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Catastrophic antiphospholipid syndrome (CAPS) is an unusual complication of antiphospholipid syndrome (APS) occurring in about 1% of patients. If left untreated, mortality can be as high as 50%. Therapy of APS and its complication CAPS is hampered by the lack of validated prospective, controlled, intervention clinical trials, although there is consensus that treatment should include anticoagulation therapy. But there are issues that need to be addressed such as duration and intensity of therapy. The present report describes our experience in 7 patients with CAPS in whom anticoagulation was discontinued after 6 months of therapy. During an average follow-up of 5.5 years, only 2 patients exhibited one episode each of recurrent venous thrombosis, but none of the patients in whom anticoagulation was discontinued experienced recurrent CAPS.Key Points• Discontinuation of long-term anticoagulation therapy in CAPS patients was not followed by recurrence of CAPS.

Background: Antiphospholipid syndrome is an autoimmune disorder characterized by recurrent vascular thrombosis and pregnancy losses in the presence of persistently positive antiphospholipid antibodies. Bibliometric analysis and altmetric analysis are methods of analyzing academic productivity and influence. Currently, the assessment of antiphospholipid syndrome through the above analyses is lacking. This study investigated the quantity and quality of studies in the field of antiphospholipid syndrome and revealed the characteristics of worldwide productivity on this disease by the bibliometric analysis and altmetric analysis. Methods: The terms "APS," "antiphospholipid syndrome," "antiphospholipid-antibody syndrome," and "Hughes syndrome" were searched on the Web of Science Core Collection from January 1, 2011, to December 31, 2021. Original articles and reviews were selected. We set the filters as "English." Results and conclusion: A total of 1818 articles were retrieved from 68 countries, of which 20 met the criteria of major active countries. High-income countries contributed 1341 articles (73.48%). The number of articles annually increased significantly in the 10-year period (P < 0.001). The USA (253, 13.91%) was the most productive country. Adjusted by population, Serbia was top of the list. According to the gross domestic product analysis, Serbia ranked first. The most used keywords such as thrombosis and antiphospholipid antibodies were presented by keywords analyses. A content analysis found antithrombotic and anticoagulant therapy as research hotspots. A significant correlation was detected between average citations and altmetric attention scores (P = 0.002) and Mendeley readers count (P < 0.001). From 2011 to 2021, the number of global articles increased rapidly. Most papers came from high-income countries. The relationship between the bibliometric and altmetric analyses were basically consistent; therefore the two can prove/complement each other. Key points • We revealed the global productivity characteristics of the papers related to antiphospholipid syndrome by using the methods of bibliometric analysis and altmetric analysis. • We found the most selected articles that describe the treatment of antiphospholipid syndrome, especially antithrombotic and anticoagulant treatments, which may be the current research hotspot. [ABSTRACT FROM AUTHOR]

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by the presence of antiphospholipid antibodies in patients with arterial or venous thrombosis or pregnancy complications. This paper reports a case of a 31-year-old woman who died after she underwent C-section for intrauterine fetal death (IUFD) at the 25th week of gestation. The patient was complaining of pelvic pressure, swelling in the lower limbs, and pain in the groin, one big toe, and both wrists. She had low platelet count, liver abnormalities, and proteinuria. After IUFD, she complained of flank pain and headache. After discharge from the hospital, the patient had constant headaches and 5 days later woke up with hemiplegia. CT scan showed cerebral hemorrhage in the right hemisphere and thrombosis in the left hemisphere. The LA and APS tests were positive. The main cause of death was hemorrhage and infarction in the brain., (© 2021. International League of Associations for Rheumatology (ILAR).)

