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Immune thrombocytopenic purpura (ITP) is an immune-mediated disorder characterized by the destruction of platelets and megakaryocytes due to autoantibodies against the platelet surface proteins. ITP without any apparent cause of thrombocytopenia is defined as primary ITP, and ITP in the setting of SLE is secondary ITP, which can be diagnosed after excluding other causes of thrombocytopenia by history, physical examination, and laboratory testing. Patients with ITP associated with SLE have higher median platelet count and less bleeding manifestations compared to the patients with primary ITP. It can be very challenging to diagnose primary ITP in SLE patients as other causes of thrombocytopenia including drug-induced thrombocytopenia, antiphospholipid syndrome, and thrombotic microangiopathic process should be ruled out. Corticosteroids are the main modality of treatment. IVIG can be used in severe cases. Splenectomy was found to be less effective in ITP associated with SLE compared to primary ITP. Control of disease activity with immunosuppressive therapy can be helpful in some cases associated with active disease flares in SLE patients. [ABSTRACT FROM AUTHOR]

Antiphospholipid syndrome (APS) is an autoimmune disorder while adrenal hemorrhage could be its rare complication. Herein, we report the case of a 32-year-old unmarried woman with a history of systemic lupus erythematosus (SLE) who was hospitalized after complaints of upper abdominal pain, limb weakness, and loss of appetite for 2 weeks. Laboratory examination revealed hyponatremia, low plasma cortisol levels, increased adrenocorticotropic hormone levels, and a positive anticardiolipin antibody status. Furthermore, computed tomography (CT) revealed the presence of bilateral adrenal masses. Ultimately, based on dynamic changes in CT images, these masses were diagnosed as adrenal hemorrhage owing to APS. A computer-assisted literature search was conducted to identify cases of primary adrenal insufficiency associated with APS and/or SLE. The clinical features, laboratory examination, treatments, and outcomes of these cases were summarized. Our findings emphasize the importance of screening for adrenal insufficiency in patients with SLE or APS who present with abdominal complaints, asthenia, and hyponatremia. It is also recommended to test for APS all patients with adrenal hemorrhage. [ABSTRACT FROM AUTHOR]

Administering neuraxial anesthesia to a patient with an underlying neurological disease and a combination of four other pathological disorders can be challenging. We report in this paper the case of a 45-year-old woman with neurological deficit due to ischemic brain infarct, multiple sclerosis, antiphospholipid syndrome, and ß-heterozygous thalassemia that was subjected to abdominal hysterectomy and bilateral salpingoophorectomy under epidural anesthesia for ovarian cancer. [ABSTRACT FROM AUTHOR]

Thyroid disorders are commonly associated with coagulopathy. Patients with hyperthyroidism have increased risk for developing thromboembolic accidents, which are favoured by a simultaneous presence of antiphospholipid antibodies syndrome. in this paper, we describe the case of a patient with Graves' disease, who developed strokes with antiphospholipid antibodies syndrome. [ABSTRACT FROM AUTHOR]

Background: The detection of antiphospholipid antibodies (aPL) is of interest because of their importance in the pathogenesis of arterial or venous thrombosis. They could be a "second hit" of an inflammatory event such as infection. The aim of our study was to assess the performance of antiphospholipid antibody biomarker to predict in-hospital mortality in intensive care unit (ICU) septic patients.Methods: We conducted a prospective single-center observational study including consecutive critically ill septic adults admitted to the intensive care unit. Clinical and laboratory data including enzyme-linked immunosorbent assay for antiphospholipid antibodies (anticardiolipin (aCL), antiphosphatidylserine (aPS)) were obtained. Blood samples were collected on days 1, 3, 5, 8, and 10 of hospitalization. The primary study endpoint was ICU mortality defined as death before ICU discharge. Secondary end points included correlation between SOFA score and biological parameters.Results: A total of 53 patients were enrolled. 18.8% of patients were aPL positive. In-hospital mortality rate was 60%. Multivariate analysis showed that age and aCL at days 3 and 5 along with SOFA at day 3 were independent outcome predictors. A significant positive correlation existed between SOFA at days 3, 5, and 8 and antiphospholipid antibody concentrations.Conclusions: Our data showed that antiphospholipid was useful biomarkers for the prediction of mortality in critically ill septic patients. We found a positive correlation between SOFA score and antiphospholipid antibodies. [ABSTRACT FROM AUTHOR]

