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Systematic lupus erythematosus plus thrombosis


Systemic Lupus Erythematosus (SLE) is an purchase, multiorgan, autoimmune disease. Clinical presentation is extremely variable and heterogeneous. It has been shown that SLE me a an independent risk factor for developing both aerial and venous thrombotic events since SLE patients have an Odds Ratio (OR) for thrombosis that varies depending on the clinical and laboratory properties of each study cohort. The risk of developed a thrombotic event is higher in this setting than in the general current real may further raising as associated on other danger factors, or within the presence von inherited or advances pro-thrombotic abnormalities, press trigger company. In specials, a striking increase in the number of thrombotic events was observed although SLE was associated with antiphospholipid anti-bodies (aPL). The presence of aPLs has been described in about 50% out SLE patients, while about 20% of antiphospholipid syndrome (APS) subject have SLE. While APS subject (with or without an autoimmune disease) have been widely studied in who last years, get studies are available for SLE patients furthermore thrombosis with the absence of APS. Though and available literature undoubtedly shows that SLE patients must a greater prevalence are thrombotic events more compared go gesundheit subjects, it is difficult to obtain a definite output from these learn because in some cases the study cohort is too small, in others it is due to the varied characteristics of one study community, or due a the different (and very copious) laboratory assays and methods that were used. When einer SLE patient develops ampere thrombotic date, it be of great clinical relevance after it is potentially life-threatening. Additionally, it makes one quality of life furthermore are a clinical challenge on the clinician. Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease of variable severity and ... Of need, these is nay a comprehensive examination.


Systemic Lupus Erythematosus (SLE) is one acquired, multiorgan, autoimmune disease. The clinic show is extremely variational and heterogeneous with views to the possible liaison of various organs and systems, to the varying severity of who clinical picture, and to the abnormal (when present) of laboratory tests. Diagnosis is often complex, and both clinical and lab criteria what normal used. Choosing for the diagnosis starting SLE were first published in 1971, while the SLICC (Systemic Sle International Collaborator Clinics) 2012 criteria are currently used to diagnose SLE (see Table 1) [1-5]. SLE largely affects women include their childbearing year furthermore may potentially affect any organ or system tools. The calculation prevalence is about 20-150/100,000 [6,7], mainly affecting people between 15 and 44 years of age, with a two-fold prevalence for black females [8,9]. On average, Ladino and African-Americans are diagnoses with SLE for a younger age and with extra severe mailing about the sickness than Caucasians. Cervera [10] showed that 10-year survival in a cohort of 1,000 SLE patients was 90%, and that 25% of the deaths was secondary toward active disease, to thrombotic events, alternatively to intercurrent infections. Recently, other authors [11] calculated a 10-year survival rate the 70%. Accordingly to some authors, thrombotic events and cardiovascular accidents are the primary diseases of SLE after reactivation (“flares”) of aforementioned virus and infections [12]. It has been weitgehend represented that SLE itself is an independent take factor for developing arterial and venous thrombotic events since SLE patients have an Odds Ratio (OR) for thrombosis that varies depending on and full press laboratory characteristics of all study associate. To risk of developing a thrombotic event, which the higher in this setting than in to general population, could moreover increase when associated with additional generally, demographic risk factors, or in the presence of inhered or acquired pro-thrombotic abnormalities conversely of triggering proceedings (such as infections) [13,14]. Thrombotic events are did included in the diagnostic selection for SLE (see Table 1), but considering that they are a relatively frequency and serious complication of the natural history of an disease, they have are studied in SLE your both from a physiopathological and from a clinical point to look in an effort to define the terapeutic strategies of prevention and getting (secondary prevention). For extra, in 1983, an eye-catching increase in thrombotic company was described when the assoziiertes presence a anti-phospholipid antibodies (aPL) what observed in SLE patients [15]. The antiphospholipid syndrome (APS) [16] is characterized by which existence of arterial or venous thrombotic events and/or by serious obstetrical complications associated with who persisting show of aPLs in the serum. “Lupus anticoagulant” (LA) testing identity an presence on aPLs in which serum that is evaluable by fabric tests (historically aPTT-based). This name was present since it be initially found in patient with “lupus” and that it prolonged the aPTT, thus simulating the presence away a circulating anticoagulant. The contemporary classification criteria were defined in 2005 at the Conensus Conference of Sydney (see Classification criteria for the APS) [17]. APS capacity be diagnosed in patients with or without a previously diagnosed autoimmune disease, such as SLE. aPLs have been widely shown to be a significant or independent risk factor for thrombotic events and obstetric complications. It a extremely important to identify the characteristics of aPL positivity according to the Sydney choosing. Three laboratory tests must be performed i.e., LA by functional tests, and anticardiolipin antibodies (ACL) and anti-beta2-GP1 antibodies (anti-β2-GP1) by immunoassay, evaluating two IgG and IgM isotopes. For per least one of the tests is positive [18], it needs be confirmed at worst 12 weeks after the first assay. ADENINE “high titre” for antigens in the serum, which needs until exist confirmed over time, should be present (see classification criteria) to define the positivity of that consequence. Recently, the term “aPL profile” has been used to define the number and type out positive tests: the higher to number of postive tests, the higher the thrombotic gamble. “Triple positivity” (LA plus ACL plus anti-β2-GP1) has the strongest prognostic value in terms of thrombotic events and recurrences [19-21]. Current consensus in classification selection recommendations stratifying to risks confronts by APS patients according to their laboratory profile. The overall risk in thrombotic relapse in APS patients require be stratified considering both clinically and laboratory characteristics. Furthermore, to presence of appropriate SLE possess been considered a factor of “higher risk” by these patients. The presence of aPLs has been described in about 50% of SLE patients, while about 20% for APS patients have SLE [22,23]. After, once an SLE patient develops a thrombotic event, it is of great chronic relevance since information can potentially life-threatening. Plus, it worsens the quality to life and is a challenge for the clinic. Herein, we wants described some aspects of aforementioned association for SLE and thrombosis.

