Livedo reticularis is a marker for predicting multi-system thrombosis in antiphospholipid syndrome (original) (raw)
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Diagnosis and classification of the antiphospholipid syndrome
The antiphospholipid syndrome (APS) is defined by the occurrence of venous and arterial thromboses, often multiple, and recurrent fetal losses, frequently accompanied by a moderate thrombocytopenia, in the presence of antiphospholipid antibodies (aPL). Some estimates indicate that the incidence of the APS is around 5 new cases per 100,000 persons per year and the prevalence around 40e50 cases per 100,000 persons. The aPL are positive in approximately 13% of patients with stroke, 11% with myocardial infarction, 9.5% of patients with deep vein thrombosis and 6% of patients with pregnancy morbidity. The original classification criteria for the APS were formulated at a workshop in Sapporo, Japan, in 1998, during the 8th International Congress on aPL. The Sapporo criteria, as they are often called, were revised at another workshop in Sydney, Australia, in 2004, during the 11th International Congress on aPL. At least one clinical (vascular thrombosis or pregnancy morbidity) and one laboratory (anticardiolipin antibodies, lupus anticoagulant or anti-b 2 -glycoprotein I antibodies) criterion had to be met for the classification of APS.
European Journal of Case Reports in Internal Medicine
Background: Antiphospholipid syndrome (APS) is defined as thromboembolic complications and/or pregnancy morbidity in the presence of persistent increased titres of antiphospholipid antibodies. Nevertheless, some patients with clinical signs suggestive of APS are negative for diagnostic antibodies and may be classified as having seronegative-APS (SN-APS). Among the 'non-diagnostic' antibodies, a few studies have suggested that the IgG anti-vimentin/cardiolipin antibodies (AVA/CL) may be associated with risk of thrombosis. Aims: The aim of this case report is to encourage the assessment of non-conventional antibodies in APS. Patient and methods: We report the case of a 69-year-old male patient with rapid onset of apparently unexplained multiple exclusively arterial thrombotic events in both coronary and peripheral vascular beds. Results: The patient did not meet the diagnostic criteria for APS but was positive for AVA/CL, which result persisted on further testing at 3 and 6 months. Discussion: Ongoing research has revealed the existence of non-criteria antibodies which may be relevant for the diagnosis of SNAPS and should be included in the classification criteria for the disease. LEARNING POINTS • In patients with unexplained multiple thrombosis without the conventional antibodies of antiphospholipid syndrome, the assessment of non-conventional antibodies should be considered. • IgG anti-vimentin/cardiolipin antibodies may be associated with risk of thrombosis. • Arterial thrombosis could be the only manifestation of antiphospholipid syndrome.
Annals of the Rheumatic Diseases, 2014
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...
Frontiers in Immunology, 2022
Background: Antiphospholipid syndrome (APS) is an acquired hypercoagulable condition associated with antiphospholipid antibody (aPL) presence. Data on rethrombosis following APS-diagnosis are limited. Methods: This is a retrospective analysis of new thrombotic events among primary APS (pAPS) patients followed for up to 15 years in three medical centers in Israel. Results: Among 312 primary-APS patients, 143 (46%) had new thrombotic event classified to three patterns: (1) Arterial-associated with heart valve disease (OR 7.24, 95% C.I. 2.26-24.6), hypertension (OR 3, 95% C.I. 1.44-6.25), elevated anti-B2-GPI IgM (OR 1.04, 95% C.I. 0.996-1.08), arterial thrombosis at presentation (OR 1.74 95% C.I. 0.992-3.26), and older age (41 vs. 34 years, p < 0.001). (2) Venous-linked with venous thrombosis at presentation (OR 12.9, 95% C.I. 5.27-31.6, p < 0.001), heart valve disease (OR 9.81 95% C.I. 1.82-52.9, p = 0.018), aGAPSS (OR 1.15 95% C.I. 1.02-1.29), and younger age (31 vs. 36.5 years, p = 0.001); and (3) Combined pattern-associated with heart valve disease (OR 40.5 95% C.I. 7.7-212) and pulmonary embolism (OR 7.47 95% C.I. 1.96-28.5). A 4th variant "the Breakthrough pattern" defined by re-thrombosis despite prophylactic therapy was observed in 100/143 (70%) patients and linked with heart valve disease (OR 8. 95% C.I. 2.43-26.3), venous thrombosis at presentation (OR 2.61 95% C.I. 1.47-4.66), leg ulcers (OR 12.2, 95% C.I. 1.4-107), hypertension (OR 1.99, 95% C.I. 0.92-4.34), and higher aGAPSS (OR 1.08, 95% C.I. 0.99-1.18). Conclusion: In this real-life observation, re-thrombosis was common among pAPS patients including in those recommended to receive prophylactic therapy. Different patterns of recurrence were identified and linked with presenting symptoms, specific serological markers, APS manifestations, and comorbidities. Studies that will address interventions to prevent recurrences of APS-related events are needed.