Catastrophic antiphospholipid syndrome (CAPS) is an infrequent but feared life-threatening complication of antiphospholipid syndrome (APS). CAPS is characterized by the rapid development of numerous thromboses across multiple organs resulting in multiorgan failure. It is rare but well-documented in the adult population. In contrast, it is exceedingly uncommon in pediatric patients and therefore not yet well described in the pediatric literature. Early recognition of APS is of the utmost importance to provide timely and effective management for a positive outcome. We present the case of an 11-year-old girl with history of systemic lupus erythematosus (SLE) and hypertension (HTN) who presented with acute onset altered mental status, found to have a large ischemic middle cerebral artery (MCA) and anterior cerebral artery (ACA) stroke as well as multiple, diffuse, and smaller ischemic lesions in the frontal lobe and cerebellum. Her presentation was further complicated by thrombocytopenia and renal and splenic infarction, as well as thrombosis of the right brachial vein consistent with a diagnosis of CAPS. [ABSTRACT FROM AUTHOR]

Objective: Antiphospholipid syndrome (APS) is a chronic autoimmune disease with a high prevalence in females. Published data have identified pregnant women with APS may suffer from recurrent miscarriage, fetal death. However, the association between antiphospholipid antibody (aPL) and fetal growth restriction (FGR) remains controversial. This study aims to systematically review the literature on population-based studies investigating an association between aPL and FGR.Methods: The literature was searched on 1 November, 2021, using Ovid MEDLINE, Embase, and Cochrane Central Register of Controlled Trials (CENTRAL), following the MOOSE checklist. Study inclusion criteria focused on peer-reviewed published articles that reported an association between aPL and FGR. Quality assessment was performed based on the Newcastle-Ottawa scale. The between-study heterogeneity was assessed by the Q test. Publication bias was assessed by funnel plots.Results: Twenty-two studies (with 11745 pregnant women) were included in the final analysis. Pooled odds ratio for association of aPL, anticardiolipin antibodies (ACA), anti-beta2 glycoprotein 1 antibodies (β2GP1), and FGR was 1.26 (95%CI 1.12, 1.40), 2.25 (95%CI 1.55, 2.94), and 1.31 (95% CI 1.12, 1.49), respectively. Lupus anticoagulant (LA) did not increase the chance of FGR (OR 0.82, 95%CI 0.54, 1.10).Conclusions: Our meta-analysis showed that aPL increased the risk of FGR. The risk of FGR varies with the aPL types. ACA and β2GP1 are strongly associated with FGR. There are currently insufficient data to support a significant relationship between LA and FGR. [ABSTRACT FROM AUTHOR]

Vascular disease, either as a direct complication of the disease or developing as an accompanying comorbidity impairs significantly the quality of life of patients with SLE and represents the most frequent cause of death in established lupus. This paper aims to give an overview of the prevalence of the different forms of vasculopathy that can be encountered in a lupus patient, describe their pathogenesis, and address their impact on disease severity and outcome. [ABSTRACT FROM AUTHOR]

Antiphospholipid syndrome (APS) is characterized by thrombosis. This systemic review and meta-analysis was to verify the hypothesis that APS might increase the risk of stroke. Studies were identified after literature searching of PubMed, Embase, and Cochrane. Newcastle-Ottawa Quality Assessment Scale Cohort Studies (NOQAS-C) was used to assess the quality of studies. The pooled effect with 95% confidence interval (95% CI) was calculated by random-effect model. I -square ( I 2 ) was used to test heterogeneity. Funnel plot was used to evaluate publication bias. A total of 17 cohort studies with overall high quality were included. There was no publication bias. Pooled hazard ratio of stroke occurrence in APS patients was 1.76 (1.39-2.21) with low heterogenicity and stable result from sensitivity analysis. In the analysis of subgroups, pooled risk ratios of stroke occurrence in patients with only positive antibodies of APS diagnosis were 1.75 (0.99-3.09), which for the APS patients with other autoimmune diseases were 14.70 (7.56-28.56). APS might be a risk factor of stroke, especially in patients with other autoimmune diseases. [ABSTRACT FROM AUTHOR]