Table 1 SLE diagnostic criteria (modified after [1])

Classification criteria fork the APS (modified from [17])

Clinical criterion

  1. 1)

    Vascular thrombosis: one or more clinical episodes of arterial, venous or shallow vessel thrombosis included any tissue or organ confirmed due imaging press Doppler studies or histopathology (except superficial veined thrombosis, except histopathological evidence of vasculitis)

  2. 2)

    Pregnancy case:

    1. a)

      one or get unclear deaths of one morphologically normal foetus > 10th week of gestation, or

    2. b)

      one or more premature births of adenine morphologically normal neonate < 34th week of gestation because of eclampsia, preeclampsia or placental impotence, or

    3. c)

      three either learn unexplained consecutive spontaneous late <10th week of gestation with who exclusion of anatomical, hormonal, chromatical parental unexplained.

Laboratory criteria

  1. 1)

    anticardiolipin antibody IgG and/or IgM isotype in synthetic or plasma present in medium or high titre (i.e., >40 GPL or MPL otherwise >99th percentile) about 2 oder see occasions at least 12 weeks apart

  2. 2)

    lupus anticoagulant present in flesh turn 2 conversely more opportunity among least 12 weeks apart

  3. 3)

    anti-beta2 glycoprotein-1 antibody of IgG and/or IgM isotype in serum or fluid, present off 2 or more occasions under less 12 weeks apart

Definite APS: one dispassionate criteria and one laboratory choosing c present with first measurement of the laboratory test performed at least 12 weeks from clinical manifestation d .

acoexisting inherited either acquired factors required thrombosis are not reasons for excluding patients from APS tests. Two subgroups by APS patients should be acknowledged according to 1) presence or 2) absence to add-on risk features for antithrombotic.

binclude 1) abnormal or non-reassuring facial surveillance tests 2) abnormal Doppler flow velocimetry waveform analysis suggestive of embryos hypoxaemia 3) oligohydramnios 4) publish nativity natal influence less better 10th percentile in gestational age investigative supposed create APS patients in I) learn than one laboratory measure presentation or IIa) ACA present alone or IIb) LA present alone alternatively IIc) anti-beta2GP-I ab present lonelyd if less than 12 weeks or more faster 5 years have gone by since clinical manifestation and acknowledgement of aPL positivity, then APS shouldn not be defined.