Antiphospholipid syndrome (APS) is a cause of infertility and fetal loss. Ovarian stimulation can induce previously unknown APS. Ovarian hyperstimulation syndrome (OHS) is uncommon but potentially life-threatening, as well as catastrophic APS. A woman that simultaneously developed a severe OHS and a catastrophic APS is described in this paper. Both entities produced thrombotic cardiac and brain thrombosis. A peculiar mechanism of cardiac ischemia is also described. In spite of the life-threatening risk of this situation, the indication for preventive anti-aggregation and/or anticoagulation is not clear.

The association of thrombotic microangiopathy (TMA) with systemic lupus erythematosus (SLE) has been described in 0.5 to 10% of cases, and patients present worse outcome. TMA is described as the association of microangiopathic hemolytic anemia, thrombocytopenia, and an organ injury, frequently the kidney. This study describes a successful case of use of eculizumab in a patient with SLE and TMA refractory to standard therapy, and provides a literature review. Case description and search in PubMed and MEDLINE using systemic lupus erythemathous and/or antiphospholipid syndrome (APS) and eculizumab retrieved 15 case reports. Eighteen-year-old female presented acute renal failure and TMA and was diagnosed with SLE. Steroids and IV cyclophosphamide were started together with plasma exchange. After 55 days, she still persisted with microangiopathic anemia, thrombocytopenia, and anuria, and eculizumab was introduced. She had rapid improvement in hematological parameters, and dialysis was discontinued 25 days after the first dose. Genetic analysis showed large heterozygous deletion encompassing the entire CFHR1 and CFHR3, a finding previously associated with patients presenting atypical hemolytic-uremic syndrome (aHUS). Twenty patients who received eculizumab with SLE and/or APS have been published to date: 11 were female and mean age at presentation was 31 years. Seven out of the 20 patients presented only SLE, 5 patients only APS and 8 patients both SLE and APS. Eighteen patients underwent plasma exchange, with a mean of 20 (4-120) sessions per patient. Thirteen patients received rituximab. Hematological response was evident in 100% and kidney recovery in 85% of patients. The terminal complement blockade with eculizumab is an optional treatment for patients with SLE and/or APS presenting TMA and refractory to current immunosuppression therapies. Genetic testing may help recognize patients with aHUS and SLE/APS and therefore help to determine length of treatment with eculizumab. [ABSTRACT FROM AUTHOR]

Introduction: Besides distinctive respiratory and digestive hallmarks, COVID-19 has been recently associated with a high prevalence of pro-inflammatory and hypercoagulable states known as "COVID-19 Associated Coagulopathy" (CAC), corresponding to a worsening in patients' conditions, whose causes are still to be elucidated. A link between anti-phospholipid antibodies (aPLs) and viral infections has long been suggested. APLs are assessed for anti-phospholipid syndrome (APS) diagnosis, characterized by thrombocytopenia, thrombosis, and coagulopathy. Furthermore, circulating immune complexes (CICs), arisen upon inflammatory responses and related immune dysregulation, can lead to endothelial cell damage and thrombotic complications. Method: We performed an extended panel including IgG/IgM anti-cardiolipin, IgG/IgM anti-β2-glycoprotein-1, coupled with IgG/IgM anti-prothrombin, IgG/IgM anti-annexin-V on two COVID-19 patient groups (early and late infection time), and a negative control group. IgG CIC analysis followed to evaluate inflammatory status, through a possible complement system activation. Results: Our results showed low positive case percentage in IgG/IgM anti-cardiolipin and IgG/IgM anti-β2-glycoprotein-1 assays (4.54%, 6.25%, and 4.55%; in early infection group, late infection group, and control group, respectively); few positive cases in IgG/IgM anti-prothrombin and IgG/IgM anti-annexin-V immunoassays; and no IgG CIC positivity in any patient. Conclusions: In conclusion, our data show a low aPL prevalence, likely excluding an involvement in the pathogenesis of CAC. Interestingly, IgG/IgM anti-prothrombin and anti-annexin-V positive cases, detected in late infection group, suggest that aPLs could temporarily increase or could trigger a "COVID-19-induced-APS-like-syndrome" in predisposed patients. Key Points •To our knowledge, anti-prothrombin (aPT) antibodies, anti-annexin-V antibodies and CICs in COVID-19 patients have not been reported in the scientific literature. •Lack of uniformity and the low percentage of aCL/aβ2GP1 positivity preclude a putative role in CAC pathogenesis. •IgG/IgM anti-prothrombin and IgG/IgM anti-annexin-V data show that distribution of positive case number increases in late infection patients, significantly in anti-annexin-V results, suggesting a possible role for these anti-phospholipid antibodies in disease course. •aPLs can arise transiently in some patients with critical illness and SARS-CoV-2 infection (disappearing in a few weeks), as well as in other genetically predisposed patients; they could trigger a "COVID-19-induced-APS-like-syndrome". [ABSTRACT FROM AUTHOR]

