Catastrophic antiphospholipid syndrome (CAPS)

Sciascia, Savino; López‐Pedrera, Chary; Roccatello, Dario; Cuadrado, María J.

doi:10.1016/j.berh.2012.07.005

Cited by 51 publications

(45 citation statements)

References 23 publications

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“…A negative aPL test is also a valid tool to distinguish between APS and other possible causes of TMA.21 Catastrophic should be ruled out, especially in patients who have thrombosis in multiple organs that develops over a short period of time. 22 Histological findings in APS neurophathy include distinctive microangiopathic features (focal and diffuse) that might affect any of the vessels within the intrarenal vasculature, including the glomeruli (Figure 3). 1,15,16 In studies of kidney biopsy samples from patients with primary APS, Nochy and co-workers18 showed that all vaso-occlusive lesions were characterized by acute thrombosis (TMA) and chronic vascular lesions, such as fibrous intimal hyperplasia of interlobular arteries, recanalizing thrombi in arteries and arterioles, fibrous occlusions and focal cortical atrophy.18 Ultrastructural findings might include glomerular basement membrane reduplication associated with glomerular basement membrane wrinkling.15,16 Fakhouri and co-workers23 found evidence of glomerulonephritis in 29 biopsy samples obtained from patients with primary APS without.…”

Section: Aps Nephropathymentioning

confidence: 99%

“…20 The use of cyclophosphamide, therefore, might be useful to treat catastrophic APS in patients with co-existing SLE . 22 Long-term intravenous immunoglobulin has been reported in a few cases of primary APS in which patients have relapsed despite standard treatment.111 New oral anticoagulants, such as dabigatran and rivaroxaban, are available. They have been efficacious in the management of venous thromboembolism and do not require laboratory monitoring.…”

Section: Treatmentmentioning

confidence: 99%

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Renal involvement in antiphospholipid syndrome

et al. 2014

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Antiphospholipid syndrome (APS) is an autoimmune disease defined by the presence of arterial or venous thrombotic events and/or pregnancy morbidity in patients who test positive for antiphospholipid antibodies (aPL). APS can be isolated (known as primary APS) or associated with other autoimmune diseases, such as systemic lupus erythematosus (SLE; known as secondary APS). The kidney is a major target organ in APS and renal thrombosis can occur at any level within the vasculature of the kidney (renal arteries, intrarenal arteries, glomerular capillaries and renal veins);events reflect the site and size of the involved vessels. Histological findings vary widely, including ischaemic glomeruli and thrombotic lesions, without glomerular or arterial immune deposits on immunofluorescence. Renal prognosis is affected by the presence of aPL in patients with lupus nephritis and can be poor. In patients with SLE and aPL, biopsy should be performed because inflammatory and thrombotic lesions require different therapeutic approaches. Renal involvement in patients with definite APS is treated by anticoagulation with long-term warfarin. The range of renal manifestations associated with APS is broadening and, therefore, aPL has increasing relevance in end-stage renal disease, transplantation and pregnancy.

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Section: Aps Nephropathymentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

Renal involvement in antiphospholipid syndrome

et al. 2014

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“…Surgery is a common trigger for C-APLAS because of the associated systemic inflammatory response syndrome. 11 Therefore, placing an LVAD via a sternotomy would likely increase the risk of an LVAD thrombus in the perioperative period. If LVAD or biventricular support is pursued, one must also consider if she would receive such support as a bridge to transplantation or recovery or as destination therapy.…”

Section: Dr Kirshenbaummentioning

confidence: 99%

“…12 Intravenous corticosteroids have been used to reduce inflammation and the systemic inflammatory response syndrome that occurs during C-APLAS. Intravenous immunoglobulin has been used to block the antiphospholipid antibodies and to increase their clearance, 11 but it would constitute a large volume load for a patient in cardiogenic shock. Plasma exchange has emerged as one of the most effective treatments of C-APLAS because it removes the pathological antibodies and inflammatory cytokines and replaces anticoagulant proteins such as proteins C and S, which become depleted during C-APLAS.…”