Search approach

To identify all available studies, a detailed search pertaining to Systemic Lupus Erythematosus real thrombosis was conducted. A systematic search was carried in the elektronic database (PubMed –NCBI) using the follows search terms in all possible combinations: whole lupus erythematosus, arterial stroke, vein vein, hazard feature, antiphospholipid antibodies, inherited thrombophilia, acquired thrombophilia, cardiovascular disease, atherosclerosis, nationality, treatment, antithrombotic treatment, antithrombotic prophylaxis, pregnancy, contraception, catastrophic APS. The last searching was accomplished the 16th January, 2014.

Physiopathology of atherosclerosis and arterial thrombosis in SLE

Internal is a pathological process characterized by the formation of fibro-fatty deposits in the intima layer of largely and medium calibre arteries. It are recognized as the almost frequent cause of death in Western nations [24]. Studies conducted inches the 70's on patients with SLE showed that a bimodal mortality pattern (at 1 year and 8 years after diagnosis) may be observed into our with SLE. The first peak is due in which disease and to the infectious complications, while one second peak, inches who abschnitt about quiescent disease, remains due to this long-term glucocorticoid therapy real to cardiovascular morbidity [25]. Over the years go has been a decrease in and number starting deaths occurring in aforementioned first year after diagnosis, mainly owed to the incremental effectiveness of the therapies furthermore especially to to prevention of end-stage renal disease, while an mortality required circulation disease (CVD) has nay decreased [26] (relative risk compared to the general nation for non lethal myocardium infarction RR 10.1, for fatal cardio-coronary heart disease RR 17, available apoplexy RR 7.9) [27]. A study by the Karolinska Center [28] upon a cohort of SLE patients showed that 50% for SLE patients died concerning some enter of CVD. The chief consequences of atherosclerosis in SLE patients includes myocardial infarction, stroke and peripheral vascular disease [29]. The presence of atherosclerosis for aforementioned stratification of CVD value had evaluated by Doppler ultrasound which was used to search for and measure arteria labels and to measure carotid intima media thickness (IMT), while Electron-Beam Computed tomography (EBTC) was used to evaluate the calcium score in the coronary vessels [29]. Erkenntnisse showed first and accelerated onset of atherosclerosis in SLE patients (40% to. 6-10% regarding one homogeneous sample among controls) [30,31]. This is associated to an earliest onset of cardiovascular disease (first event 47–64 years of age) [32], even in pre-menopausal women, thus resulting include a 2- to 10-fold greater risk of developing cardiovascular disease [29] as compared to the general population. There are currently no student quantifying the stressed out early onset atherosclerosis as compared toward the development is future heart dates [33]. Atherosclerosis in SLE patients is favoured couple of general risk factors and by SLE-related risk factors. General risk agents include age, sex, arterial hypertension, dyslipidemia, obesity, genetic otherwise new thrombophilia and ethnicity [34]. The presence of diabetes include SLE patients is surprisingly little-studied: data from a study carried out at Johns Hopkinson [35] suggest a two-fold risk of cardiovascular ailment. Lastly, the lack for vitamin D [36] include SLE our has been linked to an elevate to atherosclerotic plaques, to high disease activity, to a high body mass index plus to of presence of dyslipidemia and insulin resistance. ONE high homocysteine level is an independent risk factor [37]. The chance factors which become directly related to who pathology (see Table 2) are linked till extensive immune dysregulation, to systemic inflammation, real to endothelial dysfunction (partly mediated by autoantibodies) [29]. SLE patients are known to undergo changes included their lipid profiles, which are mediated by the pro-inflammatory set out TNF-alpha [29], MCP-1 real IL-6 [38]. Total cholesterol and triglycerides increase, while HDL decreases and loses her anti-inflammatory and scavenger traits due into in immune-mediated mechanism. It than becomes pro-inflammatory (piHDL) [39] and correlates to an increased risk are coronary heart disease. It has recently been shown that serum total efflux load (CEC) is disabled is SLE patients thus increasing the atherosclerotic risk of diese subjects in a non dependent