To perform a review on patients with primary antiphospholipid syndrome (APS) who developed an aneurysm. A review of articles published in PubMed/MEDLINE, LILACS, and SciELO dating from 1966 to October 2020 was conducted using the following search words: "Antiphospholipid syndrome" and "aneurysm." No language limitation was applied. This review includes 10 articles on APS patients and aneurysms entailing 14, including our additional case. Age varied from 20 to 76 years old, and female sex was predominant and presented in 54%. The arterial vessels compromised were aorta (n = 6), coronary (n = 2), hepatic (n = 2), renal (n = 2), middle cerebral artery (n = 2), and then splenic, superior mesenteric, pancreatic, retinal, jejunal, carotid, and pulmonary. Frequencies of antiphospholipid antibodies were described as follows: lupus anticoagulant (n = 6), anti-beta2-glycoprotein I (n = 4), IgG and IgM anticardiolipin (n = 3), IgG anticardiolipin (n = 3), and antiphosphatidylserine (n = 2). The presence of APS manifestations was distributed as deep venous thrombosis (n = 4), recurrent abortions (n = 4), pulmonary embolism (n = 3), stroke, or transitory ischemic accident (n = 3), and then limb ischemia and thrombocytopenia. The therapies used in these patients were warfarin, antiplatelet agent, vascular surgery, heparin, hydroxychloroquine, coil embolism, and intravenous immunoglobulin. Regarding outcomes, 5 patients were alive, and 3 were dead. This article reviewed all published cases on APS and aneurysm and showed that women who presented with abortions and deep venous thromboses with a lupus anticoagulant are those patients more commonly affected by aneurysms in APS. [ABSTRACT FROM AUTHOR]

Thrombotic events are the most frequent causes of death in patients with antiphospholipid syndrome (APS). Previous studies have reported infection to be the most important trigger of thrombosis in APS, with molecular mimicry considered to be a major mechanism. Although timely management of infections has been recommended in patients with high suspicion of infection, anti-infective therapy would not take effect in a short time due to the dilemma in determining the origins of infection, especially in patients undergoing immunosuppressive therapy. Here, we describe a 26-year-old patient with systemic lupus erythematosus with triple antiphospholipid antibody positivity who had a stroke involving her dorsolateral medulla, despite timely anti-infective treatment within the context of skin infection caused by Stenotrophomonas maltophilia. To the best of our knowledge, it is the first report about the association between Stenotrophomonas maltophilia infection and thrombotic complications in APS. Thus, solely focusing on anti-infective therapy by the current recommendation for the management of APS may be insufficient within the context of infection; early initiation of effective anticoagulation should also be suggested until the anti-infective therapy becomes effective, especially in patients with high-risk antiphospholipid antibody profiles, in whom the potential benefit would outweigh the risk of bleeding. [ABSTRACT FROM AUTHOR]

