Catastrophic antiphospholipid syndrome

Complement is a major driver of antiphospholipid syndrome (APS) and a promising therapeutic target in refractory and catastrophic APS. Complement testing in APS is largely limited to research settings, and reliable, rapid-turnaround biomarkers are needed to predict those at risk for adverse clinical outcomes and most likely to benefit from complement inhibition. We review complement biomarkers and their association with thrombosis and obstetric outcomes, including: (i) complement proteins and activation fragments in the fluid phase; (ii) assays that evaluate complement on cell membranes (e...

INTRODUCTION: Microvascular manifestations constitute a subtype of antiphospholipid syndrome, and those patients have relatively poor prognoses, so it is important to find markers for microvascular manifestations. This study was conducted to explore whether serum calprotectin could be a predictor of microvascular manifestations in antiphospholipid antibody (aPL)-positive patients. METHODS: Consecutive patients with persistent aPL positivity referred to Peking Union Medical College Hospital and age- and sex-matched health controls (HCs) were included...

A small subset of patients with antiphospholipid syndrome (APS) may develop widespread thrombotic disease with organ damage, referred to as catastrophic APS (CAPS) that is associated with a high mortality. Medical therapy typically involves a combination of anticoagulation, systemic glucocorticoids, plasmapheresis, and intravenous immune globulin (IVIG). There is currently no consensus for the management of refractory cases of CAPS. However, monoclonal antibodies such as rituximab and eculizumab have shown some benefits...

OBJECTIVES: To describe the pulmonary involvement in patients with catastrophic antiphospholipid syndrome (CAPS), focusing on its relationship with extrapulmonary involvement, laboratory, radiological, and pathological findings. METHODS: This retrospective cross-sectional study includes all patients grouped in the "CAPS Registry". All cases were reviewed, and those with pulmonary thromboembolism (PE) and/or diffuse alveolar hemorrhage (DAH) were selected. Data on pulmonary and extrapulmonary clinical presentation, radiologic patterns, laboratory findings, associated autoimmune diseases, treatments, and outcomes were analyzed...

OBJECTIVES: Complement activation has been advocated as one mechanism by which antiphospholipid antibodies (aPLs) can induce thrombosis. In patients with catastrophic aPL syndrome or re-thrombosis, enhanced complement activation was shown, even in quiescent phase of the disease. We aimed to assess complement activation and to investigate its association to clinical variables in aPL positive patients with a favorable disease course. METHODS: Subjects with at least two consecutive positive aPL antibody results obtained ≥12 weeks apart were enrolled...

Antiphospholipid syndrome (APS) is a chronic autoimmune disease involving vascular thrombosis and/or obstetric morbidity and persistent antibodies to phospholipids or certain phospholipid-associated proteins. It is a rare condition in adults and even rarer in children. The diagnosis of APS can be facilitated by the use of classification criteria based on a combination of clinical and biological features. APS may be rapidly progressive with multiple, often synchronous thromboses, resulting in life-threatening multiple organ failure...

BACKGROUND: Antiphospholipid syndrome (APS) is a systemic autoimmune disease clinically associated with thrombotic and obstetric events. Additional manifestations have been associated with APS, like diffuse alveolar hemorrhage (DAH). We aimed to summarize all the evidence available to describe the presenting clinical features, their prognostic factors, and short- and long-term outcomes. METHODS: We performed a mixed-method approach combining a multicenter cohort with a systematic literature review (SLR) of patients with incident APS-associated DAH...

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This case report aims to highlight the importance of keeping catastrophic antiphospholipid syndrome (CAPS) high on the list of differentials in patients with lupus who present with digital ischemia and to understand the workup and treatment of the disease. Catastrophic antiphospholipid syndrome is a life-threatening variant of antiphospholipid syndrome (APS), and it is distinguished on the APS spectrum by its increased intensity and extent of thrombotic outcomes. Less than 1% of patients with APS develop CAPS and the demographic of patients affected are primarily females, 37 ± 14 years old, and have underlying primary APS or systemic lupus erythematosus (SLE)...