In order to diagnose patients with pregnancy and antiphospholipid antibody syndrome, provide early treatment, and effectively reduce the pregnancy outcome of the abnormal pregnancy, the effect of antiphospholipid syndrome (APS) immunotherapy on the incidence of abortion was discussed based on clustering algorithm. We selected 62 cases of APS leading to recurrent miscarriage patients, in the early pregnancy injection of low molecular heparin, intravenous drip proprocyclin, and oral tactics, using B-ultrasound images to observe the pregnancy ending. The results show that the hormone levels in the two groups were different before treatment (P > 0.05); after treatment, the HCG, E2, and P hormone levels in the two groups were significantly improved, and the HCG, E2, and P hormone levels in the observation group were significantly higher than those of the control group (P < 0.05); the abortion rate of patients in the observation group was significantly lower than that of the control group (P < 0.05); the antiphosphorus antibody of the study group was significantly higher than that of the control group. For APS patients, immunotherapy is effective. Antiphospholipid syndrome causes remarkable immunotherapy effect in patients with recurrent miscarriage, effectively improves the clinical symptoms of patients, improves antiprochemical antibody rosion, and improves the patient's pregnancy outcomes, which is worth promoting. [ABSTRACT FROM AUTHOR]

Recurrent pregnancy loss (RPL) commonly refers to three or more miscarriages that occur before 20 weeks of pregnancy. The immunological cause of RPL could be either an auto- or alloimmune-related event or both. Because of the discovery of immunological abnormalities in RPL patients in clinical practice, several immunomodulatory therapies were introduced to maintain the immune balance at the maternal-fetal interface. Intravenous immunoglobulin (IVIg) is one of the immunomodulators. In recent years, several studies have analyzed the therapeutic effect of IVIg on RPL patients with antiphospholipid syndrome (APS) or unexplained RPL. However, their results are controversial. IVIg can be used in RPL patients with APS who have previously failed in other treatments. It is recommended that IVIg infusion could be considered used before conception in RPL patients who have cellular immune abnormalities such as increased natural killer (NK) cell counts, NK cell cytotoxicity, or increased T helper (Th)1/Th2 ratio, depending on the cut-off values of each hospital. The aim of this review was to summarize the mechanisms, efficacy, pharmacokinetics, and side effects associated with passive immunization using IVIg in immunologic RPL, according to the literature published in recent years. We hope that more obstetricians will be able to understand the timing and indication of IVIg properly in immunologic RPL patients and effectively enhance pregnancy outcomes for mothers and neonates. [ABSTRACT FROM AUTHOR]

Background. Heparin-induced thrombocytopenia (HIT) is a transient, antibody-mediated thrombocytopenia syndrome that usually follows exposure to unfractioned heparin (UFH) or low-molecular-weight heparin (LMWH). In contrast to other pathological conditions which lead to thrombocytopenia and bleeding complications, HIT results in a paradoxical prothrombotic state. It is caused by antibodies directed to complexes containing UFH or LMWH and a self-platelet protein: the platelet factor 4 (PF4). The heparin-PF4 immune complex leads to activation of platelets, monocytes, and endothelial cells which release procoagulant proteins and tissue factor with subsequent blood coagulation activation. Case Report. We describe the case of a woman undergone to knee replacement and affected by urosepsis who developed a HIT after exposure to enoxaparin. The thrombotic burden was very impressive involving the arterial and venous cerebral vessel and the venous pulmonary, hepatic, and inferior legs vascular beds. The patient was successfully treated with fondaparinux without recurrent thrombosis or bleeding. The clinical scenario could be named "catastrophic HIT" like the catastrophic antiphospholipid syndrome since they have a similar pathogenetic mechanism involving both platelets and monocytes procoagulant activities and a similar clinical manifestation with a life-threatening multiple arterial and/or venous thromboses. Conclusion. Patients presenting with HIT could show a very impressive thrombotic burden resembling to that of the catastrophic antiphospholipid syndrome. A careful differential diagnosis should be made towards other pathological conditions which lead to thrombocytopenia to avoid an unnecessary and potentially harmful platelet transfusion. Although fondaparinux is off-label, its use in patients with HIT is simple and seems to be effective. [ABSTRACT FROM AUTHOR]

