Quality of life measures in Systemic Lupus Erythematosus: A systematic review.

In this study we systematically investigated the health-related quality of life (HRQoL) tools, which have been most often used over the last five years to evaluate the QoL in patients with systemic lupus erythematosus (SLE), focusing on their items and applications. A detailed literature search was conducted: the inclusion criteria were as follows: 1) studies including at least 50 patients; 2) studies including at least 25 patients with SLE; 3) quality of life testing with validated measures. The systematic review was based on 119 studies for a total of 32,449 SLE patients and 3092 controls. A total of 35 different patients-reported quality of life measures, applied in cohorts of patients with SLE, were retrieved with the 36-item Medical Outcome Short Form (SF-36) (63 studies of 119 =52.95%), Lupus Quality of Life (LupusQoL) (17 studies =14.3%) and Lupus Patient-Reported Outcome (LupusPRO) (12 studies =10%) being the most commonly used tools. Overall, this systematic review of the literature indicated that quality of life in patients with SLE appears to be poor and generally lower compared to both the general population and patients with other chronic conditions, as was shown by a few studies that used SF-36 and LupusPRO. The use of HRQoL scoring in SLE is gaining increasing interest and is used both in randomized controlled trials and in real-life. Future efforts are needed to improve the understanding of the impact of the disease burden on quality of life from the patient's perspective.

The risk of ischaemic stroke in primary antiphospholipid syndrome patients: a prospective study.

BACKGROUND AND PURPOSE: The most common neurological manifestation of antiphospholipid syndrome (APS) is ischaemic stroke. Identifying patients with APS at high risk for developing any thrombotic event remains a major challenge. In this study, the aim was to identify predictive factors of ischaemic stroke in a cohort of primary APS (PAPS) patients who presented with new onset symptoms suggestive of acute stroke. METHODS: This prospective multicentre study included 36 consecutive PAPS patients who presented with new onset symptoms suggestive of an acute stroke. Patients were prospectively followed up for 12 months. RESULTS: In 10 (28%) out of 36 PAPS patients [mean age 41 years (SD 13.4), 70% female], the suspicion of an acute stroke was confirmed by brain magnetic resonance imaging. Sixty per cent of these patients were <50 years old. Eight of the 10 patients had a history of previous venous thrombosis and were receiving vitamin K antagonist (VKA), with international normalized ratio target 2-3; one patient had a history of a previous arterial event receiving treatment with VKA target international normalized ratio 2-3 plus low dose aspirin; and one patient had a history of previous pregnancy morbidity receiving only low dose aspirin. Time in the therapeutic range for patients receiving VKA was 77.7% (SD 6.6%). Hypercholesterolaemia was significantly higher in patients with confirmed stroke compared to those without (P < 0.05). Similarly, a significantly higher rate of anti-ß2 glycoprotein-I (ß2GPI) antibodies (immunoglobulin G/immunoglobulin M; P < 0.05) and higher adjusted global APS score (aGAPSS) values were found in patients with a confirmed stroke [mean aGAPSS 8.9 (SD 4.7) vs. mean aGAPSS 6.4 (SD 2.5); P < 0.05]. CONCLUSIONS: Patients with PAPS, including young patients, have a high risk of recurrent thrombosis despite anticoagulation treatment. A careful risk assessment is mandatory to identify patients at risk for recurrence.

Patients with antiphosholipid syndrome and thrombotic recurrences: A real world observation (the Piedmont cohort study).

BACKGROUND: Patients with antiphospholipid syndrome (APS) often have thrombotic recurrences, sometimes despite appropriate ongoing anticoagulant treatment. Identifying APS vascular patients at high risk for thrombotic recurrences is still an unsolved issue. OBJECTIVES: To report the real-life experience of thrombotic recurrences in APS patients included in the Piedmont observational cohort study, and evaluate clinical and laboratory risk factors for thrombotic recurrences. PATIENTS: A multi-centre observational study was performed by enrolling 177 patients with vascular APS (primary APS in 99 subjects (56%)); the median follow-up was five years (range 1-26 years). RESULTS: The observed thrombotic recurrence rate was about 7.5/100 patient years in the first five years after the first thrombotic event. While the first recurrence often occurred (45%) in patients who were not on oral anticoagulant therapy (OAT), the second recurrence mainly occurred despite ongoing OAT (80%). However, due to the real-life observational nature of this study, treatment was based on the treating physician's judgement, and no structured therapeutic protocol was applied. Moreover, compliance with OAT was not available. No differences in antiphospholipid antibodies (aPL) profile were observed between patients with or without thrombotic recurrences, but a high risk aPL profile (Miyakis type 1 and 2a) was present in 96% of our patients, 26% of whom had triple positivity. Diabetes (p < 0.01, OR 10), inherited thrombophilia (p < 0.0078, OR 4) and OAT withdrawal were independent risk factors for recurrences. CONCLUSIONS: With the limit of a real-life observational cohort study, the thrombotic recurrence rate in APS was as high as 7.5/100 patient years in the first five years after the first thrombotic event. OAT discontinuation, diabetes and inherited thrombophilia, when associated with a high-risk aPL profile, are risk factors for thrombotic recurrences.

Kidney biopsy in pregnancy: evidence for counselling? A systematic narrative review.

