with hemoptysis and a cardiac murmur. The diagnosis of SLE was established following the findings of non-infective verrucous vegetations together with diffuse alveolar hemorrhage (DAH). Treatment with high-dose corticosteroids and intravenous immunoglobulins yielded substantial resolution of the vegetations and regression of the DAH. Hence, aortic valve replacement was successfully performed as an elective procedure and without any postoperative complications. The patient is in remission after a 6-month follow-up. The clinical findings of DAH and double valve non-infectious endocarditis prompted the diagnosis of SLE with secondary APS.

scholarly journals Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients

2014 ◽  
Vol 74 (6) ◽  
pp. 1011-1018 ◽  
Author(s):  
R Cervera ◽  
R Serrano ◽  
G J Pons-Estel ◽  
L Ceberio-Hualde ◽  
Y Shoenfeld ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Current Treatment ◽  
Morbidity And Mortality ◽  
Live Births ◽  
Transient Ischaemic Attacks ◽  
Therapeutic Measures ◽  
Medical Histories ◽  
Deep Vein

ObjectivesTo assess the prevalence of the main causes of morbi-mortality in the antiphospholipid syndrome (APS) during a 10-year-follow-up period and to compare the frequency of early manifestations with those that appeared later.MethodsIn 1999, we started an observational study of 1000 APS patients from 13 European countries. All had medical histories documented when entered into the study and were followed prospectively during the ensuing 10 years.Results53.1% of the patients had primary APS, 36.2% had APS associated with systemic lupus erythematosus and 10.7% APS associated with other diseases. Thrombotic events appeared in 166 (16.6%) patients during the first 5-year period and in 115 (14.4%) during the second 5-year period. The most common events were strokes, transient ischaemic attacks, deep vein thromboses and pulmonary embolism. 127 (15.5%) women became pregnant (188 pregnancies) and 72.9% of pregnancies succeeded in having one or more live births. The most common obstetric complication was early pregnancy loss (16.5% of the pregnancies). Intrauterine growth restriction (26.3% of the total live births) and prematurity (48.2%) were the most frequent fetal morbidities. 93 (9.3%) patients died and the most frequent causes of death were severe thrombosis (36.5%) and infections (26.9%). Nine (0.9%) cases of catastrophic APS occurred and 5 (55.6%) of them died. The survival probability at 10 years was 90.7%.ConclusionsPatients with APS still develop significant morbidity and mortality despite current treatment. It is imperative to increase the efforts in determining optimal prognostic markers and therapeutic measures to prevent these complications.

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