Cardiac disease is a well-known complication of antiphospholipid syndrome (APS), with many patients presenting with valvular thickening or vegetations, referred to as Libman–Sacks endocarditis (LSE). Because cases of APS with cardiac involvement are relatively rare, paucity of large clinical trials studying this complication has made management challenging. In the absence of acute heart failure and embolic events, a medical approach is usually selected, consisting of anticoagulation and possibly corticosteroids when another underlying autoimmune disease is present. However, the role of various anticoagulant classes and the duration of steroid therapy continue to be debated. Here, we present a 45-year-old woman who developed two vegetations in the setting of secondary APS while taking rivaroxaban before experiencing marked improvement with the use of enoxaparin and steroids. [ABSTRACT FROM AUTHOR]

High titer of anti-β2-glycoprotein I antibodies (anti-β2GPI Ab) plays a pathogenic role in antiphospholipid syndrome (APS). Numerous studies have focused on the pathological mechanism in APS; however, little attention is paid to the immune mechanism of production of anti-β2GPI antibodies in APS. Our previous study demonstrated that Toll-like receptor 4 (TLR4) plays a vital role in the maturation of bone marrow-derived dendritic cells (BMDCs) from the mice immunized with human β2-glycoprotein I (β2GPI). TLR4 is required for the activation of B cells and the production of autoantibody in mice treated with β2GPI. However, TLR4 provides a third signal for B cell activation and then promotes B cells better receiving signals from both B cell antigen receptor (BCR) and CD40, thus promoting B cell activation, surface molecules expression, anti-β2GPI Ab production, and cytokines secretion and making B cell functioning like an antigen presenting cell (APC). At the same time, TLR4 also promotes B cells producing antibodies by upregulating the expression of B-cell activating factor (BAFF). In this paper, we aim to review the functions of TLR4 in B cell immune response and antibody production in autoimmune disease APS and try to find a new way for the prevention and treatment of APS. [ABSTRACT FROM AUTHOR]

Objective: To investigate disease activity around and during pregnancy and pregnancy outcome in women with systemic lupus erythematosus (SLE) considering antiphospholipid antibody status. Moreover, differences between first and consecutive pregnancies were examined.Methods: Pregnancies > 16 weeks gestation of SLE patients receiving joint care from rheumatologists and gynecologists in two tertiary centers in the Netherlands between 2000 and 2015 were included. Disease activity, flare rate, and pregnancy outcomes and complications were assessed.Results: Ninety-six women (84% Caucasian) with 144 pregnancies were included. The median SLE(P)DAI score was 2 before, during, and after pregnancy. Flare rates were 6.3%, 20.1%, and 15.3%, respectively. Severe hypertensive disorder of pregnancy, intrauterine fetal death, preterm birth, and small-for-gestational age infants occurred in 18.1%, 4.1%, 32.7%, and 14.8%, respectively. Complication rates were similar in the first and consecutive pregnancies. Half of the women did not experience any pregnancy complication whereas 42.7% developed a complication during all pregnancies. Mean number of pregnancies was 2.4 and live births 1.7.Conclusion: In this SLE population with low disease activity, pregnancy complications were present irrespective of antiphospholipid antibody status. Furthermore, there were no differences in complication rates between the first and consecutive pregnancies as seen in healthy mothers. This information is useful for patient counseling. [ABSTRACT FROM AUTHOR]