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by arterial, venous, and/or small vessel thrombosis, pregnancy morbidity, and persistently elevated levels of antiphospholipid antibodies (aPL). Cardiovascular disease (CVD) in APS can present as heart valvular disease (HVD), macro-micro-coronary artery disease (CAD), myocardial dysfunction, cardiac thrombi, or pulmonary hypertension. Brain disease presents as stroke or transient ischemic attack (TIA) and less frequently as cerebral venous thrombosis, seizures, cognitive dysfunction, multiple sclerosis (MS)−like syndrome, or chorea. Infarcts and focal white matter hyperenhancement are the commonest brain (MRI) abnormalities, while myocardial ischemia/fibrosis, valvular stenosis/regurgitation, or cardiac thrombi are the main abnormalities detected by cardiovascular magnetic resonance. This review aims to present the existing evidence on brain/heart involvement and their interrelationship in APS and the role of brain/heart MRI in their evaluation. Embolic brain disease, due to HVD, CAD, and/or cardiac thrombus, or brain hypo-perfusion, due to myocardial dysfunction, are among the main brain/heart interactions in APS and they are considered determinants of morbidity and mortality. Currently, there is no evidence to support the use of combined brain/heart MRI in asymptomatic APS patients. Until more data will be available, this approach may be considered in APS patients at high risk for CVD/stroke, such as systemic lupus erythematosus with high-risk aPL profile or high scores in CVD risk prediction models; APS patients with HVD/thrombus, CAD, or heart failure; those with classic and non-criteria neurologic APS manifestations (seizures, cognitive dysfunction, MS-like syndrome); or with aggressive multi-organ disease. Key Points • Cardiovascular disease (CVD) in antiphospholipid syndrome (APS) can present as heart valvular disease (HVD), macro-micro-coronary artery disease (CAD), myocardial dysfunction, cardiac thrombi, or pulmonary hypertension. • Brain disease presents as stroke or transient ischemic attack (TIA), and less frequently as cerebral venous thrombosis, seizures, cognitive dysfunction, and multiple sclerosis (MS). • A combined brain/heart MRI may be considered in APS patients at high risk for CVD/stroke, such as systemic lupus erythematosus with high-risk aPL profile or high scores in CVD risks; APS patients with HVD/thrombus, CAD, or heart failure; those with classic and non-criteria neurologic APS manifestations (seizures, cognitive dysfunction, MS-like syndrome); or with aggressive multi-organ disease. [ABSTRACT FROM AUTHOR]

Objectives: Antiphospholipid syndrome (APS) is an autoimmune disease characterized by obstetric complications and thrombotic events associated with antiphospholipid antibodies (aPL). We aimed to compare the clinical characteristics and treatment of primary APS (PAPS) and secondary APS (systemic lupus erythematosus-APS, SAPS) patients and investigate risk factors associated with obstetric complications in Shanghai, China. Methods: We retrospectively collected and analyzed the data of obstetric APS (OAPS) patients from 2000 to 2017 in the APS-Shanghai (APS-SH) database. Results: One hundred eighty OAPS patients with a total of 450 pregnancies were included in this study. Two hundred twenty-one (49.11%) pregnancies resulted in miscarriage, and 161 (35.77%) pregnancies resulted in intrauterine death. In our cohort, when women were treated, 57 out of 66 pregnancies resulted in live births (86%). Of the 9 treated patients who failed to have live births, 3 had intrauterine deaths, 3 had fetal growth restriction, 2 had pneumorrhagia of the newborn, and 1 had a miscarriage. OAPS patients were divided into two groups: PAPS and SAPS. More SAPS patients than PAPS patients used glucocorticoids (GCs) and hydroxychloroquine (both p < 0.001). However, there was no significant difference in the GC dosage between SAPS and PAPS patients (p = 0.188). Lupus anticoagulant (LAC) and IgG aβ2GPI were risk factors for miscarriage (odds ratio (OR) = 2.398, 95% confidence interval (CI) = 1.276–4.505, p = 0.002; OR = 2.907, 95% CI = 1.558–5.405, p = 0.001, respectively) and intrauterine death (OR = 2.439, 95% CI = 1.299–4.580, p = 0.006; OR = 2.060, 95% CI = 1.089–3.897, p = 0.026, respectively). Conclusions: The live birth rate of OAPS patients in Shanghai was 86%. Even if OAPS patients were treated, pregnancy complications could occur, and these patients might need further second-line treatment. Key Points • This is the first study to report data on Chinese OAPS patients. The live birth rate was 86%. • Lupus anticoagulant and IgG aβ2GPI were risk factors for miscarriage and intrauterine death in our cohort. • Despite active treatment, 9 patients had obstetric complications. Therefore, further second-line treatment is still needed. [ABSTRACT FROM AUTHOR]