Section: Dr Kirshenbaummentioning

confidence: 99%

“…Plasma exchange has emerged as one of the most effective treatments of C-APLAS because it removes the pathological antibodies and inflammatory cytokines and replaces anticoagulant proteins such as proteins C and S, which become depleted during C-APLAS. 11,13 Rituximab, a monoclonal antibody for CD20-positive B cells, has also been used in the treatment of C-APLAS in an effort to reduce the production of the antiphospholipid antibodies. 14 In addition to the management of the C-APLAS itself, her tenuous hemodynamics must be addressed.…”

Section: Dr Kirshenbaummentioning

confidence: 99%

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Myocardial Catastrophe

et al. 2014

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A 44-year-old woman presented to her local emergency room with a 1-day history of nausea, fatigue, and fever of 101°F. At that time, she denied chest pain, dyspnea, cough, orthopnea, or lower-extremity edema. An ECG was reportedly normal, and she was treated with antiemetics and fluids and discharged home. The next day, she developed persistent, aching pain in her chest and jaw and presented to her primary care physician. Her medical history included obesity, depression, hyperlipidemia, and antiphospholipid antibody syndrome (APLAS) initially diagnosed in 1989 when she was 20 years of age complicated by 4 miscarriages, pulmonary emboli, and deep vein thrombosis for which she underwent inferior vena cava filter placement in 1997. She had experienced no thromboembolic events over the next 15 years. Her home medications were rivaroxaban 20 mg daily, aspirin 81 mg daily, furosemide 20 mg daily, simvastatin 20 mg daily, escitalopram 20 mg daily, and omeprazole 20 mg daily. She worked as a nurse and lived with her husband and 4 adopted children. She never smoked. Her mother carried the diagnosis of factor V Leiden. She was scheduled for an elective gastric bypass surgery in 2 days and had been instructed to hold her rivaroxaban for 4 days before surgery; therefore, she stopped anticoagulation 2 days before presentation.Dr Kirshenbaum: Her initial symptoms are quite nonspecific, with nausea and a low fever suggesting a viral process. However, the subsequent development of chest and jaw pain also raises the possibility of cardiac causes such as an acute coronary syndrome. Myocarditis also often presents with chest pain that can mimic an acute coronary syndrome and can be preceded by a viral prodrome. In addition, the recent discontinuation of rivaroxaban in a patient with a history of severe thrombophilia and APLAS is worrisome. Rivaroxaban carries a boxed warning that cautions that premature discontinuation of the drug increases the risk of thrombotic events and suggests coverage with another anticoagulant if clinically warranted.1 This patient had no bridging anticoagulation prescribed. Therefore, a new thrombotic event such as mesenteric ischemia or coronary thrombosis (epicardial or microvascular occlusion) must also be considered.Patient presentation (continued): Her chest pain led to a repeat evaluation in the same emergency room where she had presented the day before. At this point, her ECG had ST-segment elevations in the inferior and apical leads (Figure 1) that were new compared with her ECG 24 hours earlier. Her laboratory work at that time was notable for a troponin I level of 19 ng/mL. She was given aspirin, clopidogrel, and atorvastatin, started on unfractionated heparin; and transferred to a percutaneous coronary intervention-capable facility for cardiac catheterization.On arrival at the closest percutaneous coronary intervention-capable facility, she was afebrile and had a heart rate of 107 bpm, blood pressure of 105/74 mm Hg, respiratory rate of 24 breaths per minute, and oxygen saturation of 97% on 2 L...

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Diagnosis and management of antiphospholipid syndrome

Heuser

Branch

2018

Evidence‐based Obstetrics and Gynecology

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Catastrophic antiphospholipid syndrome (CAPS)

Cited by 51 publications

References 23 publications

Renal involvement in antiphospholipid syndrome

Renal involvement in antiphospholipid syndrome

Myocardial Catastrophe

Diagnosis and management of antiphospholipid syndrome

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