Catastrophic antiphospholipid syndrome

Catastrophic antiphospholipid syndrome (CAPS) is a rare and life-threatening disorder characterised by arterial or venous thrombotic events, involving three or more organs in a short period of time, in the presence of persistent antiphospholipid antibodies. Long-term anticoagulation with warfarin is the standard of care to prevent recurrent vascular events. Besides supportive care, optimal management of CAPS is unclear and consensus among experts is lacking. We describe a patient with primary antiphospholipid syndrome who experienced probable CAPS after receiving rivaroxaban, resulting in extensive cutaneous ulceration, acute coronary syndrome and dialysis-dependent renal failure...

OBJECTIVES: To evaluate whether inflammatory and complement biomarkers are associated with specific characteristics of antiphospholipid syndrome (APS). METHODS: Serum levels of interleukin (IL)-1β (IL-1β), IL-6, IL-8, IL-10, tumour necrosis factor (TNF)-α, interferon-α (IFN)-α, IFN-γ, vascular endothelial growth factor (VEGF), intercellular adhesion molecule 1 (ICAM-1), E-selectin, and vascular cell adhesion molecule (VCAM)-1, and plasma levels of soluble C5b-9 (sC5b-9), C3a, C4a, Bb fragment were measured in unselected APS patients...

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombosis and pregnancy morbidity with the persistent presence of antiphospholipid antibodies (aPLs). Although anticoagulation is the primary treatment for APS, it fails in approximately 20-30% of obstetric APS cases and more than 30% of thrombotic APS cases. Therefore, there is a need for new, targeted treatments beyond anticoagulants. Biologics, such as rituximab and eculizumab, have been recommended for refractory catastrophic APS...

Hypertensive disorders of pregnancy typically occur in the third trimester, with earlier presentations associated with underlying disorders such as antiphospholipid syndrome (APLS). We describe a case of a young primigravida presenting at 15 weeks 6 days gestation with epigastric pain, vomiting, new-onset severe-range hypertension, and subsequent development of anemia, thrombocytopenia, and transaminitis. Antiphospholipid antibodies (aPL) were triple-positive and imaging was negative for thrombosis. She was treated with aspirin, therapeutic anticoagulation, and ultimately dilatation and evacuation with initial postoperative improvement...

UNLABELLED: Catastrophic antiphospholipid syndrome (CAPS) is a rare entity, approximately 600 cases have been reported around the world, and the prevalence in Mexico is unknown. OBJECTIVE: To determine the estimated prevalence of CAPS in Mexico. MATERIAL AND METHODS: A literature search of isolated clinical cases or case series was conducted in diverse search engines, using the terms: "Catastrophic Antiphospholipid Syndrome" and "Mexico" in May 2022...

Coincidences in medicine are not so common. We are presenting a case of a patient diagnosed with Moya-Moya disease and antiphospholipid syndrome (APS) who presented with clinical and laboratory characteristics of catastrophic APS versus TTP. The diagnosis was a challenge because characteristics were overlapping. Nevertheless, a decision to treat the patient for TTP was made with afterward improvement. MMD has been associated with multiple immune disorders; however, only one case of acquired thrombotic thrombocytopenic purpura has been documented in association with this disease...

Catastrophic antiphospholipid syndrome (CAPS) is the rare but most severe form of antiphospholipid syndrome with multiple organ ischemia developing over a short period of time. CAPS should be considered when imaging suggests an acute and concurrent multiorgan ischemia, associated with positive antiphospholipid antibodies. As CAPS can have fulminant irreversible complications, its early recognition is important to initiate the treatment promptly. We present three patients of CAPS who were managed at our institution...

A 44-year-old woman with obstetric antiphospholipid syndrome (APS) presented to our institution with suspected non ST-elevation myocardial infarction (NSTEMI). Shortly after coronary angiography, she developed diplopia, hypotension and chest pain with inferior ST elevation in 12-lead ECG. According to multidisciplinary evaluation, she promptly underwent systemic thrombolysis, with clinical and haemodynamic improvement. Eventually, a diagnosis of catastrophic APS was made, with multiorgan ischemic involvement confirmed by blood examinations and multimodal imaging techniques...

BACKGROUND: Antiphospholipid syndrome (APS), particularly the catastrophic antiphospholipid syndrome (CAPS), is one of the rare causes of thrombotic microangiopathy (TMA). CAPS is the most severe form of APS, especially when accompanied by complement dysregulation, causes progressive microvascular thrombosis and failure in multiple organs. In this report, a case of CAPS with TMA accompanied by a genetic defect in the complement system is presented. CASE: A 13-year-old girl was admitted to the hospital with oliguric acute kidney injury, nephrotic range proteinuria, Coombs positive hemolysis, refractory thrombocytopenia, a low serum complement C3 level and anti-nuclear antibody (ANA) positivity...