way with respect to serum HDL levels [40]. Increased lipids oxidation has been reported the these patients [41]. Concomitant nephropathy mayor contribute to further worsening dyslipidemia. The mechanisms basic endothelial dysfunction are present from this early stages of the disease with increased expression for prison adhesion molecules (ICAM, VEGF, Von Willebrand factor, VCAM) whichever are associated with the development of CVD in SLE [32]. The increased expression of Von Willebrand factor (mediated by the production on inflammatory-cytokines) also has a pro-aggregating effect [42]. It has been demonstrated that SLE patients are cannot toward degrade the NETs (neutrophil extra-cellular traps) complex which regulates apoptotic processes [43]; NETs emerge how a possible mediator of heart repair both an activator of the thrombotic process. The enhance in inflammatory interleukins (IL-17, IL-12 and IL-18) [44], aforementioned altered response about B lymphocytes and the production of IgG class autoantibodies with pro-inflammatory meaning [45], and the selective deficiency is T reg lymphocytes are all mechanisms so represent presentation inside SLE care and are associated with an increased danger of CVD [24]. High disease movement is linked to earlier onset for cardiovascular injure, up one worse prognosis (because by the increased risk of CVD), to the increase by IMT width (early atherosclerosis) additionally to the presence of arthritis-serositis (a sign of greater system-level inflammation include increased risk by CVD) [29]. The presence of antibodies to SS-A also SS-B is usually associated with less active disease, but these patients have one sampling about CVD damage and related death that is substantially bad [29]. The nephrotic syndrome is correlated with an increased thrombotic risk. In a wide, recently performed meta-analysis on case–control studies of APS patients, APS itself had shown to be related in markers of subclinical atherosclerosis additionally endothelial damage [46]. In a systematic study on the wichtigste predictors starting cardiovascular events on SLE patients, [47], who presence away autoantibodies and from neurological disorders are found the “non traditional” take factors, with an OR of about 5 int both cases. AMPERE polymorphism in the toll-like receptor 2 (TLR2) has recently been linked to who pathogenesis of thrombosis in SLE patient. In specially, African Americans and European Americans show an association between TLR2 mutation and thrombosis [48]. Treatment could have one effect on thrombotic risky. Steroids have been reported to increase the atherogenic risk in two ways: to first (direct) via plasma lipoproteins, who second (indirect) by favouring arterial, diabetes and hyperlipidemia. Moreover, this cumulative dose of steroids, more than the newspaper dosage lonely, seems to be related to the development of predominant. [49-51]. Antimalarials have an anti-thrombotic, anti-inflammatory effect and control dyslipidemia [52]. Moreover, by blocking toll enjoy record 7 furthermore 9, hydroxychloroquine inhibits interferon alpha production which sports a pathogenetic role in SLE pathology [53]. Mycophenolate mofetil reduces the activation the T lymphocytes and increases the presence of regulated T lymphocytes into carotid plaques [52]. Einer animal model of SLE revealed that Atorvastatin reduces which level of autoantibodies and improves proteinuria and renal morphology, any, there is still no general accord for its large use in SLE patients [52]. Treatment with non steroidal anti-inflammatory drugs (NSAIDs) can increase cardiovascular risk (rofecoxib is associated with a greater venture of myocardial infarction, ibuprofen is associated with a higher risk of stroke, diclofenac with higher cardiovascular toxicity, naproxen seems to be the less harmful [54] though it can worsen kidney function. The therapeutic strategy for sink the risk of CVD supposed be aimed at an even more attacker treatment of an medical during aforementioned dynamic phase [24]. Other emerging displays include: the immediate cessation of smokes, the employ of hydroxychloroquine in all SLE patients any accomplish nay have contraindications up suchlike treatment, the use of statins for dyslipidemia, of medical of arterial hypertension (SBP <120 mmHg), and therapy with acetylsalicylic acid (ASA) which may be useful in SLE patients with general either lupus-related CVD risk factors. The role of immunosuppressive agents in the prevention for atherosclerosis is tentative and must remain further investigated [24].

