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.

scholarly journals Case of a Сombination of Lupus Erythematosus, Antiphospholipid Syndrome and Myocardial Infarction

Kardiologiia ◽  
2019 ◽  
Vol 59 (12) ◽  
pp. 92-96
Author(s):  
N. A. Kosheleva ◽  
N. M. Nikitina ◽  
E. U. Andreeva
Keyword(s):  
Myocardial Infarction ◽  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
The Body ◽  
Systemic Autoimmune Disease ◽  
Unknown Etiology ◽  
Systemic Lupus ◽  
Wide Range

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology characterized by a wide range of clinical manifestations with damage to various organs and systems of the body. There are bad prognostic factors for SLE: damage to the heart, kidney, central nervous system, the development of hematological crises and secondary antiphospholipid syndrome. A number of authors consider systemic lupus erythematosus a “new” risk factor for atherosclerosis. The overall risk of myocardial infarction (MI) in patients with SLE is 10 times higher than in the general population. The article presents clinical case report of the development of myocardial infarction in a woman with SLE, receiving therapy for secondary antiphospholipid syndrome.

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.

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

scholarly journals The Wolf Hidden behind the Clots: Catastrophic Antiphospholipid Antibody Syndrome

2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Myat Han Soe ◽  
Krishna Adit Agarwal ◽  
Alueshima Akough-Weir
Keyword(s):  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Anticoagulation Therapy ◽  
Multiple Organ ◽  
Clinical Syndrome ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Case Review ◽  
Cmv Infection ◽  
Thrombophilic Condition

Catastrophic antiphospholipid syndrome (CAPS) is a rare but highly fatal clinical syndrome that occurs in up to 1% of patients with antiphospholipid syndrome (APS). The diagnosis of CAPS is often delayed because its presentation with multiple organ thromboses can be confused with other thrombotic microangiopathies and severe sepsis. We report a case of CAPS in a patient with APS and systemic lupus erythematosus (SLE) presenting with thrombotic storm precipitated by trauma, cytomegalovirus (CMV) infection, and noncompliance with anticoagulation therapy. Our case reflects the “two-hit hypothesis” of APS in which the presence of antiphospholipid antibodies (first hit) increases the thrombophilic risk, and thromboses take place in the presence of another thrombophilic condition such as CMV infection in our case. In this case review, we discuss the diagnostic challenges and management of CAPS. In clinical practice, we aim to stress the importance of thorough evaluation and management of precipitating events such as infections in addition to timely diagnosis and treatment of this catastrophic clinical entity.

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 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 Children Living a Global Pandemic: Anxiety Repercussions

2021 ◽  
Author(s):  
Salvador I. Garcia-Adasme ◽  
Alejandro López-Escobar
Keyword(s):  
Physical Activity ◽  
Social Media ◽  
Clinical Manifestations ◽  
Multiple Organ ◽  
School Closures ◽  
Balanced Diet ◽  
Global Pandemic ◽  
Wide Range ◽  
Anxiety Levels ◽  
Children’S Anxiety

A global pandemic caused by SARS-CoV-2 is still beaten our world. The disease, termed COVID-19 by the WHO, has a wide range of clinical manifestations, ranging from a mild, self-limiting form of the disease to multiple organ failure and death, forcing governments to take measures to mitigate the transmission and reduce the economic impact. However, the paediatric manifestation appears to take a milder form of the disease but they are not oblivious to the consequences of the disease. They suffered personal and parental lost, broke their social relationships, forced to home confinement, school closures, all of them with secondary implications. As a result, children’s anxiety levels and manifestations have increased during pandemic. To prevent and counteract this situation, measures were implemented like increase physical activity, a balanced diet, and regular sleep pattern; and in relationship sphere use social media to stay in touch with school mates and relatives.

scholarly journals Does Adjusted Global Antiphospholipid Syndrome Score (aGAPSS) Predict the Obstetric Outcome in Antiphospholipid Antibody Carriers? A Single-Center Study

2021 ◽  
Author(s):  
Sara Del Barrio-Longarela ◽  
Víctor M. Martínez-Taboada ◽  
Pedro Blanco-Olavarri ◽  
Ana Merino ◽  
Leyre Riancho-Zarrabeitia ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Antiphospholipid Syndrome ◽  
Adverse Pregnancy Outcome ◽  
Clinical Manifestations ◽  
Live Birth Rate ◽  
Response To Treatment ◽  
Antiphospholipid Antibody ◽  
Single Center Study ◽  
Patients At Risk ◽  
Adverse Pregnancy

AbstractThe adjusted Global Antiphospholipid Syndrome (APS) Score (aGAPSS) is a tool proposed to quantify the risk for antiphospholipid antibody (aPL)-related clinical manifestations. However, aGAPSS has been validated mainly for thrombotic events and studies on APS-related obstetric manifestations are scarce. Furthermore, the majority of them included patients with positive aPL and different autoimmune diseases. Here, we assess the utility of aGAPSS to predict the response to treatment in aPL carriers without other autoimmune disorders. One-hundred and thirty-seven women with aPL ever pregnant were included. Sixty-five meet the APS classification criteria, 61 had APS-related obstetric manifestations, and 11 were asymptomatic carriers. The patients’ aGAPSS risk was grouped as low (< 6, N = 73), medium (6–11, N = 40), and high risk (≥ 12, N = 24). Since vascular risk factors included in the aGAPSS were infrequent in this population (< 10%), the aGAPSS score was mainly determined by the aPL profile. Overall, the live birth rate was 75%, and 37.2% of the patients had at least one adverse pregnancy outcome (APO). When considering patients according to the aGAPSS (high, medium, and low risk), no significant differences were found for pregnancy loss (29.2%, 25%, and 21.9%) or APO (33.3%, 47.5%, and 32.9%). In the present study, including aPL carriers without other autoimmune diseases, aGAPSS is not a valuable tool to identify patients at risk for obstetric complications despite treatment. In these patients with gestational desire, in addition to the aPL profile, other pregnancy-specific factors, such as age or previous obstetric history, should be considered.

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