2011
Central nervous system involvement is one of the most prominent clinical manifestations of antiphospholipid syndrome (APS), and includes thrombotic events, psychiatric features and a variety of other non-thrombotic neurological syndromes. The aim of our prospective study was to investigate relationship between nonthrombotic neurological and cardiac manifestations. We analyzed 218 patients with primary (PAPS) and 115 patients with secondary APS (SAPS). Chorea, migraine and epilepsy occurred more frequently in SAPS, while headache and depression were more common in PAPS. Patients from both subgroups with unstable angina pectoris were more prone to TIA, epilepsy and transient global amnesia in PAPS and acute ischemic encephalopathy in SAPS. Patients with valve vegetations were more prone to epilepsy and depression. We revealed positive relationship between serum aCL IgG levels and incidence of acute ischemic encephalopathy in SAPS, aCL IgM and epilepsy in SAPS, aCL IgM and migraine in ...
Current Internal Medicine Research and Practice Surabaya Journal
Upper extremity deep vein thrombosis is less common than in the lower extremity site. Such conditions can appear primarily or secondary to other conditions such as thrombophilia. One of the unique forms of acquired autoimmune thrombophilia is antiphospholipid syndrome (APS). We describe a 25 years old female admitted with sudden swelling, redness, and pain in her left arm. Venous ultrasound confirmed the diagnosis of thrombosis in the left subclavian vein, left axillary vein, left proximal brachial vein, and left proximal basilic vein. The patient was known to have a spontaneous miscarriage in the second pregnancy at eight weeks of gestation. Screening for autoimmune and antibody phospholipid was done, and primary APS was confirmed. She has been treated with a subcutaneous injection of fondaparinux 2.5 mg for five days and oral rivaroxaban 15 mg twice daily for 21 days. But four months later, the patient came with a thrombus in the subclavian vein due to inadequate treatment, then l...
Antiphospholipid syndrome; its implication in cardiovascular diseases: a review
Journal of Cardiothoracic Surgery, 2010
Antiphospholipid syndrome (APLS) is a rare syndrome mainly characterized by several hyper-coagulable complications and therefore, implicated in the operated cardiac surgery patient. APLS comprises clinical features such as arterial or venous thromboses, valve disease, coronary artery disease, intracardiac thrombus formation, pulmonary hypertension and dilated cardiomyopathy. The most commonly affected valve is the mitral, followed by the aortic and tricuspid valve. For APLS diagnosis essential is the detection of so-called antiphospholipid antibodies (aPL) as anticardiolipin antibodies (aCL) or lupus anticoagulant (LA). Minor alterations in the anticoagulation, infection, and surgical stress may trigger widespread thrombosis. The incidence of thrombosis is highest during the following perioperative periods: preoperatively during the withdrawal of warfarin, postoperatively during the period of hypercoagulability despite warfarin or heparin therapy, or postoperatively before adequate anticoagulation achievement. Cardiac valvular pathology includes irregular thickening of the valve leaflets due to deposition of immune complexes that may lead to vegetations and valve dysfunction; a significant risk factor for stroke. Patients with APLS are at increased risk for thrombosis and adequate anticoagulation is of vital importance during cardiopulmonary bypass (CPB). A successful outcome requires multidisciplinary management in order to prevent thrombotic or bleeding complications and to manage perioperative anticoagulation. More work and reporting on anticoagulation management and adjuvant therapy in patients with APLS during extracorporeal circulation are necessary.