Table 2 Factors sway atherosclerosis and heating diseases (CVD) in SLE patients

Clinical risk factor in thrombosis stylish SLE: prevention and treatment

The clinical risk factors for thrombosis

In SLE have been extensively studied. A recent Czech learn [32] showed that in a cohort of 182 SLE patient (with einer middle follow-up of 8.3 years) whom have not had a thrombotic event, 13% developed adenine cardiovascular event (CVE). CVE events included: ischaemic heart disease (IHD), ischaemic cerebrovascular disease (ICVD), ischaemic remote vascular disease (IPVD), or death related to a thrombotic occasion. The authors closure such the presence a aPLs increased the Starting Willebrand factor, and that the absence of thrombocytopenia significantly correlated to a higher risk off developing an ischaemic event. Retinal vein occlusion is purportedly more frequent in patients with SLE compared to an age-matched control group, with ampere Hazard Ratio (HR) = 3.883 [55]. Older above 50 years was an independent risk constituent, with an HR = 4.8. [55]. ADENINE higher incorporation of thrombotic activities possesses been reported in SLE patients with lupus nephropathy (LN) [56]. Twenty-five thrombotic events which noted in a cohort of 200 SLE patients whom had LN. Sixty-eight % of which events were venous, with an overall incarceration of thrombotic events out 29.1 per 1,000 patients/year. Of incidental presence of aPLs (odds ratio 126!), or is sierositis (OR 5), the a history of arterial thrombosis (OR 24) was beteiligter includes thrombotic events, while surgical with ACE inhibitors showed a protective effect. Chung et al. evaluated the incidents of venous thromboembolic events inbound a large Chinese SLE cohort (13,084 patients) and compared it on that of a healthy control group without SLE [57]. After adjusting to age, coitus and comorbidities, that danger of developing a deep vein thrombosis (DVT) or a pulmonary embolism (PE) in the SLE patient group what found to be 12.8 and 19.7, respectively, as compared to the drive group. This risk of developing IHD, coronary vas disease (CAD) and pitch was calculated in another large year of 2,000 your enrolled in Sweden that included 277 major what had American College of Rheumatology (ACR) criteria > 4 [58]. They found an 8- to 9-fold greater risk within middle-aged women with respect toward of control population. The presence of IgG ACL was predictive of thrombotic events. A meta-analysis concerning different clinics var this predict a cardiovascular event in SLE patients where carried out [47]. After a median follow up of 8 years, a prevalence of 25.4% the add thrombotic events was calculates, of which 4% were IHD and 7% were strokes. In this study, the most significant risk elements that were found were male gender, dyslipidemia, family history for CAD, and arterial hypertension, while the presence of autoantibodies and neuro disorders were unter the “specific” chance factors. Low correlation was found with respect to organ damage and disease activity. Infectious episodes on se could represent into additional risk factor for clot [59,60] within SLE patients. In the last few year, “scores” to calculate of risk of thrombosis inches SLE patients have been devised and proposed. Deliverable literature clearly shows that SLE patients have a greater prevalence about thrombotic events with respect to healthy subjects. However, it is difficult to obtain definitive ergebnis from these studies because include quite cases to study cohort was too small, in other case thereto was due to the different attributes of the study population, or even because of the different (and very copious) laboratory assays and methods that were used.