Adrenal incidentalomas (AIs) are mostly benign and nonsecretory. Management algorithms lack sensitivity when assessing malignant potential, although functional status is easier to assess. We present a subject whose AI was a retroperitoneal leiomyosarcoma (RL). Case Presentation. A woman on warfarin with SLE and the antiphospholipid syndrome, presented with left loin pain. She was normotensive and clinically normal. Ultrasound scans demonstrated left kidney scarring, but CT scans revealed an AI. MRI scans later confirmed the AI without significant fat and no interval growth. Cortisol after 1 mg dexamethasone, urinary free cortisol and catecholamines, plasma aldosterone renin ratio, and 17-hydroxyprogesterone were within the reference range. Initially, adrenal haemorrhage was diagnosed because of warfarin therapy and the acute presentation. However, she underwent adrenalectomy because of interval growth of the AI. Histology confirmed an RL. The patient received adjuvant radiotherapy. Discussion. Our subject presented with an NSAI. However, we highlight the following: (a) the diagnosis of adrenal haemorrhage in this anticoagulated woman was revised because of interval growth; (b) the tumour, an RL, was relatively small at diagnosis; (c) this subject has survived well over 60 months despite an RL perhaps because of her acute presentation and early diagnosis of a small localised tumour. [ABSTRACT FROM AUTHOR]

Objectives. To evaluate the frequency of seizures in primary antiphospholipid syndrome (PAPS) and their possible clinical and laboratory associations. Methods. Eighty-eight PAPS patients (Sydney's criteria) were analyzed by a standard interview, physical examination and review of medical charts. Risk factors for seizures, clinical manifestations, associated comorbidities, and antiphospholipid antibodies were evaluated. Results. Nine (10.2%) patients with seizures were identified, 77.8% had convulsions onset after PAPS diagnosis.Mean age, gender, and race were comparable in groups with or without seizures. Interestingly, a higher frequency of current smoking (44.4 versus 10.1%, P = 0.019) was observed in the first group. Stroke, Sneddon's syndrome, and livedo reticularis were more frequent in PAPS patients with seizures than those without seizures, although not statistically significant (P > 0.05). Comparison between patients with seizures onset after PAPS diagnosis (n = 7) and those without convulsions (n = 79) demonstrated a higher frequency of current smoking (42.9 versus 10%, P = 0.042) and stroke in the first group (71.4 versus 30.4%, P = 0.041). Regression analysis confirmed that smoking (P = 0.030) and stroke (P = 0.042) were independently associated to seizures. Conclusion. About 10.2% of PAPS patients had convulsions, predominantly after PAPS diagnosis, and seizures were associated to current smoking and stroke. [ABSTRACT FROM AUTHOR]

Thrombosis is a well-known clinical entity in systemic lupus erythematosus (SLE), and it is multifactorial. The most important risk factor is the presence of antiphospholipid antibodies (APLAs). However, approximately 40% of adults with SLE who are negative for APL A are diagnosed with thrombosis, indicating the importance of other risk factors. Thus, the thrombosis risk factors should be evaluated extensively and regularly and treated aggressively in every patient with systemic lupus erythematosus. [ABSTRACT FROM AUTHOR]

Cardiovascular diseases are common in systemic rheumatologic diseases. They can be presented at the time of diagnosis or after diagnosis. The cardiac involvements can be the first presentation of rheumatologic conditions. It means that a patient with rheumatologic disease may go to a cardiologist when attacked by this disease at first. These manifestations are very different and involve different structures of the heart, and they can cause mortality and morbidity of patients with rheumatologic diseases. Cardiac involvements in these patients vary from subclinical to severe manifestations. They may need aggressive immunosuppressive therapy. The diagnosis of these conditions is very important for choosing the best treatment. Premature atherosclerosis and ischemic heart disease are increased in rheumatoid arthritis and systemic lupus erythematosus, and may be causes of mortality among them. The aggressive control of systemic inflammation in these diseases can reduce the risk of cardiovascular disease especially ischemic heart disease. Although aggressive treatment of primary rheumatologic diseases can decrease mortality rate and improve them, at this time, there are no specific guidelines and recommendations, to include aggressive control and prevention of traditional risk factors, for them. [ABSTRACT FROM AUTHOR]