BACKGROUND: Avatrombopag is an orally administered second-generation thrombopoietin receptor agonist (TPO-RA) approved for the treatment of chronic immune thrombocytopenia (ITP). However, increased thrombogenicity in patients with ITP after initiation of TPO-RA treatment has been reported. KEY CLINICAL QUESTION: We report a case of a patient with ITP who developed a catastrophic antiphospholipid antibody syndrome (CAPS), following treatment with avatrombopag. CLINICAL APPROACH: A20-year-old known chronic patient with ITP presented at the emergency department with a 2-week history of headache, nausea, and abdominal pain, 3 weeks after initiating avatrombopag...

COVID-19 infection has been found to precipitate hypercoagulability and transiently increase antiphospholipid antibodies. However, it is yet to be determined how likely these transient changes contribute to thrombotic events and antiphospholipid syndrome. We present a case in which antiphospholipid antibodies were detected in the presence of significant thromboses. The patient was subsequently treated for suspected catastrophic antiphospholipid syndrome following COVID-19 infection.

Catastrophic antiphospholipid antibody syndrome (CAPS) is a life-threatening disorder. It is a rare and severe form of antiphospholipid antibody (APL) syndrome characterized by widespread multisystemic thrombosis. We present a 55-year-old male patient with acute cerebellar hemorrhagic stroke who developed widespread progressive microthrombosis and macrothrombosis manifesting as progressive bilateral ischemic strokes with lower extremities deep vein thrombosis (DVT) and acute renal failure within a week of presentation...

INTRODUCTION: Antiphospholipid syndrome (APS) is an acquired autoimmune thrombophilia, characterized by vascular thrombosis or obstetric compromise, associated with the presence of antiphospholipid antibodies. Large international studies have analyzed the clinical/serological behavior of the disease and in Colombia, there are few cohorts that have been evaluated. OBJECTIVE: The main objective is to characterize the patients with APS followed in the anticoagulation clinic of a tertiary care hospital and to determine the clinical manifestations and serological findings at diagnosis...

We aimed to investigate the epidemiology, the clinical and laboratory characteristics of the pediatric involvement of antiphospholipid syndrome (APS), by performing a review of the current evidence and reviewing local experience in the Northwest Italy. To achieve this, we performed a detailed literature search to identify articles describing clinical and laboratory characteristics of pediatric APS. In concomitance, we conducted a registry-based study collecting data from the Piedmont and Aosta Valley Rare Disease Registry including pediatric patients diagnosed with APS in the last 11 years...

Antiphospholipid syndrome (APS) is a condition in which the body produces antiphospholipid antibodies, resulting in arterial and venous thrombosis. Catastrophic antiphospholipid syndrome (CAPS) is a rare APS subtype characterized by acute thrombotic microangiography. Antiphospholipid antibodies cause thrombosis through activating and inhibiting properties. CAPS is caused by conditions or factors that trigger the production of antiphospholipid antibodies: genetics that increases the risk of antiphospholipid antibody-associated thrombosis, infection, surgery, medications, and malignancy...

PURPOSE OF REVIEW: Antiphospholipid syndrome (APS) is an acquired thrombo-inflammatory disease that has morbid and sometimes devastating effects on patients and their families. This review will discuss the most recent international societal treatment guidelines and propose practical management algorithms for various APS sub-types. RECENT FINDINGS: APS represents a disease spectrum. Although thrombosis and pregnancy morbidities are traditional hallmarks of APS, a variety of extra-criteria clinical phenotypes can often be seen, which makes clinical management more challenging...

BACKGROUND: Eltrombopag, a thrombopoietin receptor (TPO-R) agonist, is considered a second-line treatment for patients with refractory immune thrombocytopenia (ITP). Systemic lupus erythematosus (SLE) is frequently associated with ITP. In some cases, thrombocytopenia in SLE patients is attributed to concurrent antiphospholipid antibodies (APLA). Currently, data regarding treatment with TPO-R agonists for ITP in SLE or APLA patients are limited. The incidence of SLE flare or antiphospholipid syndrome while on TPO-R agonists has not been well-studied...

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We report a patient with catastrophic antiphospholipid syndrome who had significant improvement after corticosteroids, plasmapheresis, argatroban, rituximab, and sirolimus. Argatroban was used instead of heparin due to a history of heparin-induced thrombocytopenia.

Systemic lupus erythematosus is a chronic autoimmune disease that affects most tissues. Cardiovascular events are critical, life-threatening, long-term complications of systemic lupus erythematosus (SLE). We report our single-center experience of performing cardiovascular surgery in patients with SLE while avoiding postoperative complications. We also suggest a new approach for cardiopulmonary bypass and perioperative management. We applied the antiphospholipid antibody syndrome (APS) severity classification published by the Japan Intractable Disease Information Center to patients with SLE for perioperative management...