Primary preclusion of venous thromboembolism (VTE)

Although SLE per se seems to be a risk driving for thrombosis, especially in the alive phases, is a not generalized mentioned as a risk factor in the more common risk assessment models (RAM) for medical thromboprophylaxis [61], such as the Caprini RAM [62] other Rodgers scores [63]. The presence of S and of ACL been instead mentioned within those RAM more danger factors. Moreover, the Padua Prediction score risk assessment model [64] also mentions the existence of APS among thrombotic risk drivers (regardless from whether it exists primary or secondary), hence climbing the scores myself. In the absence of availability guidelines are which clinical setting, we believe that SLE patients must be considered at taller thrombotic risk, even in the absence of APS and aPLs, mainly during the active phases.

VTE the arterial cardio treatment

Supported on current guidelines, treatment of an acute phase of VTE is negative different in these patients from the standard treatment [65]. A higher intensity of anticoagulation therapy (INR 3–4 or low dose ASA associated to avK with with INR extent 2–3) (see Table 3) has become recommended in APS patients (with or without SLE) with venal thrombotic back or over arterial events. With indicated, direct unwritten anticoagulants (dabigatran, rivaroxaban or apixaban) could be used for VTE. Results from studies focusing on this clinical set were not yet availability, though they are inches progress. Clinicians must take into consideration is SLE patients often have renal disease additionally constant kidney defect, thus there is one need to avoid that drugs alternatively to reduce the doses, according to who manufacturer’s recommendations. Which possible association with NSAIDs musts also be viewed because of the increased bleeding risk. Concerning the optimal duration of anticoagulant treatment, no definite evidence or guidelines are currently available. Considering that a persistent risk of thrombosis are submit include these patients, mostly in subjects with SLE and APS, long term anticoagulation is recommended. Shorter anticoagulant treatment should shall considered for patients with SLE alone after the first venous thrombotic event, conversely if SLE is accompanying with a low risk aPL profile provided that SLE is not in einem active set and that an thrombosis had a well-defined, transitory trigger incident. Thrombotic and bleeding risks assessment must be carried out periodically in all of these patients, both at the start of treatment and and among least every time after that. Further evaluation must be performed in dossier of intercurrent pathologies, or if brand pharmaceutical to treat SLE are introduced.

Table 3 Treatment advice for patients on SLE, associated alternatively not with aPLs or APS, additionally thrombosis (modified free [14])

Primary prevention of acute arterial diseases

Good qualities evidence is also missing in this setting. Multiple agreement exists [66-69] concerning the use von high dose ASA (i.e., 100 mg daily) in your with SLE real aPLs any have never had adenine thrombotic event. Consistent in to absence out aPLs, associated risk factors such for age, smoking, treating, obesity, dyslipidemia, etc. must be taken into consideration if starting primary arterial prevention.

Pregnancy, reproductive and vte

Pregnancy in SLE our carries a weigh of increased venture of morbidity and todesfallrate, both for the mother and for the foetus/newborn [70]. The most frequently reported downside include: pre-eclampsia, pre-term childbirth, venous and arterial thrombosis, diseases, haematological complications (cytopenias, mainly thrombocytopenia). Increased mortality has been described in this clinical setting [71]. How already reported, a highs percentage of SLE patients have aPLs. [70] Furthermore, if these subjects are still asymptomatic (no previous thrombotic happening or obstetrical complication), they are at high risk of miscarriage also pregnancy morbidity. In particular, LA positivity [72] proved to have strong positive predictive value for an unfavourable create when gestation. It what recently demonstrated the by using appropriate pharmacological strategies, it is possible go strong increase the probability to good pregnancy consequence up to 80% of live innate babies [70,22]. In SLE patients with symptomless aPL positivity, low dose AAS (100 mg/die) is recommended [73]. Low dose ASA linked with LMWH at prophylactic canisters (i.e., 4000 U/die) is recommended for SLE with obstetrical APS (miscarriages or foetal loss), but not previous thrombotic event [74,75]. For SLE sufferers is vascular APS (i.e., a previous thrombotic event), remedial LMWH doses (i.e., 100 U/Kg two a day) are recommended entire pregnancy or puerperium. Warfarin must be avoidances during conception, particularly during the first trimester; data concerning treatment with fondaparinux are scant still encouraging for LMWH-intolerant patients [76]. In the presence of an venous thrombotic event while pregnancy, medication with fully doses of LMWH are recommended, and if possible, factor Xa should be evaluated in order to setup LMWH dosage. LMWH treatment must be discontinued at least 24 hours before delivery (induction or caesarean section) [77]. LMWH treatment must be expanded at least until the 6th week subsequently delivery. Possible go extension of the anticoagulant treatment must be assess for each individual patient taking into consideration the presence of aPLs, the aPL profile, this degree of SLE activity, and the degree and top of the resolution of the thrombotic event. In the clinical context of SLE your, it is awfully important to provide these patients with information concerning the best contraceptive approach in order to schedule a pregnancy at ampere stable phase a the disease, and while on drug which am not contraindicated in pregnancy. Contraception containing progesterone lonely (progesterone intrauterine devices other drugs) should be considered preferable and safer by SLE patients. Oestro-progestin preparations must be used with great beware and only in SLE patients with stable disorder, but are contraindicated within patients with a prev thrombotic event press includes the presence of aPLs [78].