Antiphospholipid syndrome is a rare, autoimmune thrombophilia defined by vascular thrombosis and pregnancy morbidity, in the setting of documented persistent antiphospholipid antibodies including the lupus anticoagulant, anticardiolipin antibodies, or anti-β2 glycoprotein I antibodies. The presence of antiphospholipid antibodies can be completely asymptomatic, or they can lead to clinical manifestations as severe as catastrophic antiphospholipid syndrome, which involves widespread coagulopathy over a very short period of time...

BACKGROUND: Antiphospholipid syndrome (APS), defined by thrombotic events or obstetric complications in the presence of persistently high antiphospholipid antibodies, is characterized by a wide variety of clinical presentations and the effects of vascular occlusion can impact almost any organ system or tissue. Since adult-onset APS classification criteria are not well verified in pediatrics (where pregnancy-related problems are rare), estimating childhood prevalence is challenging. Stroke and pulmonary embolism are thromboembolic events occurring in children that can cause considerable long-term morbidity...

Catastrophic antiphospholipid syndrome (CAPS) is a rare variant of antiphospholipid syndrome (APLS). CAPS is a syndrome characterized by microvascular thrombosis leading to multi-organ failure, including acute heart failure. Diagnosis is challenging, and disease progression is rapid. Treatment includes triple therapy with anticoagulation, glucocorticoids, and plasma exchange. We present a case of a patient with CAPS who developed de novo acute heart failure. With treatment, the patient's multi-organ failure improved, including cardiac function...

OBJECTIVES: Antiphospholipid syndrome (APS)-associated heart valve disease (HVD) is well described. Nonetheless, limited data exist on clinical parameters associated with the course of primary APS (pAPS) patients with HVD. The goal of this study was to assess clinical features and related outcomes in patients with APS associated HVD. METHODS: In this multicentre retrospective study, we identified 33 pAPS patients with HVD (pAPS-HVD group) and compared their clinical course with 128 pAPS patients with normal heart valves on echocardiography (pAPS-control group)...

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder clinically characterized by recurrent arterial and venous thrombosis and/or pregnancy morbidity in the presence of antiphospholipid antibodies. Currently, treatment is mainly focused on anticoagulation, but therapies targeting mechanisms involved in APS autoimmune pathogenesis could play an important role in specific settings. An evidence-based therapeutic approach is limited by the broad clinical spectrum of the syndrome and the nature of a "rare disease" that makes it difficult to carry out well-designed prospective studies...

A very rare and severe disease catastrophic antiphospholipid syndrome is defined by small vessel occlusions resulting in multi-organ involvement in the presence of antiphospholipid antibodies. This case report presents a case of catastrophic antiphospholipid syndrome in a young female without past medical history.

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Penile glans necrosis is a rare clinical condition caused by trauma, diabetes mellitus, adverse effect of vasoconstrictive solutions, and circumcision. Antiphospholipid syndrome (APS) is categorized as an autoimmune disease with the presence of antiphospholipid antibodies that results in an increased risk of vascular thrombosis and obstetrical complications. In this article, we report a rare case of a 20-year-old boy with penile glans necrosis due to penile vascular thrombosis following catastrophic antiphospholipid syndrome (CAPS) which we successfully treated at People's Hospital 115...

BACKGROUND/AIM: Catastrophic antiphospholipid syndrome (CAPS) may be the first manifestation ("de novo") of antiphospholipid syndrome (APS) or a complication in the clinical course of patients known to have this syndrome. Approximately 40% of patients had an associated autoimmune disease, mainly, systemic lupus erythematosus (SLE). The trigger can be one of the following: infections, surgical interventions, neoplasms, pregnancy, discontinuation of anticoagulant treatment, and others. CAPS is a medical emergency in which early identification and prompt initiation of aggressive therapy is extremely important...

PURPOSE: To describe choroidal involvement in catastrophic antiphospholipid syndrome (CAPS). METHODS: We report here two cases of bilateral CAPS choroidopathy in two female patients. RESULTS: Case report 1: A thirty-five-year-old female patient, with history of primary anti-phospholipid syndrome (APS), treated with anticoagulants, presented an acute renal failure following a salpingectomy. She complained of bilateral acute blurred vision. Ophthalmologic evaluation revealed visual acuity (VA) of 5/10, extensive serous retinal (SRD) detachment, areas of hypofluorescence on fluorescein angiography (FA), and non-perfusion areas in the choriocapillaris , on optical coherence tomography angiography (OCT-A), in both eyes...

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...