Classification criteria for antiphospholipid syndrome and its non-criterial manifestations.

Author(s):  
Т.М. Решетняк ◽  
Ф.А. Чельдиева

Вопросы по критериальным признакам антифосфолипидного синдрома (АФС), в частности о необходимости включения некоторых клинических проявлений, а также серологических маркёров, отличных от классических, остаются актуальными. В обзоре приведены предлагаемые профили некритериальных проявлений АФС, обсуждается необходимость пересмотра классификационных критериев АФС. Today questions about the antiphospholipid syndrome (APS) criteria remain actual. This applies in particular about the need to include some clinical manifestations, as well as serological markers that are distinguishable from those classic ones, in APS criteria. The review presents the proposed profiles of non-criterial APS manifestations, and discusses the need to revise APS classification criteria.

2021 ◽  
Vol 10 (6) ◽  
pp. 1240
Author(s):  
Takako Miyamae ◽  
Tomohiro Kawabe

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder mainly characterised by increased risks of thrombosis and pregnancy morbidity and persistent positive test results for antiphospholipid antibodies (aPLs). The criteria for diagnosing juvenile APS have yet to be validated, while the Sydney classification criteria do not contain several non-thrombotic clinical manifestations associated with the presence of aPLs. As such, difficulties have been encountered in the diagnosis of patients who have no certain thrombotic occlusions. Moreover, extra-criteria manifestations (i.e., clinical manifestations not listed in the classification criteria), including neurologic manifestations (chorea, myelitis and migraine), haematologic manifestations (thrombocytopenia and haemolytic anaemia), livedo reticularis, nephropathy and valvular heart disease have been reported, which suggests that the clinical spectrum of aPL-related manifestations extends beyond that indicated in the classification criteria. Studies have demonstrated that more than 40% of children with aPLs demonstrated non-thrombotic aPL-related clinical manifestations alone. Moreover, our results showed that the pathogenesis of non-criteria manifestations is characterised by “APS vasculopathy”. The present review introduces the characteristics and findings of non-criteria manifestations observed in juvenile APS.


2017 ◽  
Vol 44 (11) ◽  
pp. 1597-1602 ◽  
Author(s):  
Navid Zohoury ◽  
Maria Laura Bertolaccini ◽  
Jose Luis Rodriguez-Garcia ◽  
Zakera Shums ◽  
Oier Ateka–Barrutia ◽  
...  

Objective.Most clinicians use the 2006 Sydney classification criteria to evaluate patients suspected of having antiphospholipid syndrome (APS). Although sensitive and specific for APS, many patients fulfilling clinical criteria for the syndrome are persistently negative for the specific serological tests (“laboratory criteria”). These “seronegative APS” (SN-APS) patients can go undiagnosed and untreated until they experience serious clinical events. This study’s objective was to describe antibody profiles of SN-APS patients using non-criteria markers, assess the clinical utility of these markers separately and in combination, and suggest incorporation into guidelines for patients suspected of APS.Methods.We categorized 175 consecutive patients suspected of APS into 2 subgroups: 107 fulfilling Sydney APS classification for seropositive APS (SP-APS) and 68 with clinical manifestations suggestive of APS but having negative serology, on 2 occasions, for criteria markers (SN-APS). On study inclusion, samples were retested for criteria and 11 non-criteria markers, including antiphosphatidylserine/prothrombin antibodies.Results.Using 4 of 11 non-criteria tests, a cumulative 30.9% of SN-APS patients were detected. Combining results of all 11 non-criteria tests, 25 SN-APS (36.8%) and 89 SP-APS (83.2%) were positive for 1 or more non-criteria antibodies.Conclusion.Failure to diagnose APS can result in severe clinical consequences. Patients displaying clinical features of APS, but negative for conventional criteria markers, should undergo additional testing for non-criteria biomarkers. In our cohort, around one-third of SN-APS patients showed reactivity to 1 or more non-criteria markers. An update to the current classification criteria incorporating new serological markers should be considered to identify and stratify patients with APS for more effective treatment and management.


2019 ◽  
Vol 10 (1) ◽  
Author(s):  
Carmela Mazzoccoli ◽  
Domenico Comitangelo ◽  
Alessia D’Introno ◽  
Valeria Mastropierro ◽  
Carlo Sabbà ◽  
...  