Vascular antiphospholipid syndrome (VAPS) and obstetric (OAPS) are different entities because some patients only develop thrombosis (without recurrent pregnancy losses) and vice versa. Only two articles have reported that low 25-hydroxy-cholecalciferol (vitamin D3, VD3) levels were not correlated with the presence of conventional antiphospholipid antibodies (aPL Abs: anticardiolipin (aCL), anti-beta2glycoprotein I (aβ2gpI), and lupus anticoagulant (LA)), but no article analyzed the association of VD3 and anti-annexin A5 (aanxA5) Abs. The aim of our study was to investigate the association between VD3, multiple positivity of conventional aPL and aanxA5 Abs levels only in female OAPS vs. VAPS. Our study included 62 consecutive female PAPS patients. Concentrations of Abs were measured by ELISA, while VD3 levels were determined by immunochemiluminescence. Only 10/62 (16.13%) had sufficient (≥ 30 ng/ml) VD3 levels, while 48/62 (77.42%) and 4/62 (6.45%) had insufficiency and VD3 deficiency, respectively. Statistically significant VD3 deficiency was noticed in VAPS (vs. OAPS, P = 0.013). A negative correlation between VD3 levels and the age of patients was noticed (r = − 0.493, P = 0.032) only in VAPS subgroup. Multiple positivity of aPL and aanxA5 Abs was not associated with VD3 deficiency. Newly emerging aPL Abs, such as aanxA5 Abs, or their combinations with classical aPL Abs are not associated with VD3 deficiency in neither OAPS nor VAPS patients. Due to its immunomodulatory roles in B-Ly homeostasis, supplementation with VD3 should be considered in APS, at least in subgroup with severe form of the disease, i.e., VAPS. [ABSTRACT FROM AUTHOR]

The study aims to analyse the clinical and immunological manifestations of paediatric antiphospholipid syndrome (APS) in patients, based on the 2006 revised classification criteria of definite APS. Fifty-eight paediatric patients with APS were enrolled and analysed retrospectively. A total of 37 female and 21 male patients with a mean age of 14 ± 3 years at disease onset were included. Fourteen (24%) cases were primary APS, and 40 (69%) cases were secondary to systemic lupus erythaematosus (SLE). Anti-nuclear antibody (ANA) positivity and hypocomplementemia were more common in secondary APS than in primary APS. The most common manifestations of thrombosis were deep vein thrombosis of the lower extremities (25 cases, 37%). Non-thrombotic manifestations were mainly immunologic thrombocytopenia, autoimmune haemolytic anaemia, skin lesions, arthritis, pulmonary hypertension, heart valve vegetations and spontaneous abortion. LA, ACL and anti-β2GPI were positive in 42 (95%), 28 (64%) and 34 (77%) cases, respectively. Over half (23 cases, 52%) of the patients were triple-positive for antiphospholipid (aPL) antibodies. Among patients with single-positive LA and anti-β2GPI, the proportion with venous thrombosis was 100% (5 cases) and 0% (0 cases), respectively. The arterial thrombosis proportions were 22% (5 cases), 21% (3 cases) and 14% (1 case) in the triple-, double- and single-aPL-positive groups, respectively (P > 0.05). Fifty-three (91%) cases were followed up for 3 to 140 months, with a median time of 32 months. Seven (13%) cases had recurrences or appearances of thrombosis during follow-up, all of which were double- or triple-aPL positive. APS in the paediatric patients is mostly secondary to SLE. ANA positivity and hypocomplementemia are more common in secondary APS, but there are no differences in the other clinical manifestations between the primary and secondary APS groups. Deep vein thrombosis is the most common thrombotic event. Positive LA may increase the risk of venous thrombosis. Multiple-aPL positivity does not increase the proportion of thrombosis. Long-term anticoagulant or antiplatelet therapy is needed to prevent thrombosis recurrence in double- or triple-positive aPL cases. [ABSTRACT FROM AUTHOR]