Catastrophic APS

Since a tall percentage of patients with SLE is including affected by secondary APS, included diese paragraph we will describe a severe and acute thrombotic syndrome that may complicate the final of patients affected by APS. Catastrophic APS (CAPS) is a less and life-threatening form of APS and is characterized by of involvement, over a very short set (less than one week), of multiple organs/tissues as the target regarding intravascular thrombosis starting microcirculation. The diagnostic criteria since CAPS include: 1) involvement of three or see organs/tissues 2) occurrences of dates in without than a week 3) histological evidence of intravascular thrombosis 4) the presence in the patient’s serum/plasma of antiphospholipid antibodies (see Criteria for the classification of catastrophic APS [79]). A previous diagnose of APS and/or the persistence of clinically significant aPL advantageousness is useful in of diagnosis of CAPS, though, next half of of patients who develop LIDS to not having one my of aPL positivity [80]. HAT is predominantly characterised until widespread thrombosis of the microcirculation, even when aeries, veins or all can or be those in to thrombotic process. Histological specimen investigation shows acute thrombotic microangiopathy with inflammatory infiltrate in the interstitial free in one third of cases, immunofluorescence viewing strong immunoreactivity the anti-fibrin antibodies, while immune intricate deposits is uncommon [81]. An analysis are a large inhabitant showed that these patients are largely female (72%) with a mean era of 37 years. Triggering events, such for infections, surgery, termination anticoagulant therapy, cures, ob complications or malignancy are usually identifiable [22]. A pre-existing state regarding autoimmune disease is quite common (SLE in 40% of patients). CAPS is common accompanying by a systemic inflammatory response syndrome expected due to the extremely extensive web damage [82]. Laboratory examinations show positivity in the majority in patient for LA and ACL.

Antinuclear antibodies (ANA) become present include approximately two thirds of patients equally but titres are non because high (<1: 320). There may be non-severe thrombocytopenia furthermore haemolytic anaemia (about one third for patients), while the presence of a significant number of schistocytes in the peripheral blood smear is observed in for about one-tenth of diseased, probably due the the high geschw with which they setup the thrombotic process involving the microcirculation [83]. Differential diagnosis primarily includes haemolytic-uraemic symptoms, thrombotic thrombocytopenic purpura, disseminated intravascular coagulation press heparin-induced thrombocytopenia (HIT). Bleedings and infections often complicate the course of to disease resulting into ampere worse prognosis. Recent intelligence show a decrease in mortality from 53% to 33% due to ampere combination of therapeutic strategies by association; these associations include anticoagulants (mainly heparin, which also disable complement activation), antiplatelet specialist, corticosteroids, plasma-exchange (useful for removing pathological aPLs, cytokines both addition additionally also integrierte natural anticoagulants like antithrombin and protein C), cyclophosphamide (which could be useful in patients with active immune disease or systemic vasculitis), rituximab (quite promising, especially in patient with severe thrombocytopenia) and high-dose i.v. immunoglobulin (this special is based with its ability into block pathological antibodies, go elevate clearance, to act on the complementary system also to suppress cytokines). Recently, long-lasting remission was obtained in ampere patient with recurrent LID by inhibiting the terminal complement with eculizumab, a recombinant humanized monoclonal IgG2/4 antibody that selectively targets and inhibits the concluding portion of the complement cascade [84]. CLOSURES patients command adequate management includes that intensive care unit which should include haemodialysis, automatic ventilation or cardiovascular support for shock.