Abstract Background Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis, and the detection of circulating antiphospholipid antibodies. The classification criteria for definite APS are actually met when at least one clinical criterion (thrombosis or pregnancy morbidity) is present in association of one laboratory criterion (LAC, aCL antibody or aβ2GPI antibody present on two or more occasions, at least 12 weeks a part), and thrombosis should be confirmed by objective validated criteria. The average age of primary APS patients has been reported to be about 35–40 years and the disease is more common in women than in men. Case presentation In this report, we described a rare case of an adult male who presented over a period of 9 years with a wide spectrum of clinical manifestations involving different organs that were not initially diagnosed as APS. Dizziness and syncope were his first clinical symptoms, and a non-bacterial thrombotic endocarditis (NBTE) involving the mitral valve was at first diagnosed. Subsequently, the patient also presented with generalized seizures and subsequent head injury. When the patient was admitted to our clinic with bilateral epistaxis and fever, thrombocytopenia was revealed. Moreover, laboratory examinations showed acute pancreatitis with an increase of levels of inflammation markers. Conclusion Based on the patient’s medical history and all the examination results, it was possible to make a diagnosis of primary APS and, starting from diagnosis of thrombocytopenia, we were allowed to conclude that all of manifestation were epi-phenomena of a unique clinical entity, rather than unrelated diseases. Though APS is one of the most common thrombocytophilias, unfortunately, it is not recognized often enough. The lack of prevention in undiagnosed patients may cause severe complications which can in turn result in the death of those patients.


2010 ◽  
Vol 30 (03) ◽  
pp. 139-143 ◽  
Author(s):  
G. M. A. van Os ◽  
R. T. Urbanus ◽  
C. Agar ◽  
J. C. M. Meijers ◽  
P. G. de Groot

SummaryThe antiphospholipid syndrome (APS) is a non-inflammatory autoimmune disease characterized by the presence of antiphospholipid antibodies (aPL) in the plasma of patients with vascular thrombosis, recurrent complications of pregnancy, or both (1, 2). The presence of aPL in plasma of patients can be detected with either a prolongation of phospholipid dependent coagulation tests (lupus anticoagulant, LAC), or with solid phase immune assays against the protein β2-glycoprotein I (β2-GPI) or the phospholipid cardiolipin (anti-β2-GPI antibody ELISA and anti-cardiolipin antibody ELISA, respectively) (3). For a long time there was a lot of confusion on who had the syndrome and who not. To solve this dispute, an international consensus meeting was organized in Sapporo in 1999 to formulate classification criteria for patients with the antiphospholipid syndrome (4). These criteria have been updated in 2004 at another international consensus meeting in Sydney (5). The classification criteria were defined for scientific purposes and were aimed to be used as inclusion criteria in patient related studies. They were specifically not defined for diagnostic purposes. However, current practice is that these criteria are used as a diagnostic tool. This is very unfortunate because the specificity of the different aPL assays to detect the clinical manifestations that characterize APS are disputable. One of the aims of defining the criteria was to initiate studies to determine the value of the different anti-phospholipid antibody assays to serve as biomarker for the risk of thrombosis and pregnancy morbidity. The recent progress made on this important topic will be discussed.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 602.1-603
Author(s):  
E. S. Torun ◽  
E. Bektaş ◽  
F. Kemik ◽  
M. Bektaş ◽  
C. Cetin ◽  
...  