Although numerous studies investigated the association between homocysteine (Hcy), tumor necrosis factor alpha (TNF-α), C-reactive protein (CRP) and apolipoproteins (apo) with thrombosis and/or recurrent pregnancy losses, studies that analyzed the abovementioned parameters and multiple positivity of antiphospholipid antibodies (aPL Abs) in patients with primary antiphospholipid syndrome (PAPS) are lacking. Therefore, the aim of this study was to analyze the presence of various combinations of the abovementioned parameters and their associations with clinical and/or serological features of PAPS. High-pressure liquid chromatography (HPLC) was used for determination of Hcy, while apoAI, apoB, and lipoprotein (Lp) (a) concentrations were estimated by immunonephelometry. High-sensitivity C-reactive protein (hsCRP) was measured by immunoturbidimetry. Apo (a), TNF-α, and aPL Abs were measured by ELISA. Various combinations of analyzed parameters (Hcy/CRP/TNF/apoAI/apoB/apo (a)/Lp (a)) were not associated with a single presence of either aPL Abs. Double positivity for both isotypes of anticardiolipin (aCL) Abs (IgG + IgM) was associated with the increased apoB levels. The presence of the IgG isotype of both aCL + anti-beta2 glycoprotein I (aβ2gpI) Abs was associated with the highest number of analyzed parameters (i.e., increased levels of hsCRP, Lp (a), and apo (a)). The presence of the IgG isotype of both aCL + aβ2gpI Abs was associated with the highest number of vascular impairment parameters in patients with PAPS, and this combination confers the highest risk for the recurrence of thrombotic episodes. This is the first report that analyzed the association between various combinations of vascular impairment parameters with multiple aPL Ab positivity. Our results provide a rationale for further investigations of therapeutic approaches for PAPS patients. [ABSTRACT FROM AUTHOR]

This study aims to identify risk factors for thrombosis in patients with systemic lupus erythematosus (SLE) and to validate the efficacy of the partial antiphospholipid (aPL) score for thrombosis prediction and diagnosis of antiphospholipid syndrome (APS). This study included 325 SLE patients, 188 of whom completed a follow-up of 31.01 months (range 23-48 months). Partial aPL score was calculated by adding up the individual scores for activated partial thromboplastin time (APTT), lupus anticoagulant, IgG/IgM anticardiolipin antibodies (aCL), and IgG/IgM anti-β2-glycoprotein I (anti-β2GPI). A simplified aPL score was developed using only APTT, IgG/IgM aCL, and IgG/IgM anti-β2GPI. Partial aPL scores were significantly higher in SLE patients with thrombosis ( p < 0.0001). A history of thrombosis ( p < 0.0001), a partial aPL score >10 ( p < 0.0001), and immunosuppressant use ( p = 0.012) were independent risk factors for thrombosis. For patients with a history of thrombosis, partial aPL score was the strongest risk factor for recurrent thrombosis ( p < 0.0001, odds ratio = 30.34 (95 % CI 7.70-118.81)). For APS diagnosis, the area under the receiver-operating characteristic curve (AUC) was 0.809 (95 % CI 0.73-0.89) using the partial aPL score. Similarly, the simplified aPL score was significantly associated with thrombosis ( p < 0.0001) and was acceptable for APS diagnosis (AUC 0.797, 95 % CI 0.72-0.88). An elevated partial aPL score is a strong risk factor for thrombosis in SLE patients and is a useful tool to predict recurrent thrombosis. Partial aPL score and simplified aPL score, although comprising fewer items than the original aPL score, also represent valuable quantitative indices for APS diagnosis. [ABSTRACT FROM AUTHOR]