In order to establish if neuropsychiatric systemic lupus erythematosus (NPSLE) can be identified by any characteristic other than those used to diagnose the neuropsychiatric (NP) disease itself, we retrospectively reviewed 98 systemic lupus erythematosus (SLE) patients followed over a mean period of 10 years. NPSLE was identified in 22 patients. Stroke and generalized seizures were the most frequent NP manifestations. The NPSLE and non-NPSLE groups were similar with regard to demographic characteristics, ACR criteria, serum autoantibodies, and frequency of hypertension and hypercholesterolemia. Of note, compared to the non- NPSLE group, NPSLE was associated with a higher frequency of smoking (78 versus 26%), organ damage (73 versus 34%), and cumulative mortality rate (14 versus 7%). The series of patients was further analysed according to the presence of antiphospholipid syndrome (APS). Significantly, the interval between the onset of NP disease and SLE diagnosis was shorter in the APS- (0.3 ± 1 years) than in the APS+ (5±7 years) groups. Recurrence and/or persistence of NP events were only documented in the APS- group. Overall cumulative mortality was highest in NPSLE and in APS+ patients with inadequate anticoagulation control, identifying an aspect that requires improved vigilance and the development of novel therapeutic modalities. [ABSTRACT FROM AUTHOR]

We report the case of a 24-year-old nondiabetic, nonhypertensive lady with history of fatigue, dyspnoea and limb claudication. She has been diagnosed with Takayasu’s arteritis. Subsequently she developed rash, alopecia, joint pain, and various other laboratory abnormalities which led to a diagnosis of SLE. Takayasu’s arteritis (TA) rarely coexists with systemic lupus erythematosus (SLE). The absence of specific SLE markers in patients with TA who subsequently develop SLE suggests that the coexistence of these conditions may be coincidental. The antiphospholipid syndrome in patients with SLE may mimic the occlusive vasculitis of TA. [ABSTRACT FROM AUTHOR]

The antiphospholipid antibody syndrome (APS) is defined by the persistent presence of antiphospholipid antibodies in patients with recurrent venous or arterial thromboembolism or pregnancy morbidity. Antithrombotic therapy is the mainstay of treatment given the high risk of recurrent thromboembolism that characterizes this condition. Despite the prothrombotic nature of APS, thrombocytopenia is present in a proportion of patients, which can complicate management and limit the use of antithrombotic therapy. The mechanism of APS-associated thrombocytopenia is multifactorial and its relation to thrombotic risk is poorly characterized. The presence of thrombocytopenia does not appear to reduce thrombotic risk in patients with APS, who can develop thromboembolic complications necessitating antithrombotic treatment. In these cases, treatment of the thrombocytopenia may be necessary to facilitate administration of antithrombotic agents. We present such a pregnant lady with history of recurrent pregnancy losses who presented with severe thrombocytopenia and bleeding manifestations, who was subsequently diagnosed to have antiphospholipid antibody syndrome. She was initially managed with steroids and when her platelet counts improved, antithrombotic therapy was started. She delivered an uneventful and successful pregnancy outcome without any complications during follow-up. [ABSTRACT FROM AUTHOR]

We report the case of a male patient with limited cutaneous systemic sclerosis (SSc) that was complicated with severe digital ischemia, resistant to medical treatment. Due to the lack of treatment response, further laboratory and imaging studies were conducted. Findings were compatible with antiphospholipid syndrome and oral warfarin was added to the treatment regimen. After successful anticoagulation no further recurrences of digital ischemia were seen. An underlying etiology in SSc patients with treatment resistant digital ischemic necrosis should be suspected for accompanying antiphospholipid syndrome (APS). [ABSTRACT FROM AUTHOR]

The case of an elderly patient who had chorea as an initial symptom of systemic lupus erythematosus (SLE) accompanied by antiphospholipid syndrome (APS) is reported. A 68-year-old woman suddenly developed chorea of her left arm and leg. Magnetic resonance imaging (MRI) of the brain demonstrated a focal lesion in the right caudate head, which showed hyperintensity on fluid-attenuated inversion recovery and diffusion-weighted imaging. This condition was thought to be a common form of vascular chorea, which is likely to occur in elderly individuals; however, the laboratory data of this patient finally fulfilled the diagnostic criteria of SLE and APS. Physicians should be careful in diagnosing elderly individuals simply as having a vascular chorea because this symptom can be the initial manifestation of SLE or APS. [ABSTRACT FROM AUTHOR]