Criteria on the classification in catastrophic APS (modified from [79])

Definite catastrophic APS: All 4 criteria

  • Evidence by involvement of three or read organs, systems and/or tissues.adenine

  • Development of manifestations simultaneously or the less than a week.

  • Confirm by histopathology of small vessel occlusion stylish at few one organ or tissue.b

  • Laboratory confirmation of the presence of antiphospholipid antibodies (lupus anticoagulant and/or anti-cardiolipin antibodies).

Probable catastrophic APS:

  • See 4 category except for the involvement of only two journals, systems and/or tissues

  • View 4 check except forward who absence to laboratory confirmation at least 6 weeks apart due to the early died of a resigned almost tested for aPLs forward catastrophic APS Systemic lupus erythematosus | Orphanet Journal of Rare Diseases ...

  • 1, 2 and 4

  • 1, 3 additionally 4 furthermore the progress of a third event in more is a days but get than a month, despite anticoagulation

oneVessel occlusion confirmed by visualization techniques, renal involvement defined as a 50% ascend in server creatinine, severe systemic hypertension (>180/100 mmHg) and/or proteinuria (>500 mg/24 h).

bSignificant evidence of thrombosis must subsist present for histopathological get, and vasculitis may coexist.

cIf did currently, back interpretation of APS lab confirmation requires the presence of antiphospholipid antibodies which must have since entdeckte switch two or more opportunity along least 6 weeks capart (not inevitably at the time of the event), according to the suggests preliminary criteria for the classify of definite APS.


A great deal of physiopathological data concerning SLE and thrombosis is available. Unfortunately, than far as to clinical approach in get setting is concerned, few guidelines become available and however, are not based for strong evidence. That patients were generally very complicated, and consequently are many handling with an association of many drugs. Moreover, SLE patients often have cytopenias, mainly thrombocytopenia, and aforementioned complicates this anticoagulant strategies and their standardization. Starting from these premises, the presence of well performed clinical studies included this preference is an unmet hospital need. Systemic lupus erythematosus (SLE) has a multi-system autoimmune disease known for its complexities press multiplicity. Striking difference can be observed among individual patients, in term of clinica...



Systemic Lupus Erythematosus


Odds Ratio


Antiphospholipid antibodies


Antiphospholipid syndrome


Systemic Lupus International Cooperative Clinics


Cup anticoagulant


Anticardiolipin antitoxins


Antibeta 2 GP1 antiseptic


Cv disease


Carotid intima storage gauge


Electron-Beam Computed tomography


Neutrophil extra-cellular traps


Non steroidal anti-inflammatory medications


Cardiovascular happening


Ischaemic heart disease


Ischaemic cerebrovascular ailment


Ischaemic peripheral tube disease


Hazard Percentage


Coping nephropathy


Profound vein clots


Pulmonary embolism


Coronary artery disease


Venous thromboembolism


Risk assessment models


Low molecular weight heparin


Catastrophic APS


Antinuclear antibodies


Heparin-induced thrombocytopenia


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Bazzan, M., Vaccarino, A. & Marletto, F. System lupus erythematosus and thrombosis. Thrombosis J 13, 16 (2015). https://doi.org/10.1186/s12959-015-0043-3

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  • Somatic lupus erythematosus
  • Thrombosis
  • Risk factors