Catastrophic antiphospholipid syndrome (CAPS) is a rare variant of antiphospholipid syndrome characterized by widespread thrombotic microangiopathy and multiorgan failure. Clinically, CAPS signs and symptoms can mimic vasculitis of systemic lupus erythematosus, disseminated intravascular coagulation, and thrombotic thrombocytopenic purpura. CAPS is burdened by high mortality, nearly 50 % in most series. However, patients surviving the acute phase rarely suffer of CAPS relapses. Moreover, concomitant pulmonary hemorrhagic alveolitis is a very rare complication warranting an ominous prognosis. Only few reports of relapsing CAPS are described in literature, and pathogenetic mechanisms are poorly understood and the optimal treatment is yet unknown. We report a case of a young man suffering from multiple relapses of CAPS and recurrent hemorrhagic pulmonary alveolitis refractory to aggressive combination treatment. [ABSTRACT FROM AUTHOR]

The objectives of the study were to describe the experience of patients immediately prior to a diagnosis of Hughes syndrome (HS) or antiphospholipid syndrome and post-diagnosis. A questionnaire survey was carried out set in the Hughes Syndrome Foundation, St. Thomas’ Hospital, London, 2006. Participants were all patients who are members of the Hughes Syndrome Foundation. The main outcome measures were responses to a questionnaire relating to the experiences of people with a diagnosis of HS, such as number of hospitalisations, number of consultants seen, number of miscarriages, etc. A total of 157 patients completed the questionnaire, giving a response rate of 60.4%. Most (85%) were women and mean age was 46 years (SD 12). The median time to diagnosis was 3 years. The median number of consultants seen was 2 (max 19) with a median time in hospital pre-diagnosis of 10 days. The most common initial diagnoses were migraines, multiple sclerosis and systemic lupus erythematosus. Among women, 46% had had a miscarriage. Two thirds of respondents thought a blood test would have led to an earlier diagnosis. Comments from patients indicated a lack of awareness among specialists and general practitioners. The survey demonstrated a long time lag for diagnosis of Hughes syndrome, with increased costs to the NHS and emotional and financial cost to the patient. Greater awareness of this condition would benefit patients and the NHS. [ABSTRACT FROM AUTHOR]

The purpose of the following study was to analyze maternal and fetal outcomes in pregnant patients with systemic lupus erythematosus (SLE) and the influence of SLE exacerbations on those pregnancies. Seventy-two pregnancies in 61 SLE patients treated between January 1986 and February 2004 in Hospital de Clínicas “José de San Martin” were reviewed retrospectively. Patient age was 28.1 ± 6.2 years (mean±standard deviation [SD]). Mean SLE duration was 4.5 ± 3.2 years (range 6 months–10 years). No patient acquired the disorder during gestation. Four (5.5%) patients had signs of active disease at the beginning of her pregnancy. Sixteen patients, accounting for 20 pregnancies, had a history of lupus nephritis. Nine patients met secondary antiphospholipid syndrome criteria and had 13 pregnancies. There were 14 exacerbations of the disease during pregnancy (19.4%), with most flares being mild. The most common obstetric complications were gestational hypertension in 15 pregnancies (20.8%) and preeclampsia in 8 pregnancies (11%). Forty-six percent of pregnancies ended in preterm deliveries. There were 62 live births (1 twin birth; 85%), 6 stillbirths (8%), and 5 spontaneous abortions (7%). Thirty-nine percent of newborns had low birth weight. Adequate pregnancy follow-up and delivery care by an interdisciplinary team in Argentine SLE patients with no pre-gestational preparation resulted in maternal and fetal outcomes similar to those seen in world reference centers. [ABSTRACT FROM AUTHOR]