Background:Recently developed EULAR/ACR classification criteria for systemic lupus erythematosus (SLE) have important differences compared to the 2012 Systemic Lupus International Collaborating Clinics (SLICC) SLE classification criteria and the revised 1997 American College of Rheumatology (ACR) criteria: The obligatory entry criterion of antinuclear antibody (ANA) positivity is introduced and a “weighted” approach is used1. Sensitivity and specificity of these three criteria have been debated and may vary in different populations and clinical settings.Objectives:We aim to compare the performances of three criteria sets/rules in a large cohort of patients and relevant diseased controls from a reference center with dedicated clinics for SLE and other autoimmune/inflammatory connective tissue diseases from Turkey.Methods:We reviewed the medical records of SLE patients and diseased controls for clinical and laboratory features relevant to all sets of criteria. Criteria sets/rules were analysed based on sensitivity, positive predictive value, specificity and negative predictive value, using clinical diagnosis with at least 6 months of follow-up as the gold standard. A subgroup analysis was performed in ANA positive patients for both SLE patients and diseased controls. SLE patients that did not fulfil 2012 SLICC criteria and 2019 EULAR/ACR criteria and diseased controls that fulfilled these criteria were evaluated.Results:A total of 392 SLE patients and 294 non-SLE diseased controls (48 undifferentiated connective tissue disease, 51 Sjögren’s syndrome, 43 idiopathic inflammatory myopathy, 50 systemic sclerosis, 52 primary antiphospholipid syndrome, 15 rheumatoid arthritis, 15 psoriatic arthritis and 20 ANCA associated vasculitis) were included into the study. Hundred and fourteen patients (16.6%) were ANA negative.Sensitivity was more than 90% for 2012 SLICC criteria and 2019 EULAR/ACR criteria and positive predictive value was more than 90% for all three criteria (Table 1). Specificity was the highest for 1997 ACR criteria. Negative predictive value was 76.9% for ACR criteria, 88.4% for SLICC criteria and 91.7% for EULAR/ACR criteria.In only ANA positive patients, sensitivity was 79.6% for 1997 ACR criteria, 92.2% for 2012 SLICC criteria and 96.1% for 2019 EULAR/ACR criteria. Specificity was 92.6% for ACR criteria, 87.8% for SLICC criteria 85.2% for EULAR/ACR criteria.Eleven clinically diagnosed SLE patients had insufficient number of items for both 2012 SLICC and 2019 EULAR/ACR criteria. Both criteria were fulfilled by 16 diseased controls: 9 with Sjögren’s syndrome, 5 with antiphospholipid syndrome, one with dermatomyositis and one with systemic sclerosis.Table 1.Sensitivity, positive predictive value, specificity and negative predictive value of 1997 ACR, 2012 SLICC and 2019 EULAR/ACR classification criteriaSLE (+)SLE (-)Sensitivity (%)Positive Predictive Value (%)Specificity (%)Negative Predictive Value (%)1997 ACR(+) 308(-) 841527978.695.494.976.92012 SLICC(+) 357(-) 352626891.193.291.288.42019 EULAR/ACR(+) 368(-) 242826693.892.990.591.7Conclusion:In this cohort, although all three criteria have sufficient specificity, sensitivity and negative predictive value of 1997 ACR criteria are the lowest. Overall, 2019 EULAR/ACR and 2012 SLICC criteria have a comparable performance, but if only ANA positive cases and controls are analysed, the specificity of both criteria decrease to less than 90%. Some SLE patients with a clinical diagnosis lacked sufficient number of criteria. Mostly, patients with Sjögren’s syndrome or antiphospholipid syndrome are prone to misclassification by both recent criteria.References:[1]Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Ann Rheum Dis 2019;78:1151-1159.Disclosure of Interests:None declared


2020 ◽  
Vol 2020 (12) ◽  
Author(s):  
Stathis Tsiakas ◽  
Chrysanthi Skalioti ◽  
Paraskevi Kotsi ◽  
Ioannis Boletis ◽  
Smaragdi Marinaki

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.


Author(s):  
Gunay Uludag ◽  
Neil Onghanseng ◽  
Anh N. T. Tran ◽  
Muhammad Hassan ◽  
Muhammad Sohail Halim ◽  
...  

AbstractAntiphospholipid syndrome (APS) is an autoimmune disorder associated with obstetrical complications, thrombotic complications involving both arteries and veins, and non-thrombotic manifestations affecting multiple other systems presenting in various clinical forms. Diagnosis requires the presence of antiphospholipid antibodies. The exact pathogenesis of APS is not fully known. However, it has recently been shown that activation of different types of cells by antiphospholipid antibodies plays an important role in thrombosis formation. Ocular involvement is one of the important clinical manifestations of APS and can vary in presentations. Therefore, as an ophthalmologist, it is crucial to be familiar with the ocular findings of APS to prevent further complications that can develop. Furthermore, the ongoing identification of new and specific factors contributing to the pathogenesis of APS may provide new therapeutic options in the management of the disease in the future.


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