The catastrophic anti-phospholipid syndrome (CAPS) differs from the anti-phospholipid syndrome in its accelerated systemic involvement leading to multi-organic failure. In this study, the occurrence of malignancies in patients with CAPS was evaluated and the clinical findings of CAPS patients with and without malignancies were compared. We investigated the web site-based international registry of patients with CAPS for all cases in which both CAPS and underlying malignancies were present. The clinical characteristics of these cases were subsequently evaluated to establish common characteristics. The CAPS registry included information on a total of 262 cases. Twenty-three (9%) patients suffered from malignancies. In 78% of these patients, the malignancy itself or the treatment modalities instituted for the carcinoma was the precipitating factor of CAPS. Only 39% of CAPS patients with malignancies recovered in comparison to 58% of patients without malignancies ( p = 0.07). Treatment modalities, however, did not differ significantly between these patients. Infections were not evident as precipitating factors for any of the malignancy patients. The mean age of patients with malignancies was 9 years older than the average age of other patients with CAPS and the prevalence of SLE was significantly less common than in patients without malignancy. Malignancy may play a pathogenic role in patients with CAPS, whereas infections are more important as triggering factors in patients without malignancies. CAPS patients with malignancies are generally older than CAPS patients without malignancies; they generally have the worst prognosis of the entire CAPS cohort. [ABSTRACT FROM AUTHOR]

An episode of gastroenteritis triggered severe necrosis of all extremities in a previously asymptomatic male. Hepatic and renal involvement were also manifest, while the hematological picture was one of thrombotic microangiopathic hemolytic anemia. Antiphospholipid antibodies were negative. He responded well to a combination of plasma exchange, anticoagulation (heparin), parenteral steroids, and antibiotics, as well as vasodilators (prostacycline) and hyperbaric oxygen, but died because of a cerebral hemorrhage. The differential diagnosis included thrombotic thrombocytopenic purpura/hemolytic–uremic syndrome, or seronegative catastrophic antiphospholipid (Asherson’s) syndrome. The dangers of administering such a combination of therapies with anticoagulation, as well as vasodilatation (prostacycline) and hyperbaric oxygen, are highlighted by the case report and emphasized. [ABSTRACT FROM AUTHOR]

: Transverse myelitis (TM) is a rare manifestation of systemic lupus erythematosus (SLE) and the antiphospholipid syndrome (APS). No uniform therapeutic protocol exists for its treatment, and the prognosis is usually poor. Here we describe four patients having TM associated with antiphospholipid antibodies. Treatment measures and delay in diagnosis between symptom onset and the initiation of treatment varied between patients, but the earlier the diagnosis and the more aggressive the treatment the better was the patient’s outcome. Based on these cases and on a literature review we suggest that early aggressive treatment (usually with pulses of methylprednisolone and cyclophosphamide) might improve the prognosis of patients with TM associated with antiphospholipid antibodies. [ABSTRACT FROM AUTHOR]

Dilated cardiomyopathy secondary to a devastating anterior myocardial infarction developed in a young patient with the antiphospholipid antibody syndrome. Due to severe left ventricular failure, heart transplantation was considered. To reduce antiphospholipid antibody titer, plasmapheresis followed by immunosuppressive treatment with cyclosporin and azathioprine were attempted in the pretransplant period. The antibody titer normalized after plasmapheresis, but then rose sharply despite immunosuppressive drugs. This case report underscores the failure of cyclosporin, a treatment not previously reported, to control autoimmunity in antiphospholipid syndrome. Moreover, progressive renal insufficiency can develop as a result of the long-term use of this drug. [ABSTRACT FROM AUTHOR]