BACKGROUND: Kidney diseases, which have a prevalence of 3% in women of childbearing age, are increasingly encountered in pregnancy. Glomerulonephritis may develop or flare up in pregnancy, and a differential diagnosis with pre-eclampsia may be impossible on clinical grounds. Use of kidney biopsy is controversial, but a systematic review has not been carried out to date. OBJECTIVES: To review the literature on kidney biopsy in pregnancy, with a focus on indications, risks and timing. SEARCH STRATEGY: Medline, Embase, CHINAL and the Cochrane Library were searched in September 2012, with 'pregnancy' and 'kidney biopsy' used as MESH and free terms, for the period 1980-2012. Results were filtered for 'human' if this option was available. SELECTION CRITERIA: Biopsies during pregnancy and within 2 months after delivery. Case reports (fewer than five cases) and kidney grafts were excluded. Paper selection was performed in duplicate. DATA COLLECTION AND ANALYSIS: Data were extracted in duplicate. The high heterogeneity in study design necessitated that the review be narrative, except for data on adverse events, which were analysed with regard to the timing of kidney biopsy. MAIN RESULTS: Of 949 references, 39 were selected, providing data on 243 biopsies in pregnancy and 1236 after delivery (timing was unclear in 106 women). The main aims of the studies were to define morphology in pre-eclampsia (23 studies), to carry out a risk-benefit analysis of kidney biopsy (11 studies), and to investigate pregnancy-related acute kidney injury (five studies). Four cases of major bleeding complications occurred at 23-26 weeks of gestation. Relevant complications were observed in 7% of women during pregnancy and 1% after delivery (P = 0.001). Kidney biopsy performed for the diagnosis of glomerulonephritis or pre-eclampsia led to therapeutic changes in 66% of cases. AUTHORS' CONCLUSIONS: The evidence on kidney biopsy in pregnancy is heterogeneous, but a significantly higher risk of complications (relative to postpartum biopsy) was found, with a possible peak at around 25 gestational weeks.

A randomized controlled trial of rituximab for the treatment of severe cryoglobulinemic vasculitis.

OBJECTIVE: To conduct a long-term, prospective, randomized controlled trial evaluating rituximab (RTX) therapy for severe mixed cryoglobulinemia or cryoglobulinemic vasculitis (CV). METHODS: Fifty-nine patients with CV and related skin ulcers, active glomerulonephritis, or refractory peripheral neuropathy were enrolled. In CV patients who also had hepatitis C virus (HCV) infection, treatment of the HCV infection with antiviral agents had previously failed or was not indicated. Patients were randomized to the non-RTX group (to receive conventional treatment, consisting of 1 of the following 3: glucocorticoids; azathioprine or cyclophosphamide; or plasmapheresis) or the RTX group (to receive 2 infusions of 1 gm each, with a lowering of the glucocorticoid dosage when possible, and with a second course of RTX at relapse). Patients in the non-RTX group who did not respond to treatment could be switched to the RTX group. Study duration was 24 months. RESULTS: Survival of treatment at 12 months (i.e., the proportion of patients who continued taking their initial therapy), the primary end point, was statistically higher in the RTX group (64.3% versus 3.5% [P < 0.0001]), as well as at 3 months (92.9% versus 13.8% [P < 0.0001]), 6 months (71.4% versus 3.5% [P < 0.0001]), and 24 months (60.7% versus 3.5% [P < 0.0001]). The Birmingham Vasculitis Activity Score decreased only after treatment with RTX (from a mean ± SD of 11.9 ± 5.4 at baseline to 7.1 ± 5.7 at month 2; P < 0.001) up to month 24 (4.4 ± 4.6; P < 0.0001). RTX appeared to be superior therapy for all 3 target organ manifestations, and it was as effective as conventional therapy. The median duration of response to RTX was 18 months. Overall, RTX treatment was well tolerated. CONCLUSION: RTX monotherapy represents a very good option for severe CV and can be maintained over the long term in most patients.

Antiphospholipid syndrome in northwest Italy (APS Piedmont Cohort): demographic features, risk factors, clinical and laboratory profile.

We report the experience from the Antiphospholipid Antibodies (aPL) Regional Consortium in northwest Italy, meant to support clinical research and foster collaboration among health professionals regarding the diagnosis and management of antiphospholipid syndrome (APS) patients. This cohort-study (APS Piedmont Cohort) was designed to register the clinical characteristics at inception and associated immunological manifestations at diagnosis (if any) of patients who strictly fulfilled the current criteria for APS, all recruited at the Piedmont and Valle d'Aosta regions. Clinical and laboratory data from 217 APS patients (171 with vascular events, 33 with pregnancy morbidity and 13 with both), from 16 centres within the geographical area were collected. Venous thrombosis was recorded in 45.6% of patients, arterial thrombosis in 35%, small-vessel thrombosis in 1.12% and mixed arterial and venous thrombosis in the remaining 19.4% of the cases. Pregnancy morbidity included 19 patients with unexplained fetal death beyond the 10th week of pregnancy, 17 with premature birth before the 34th week and 10 with three or more unexplained spontaneous abortions before the 10th week of gestation. This consortium represents an instrument by which to audit clinical practice, to provide counselling to local centres and to sustain future basic and clinical APS research.

NEMO syndrome (incontinentia pigmenti) and systemic lupus erythematosus: a new disease association.

Congenital diseases are increasingly being recognised in adults because of clinical mimicry, variable clinical picture or rarity of the disease; pregnancy is a valuable diagnostic occasion. The present case is the first report of an association report between NEMO syndrome (an acronym of the mutated, non-functioning gene, NF-kB essential modulator), a rare X-linked disease, characterised by developmental anomalies, immunodepression and skin lesions, and systemic lupus erythematosus (SLE). A 35-year-old patient affected by SLE sought clinical advice in the 8th week of gestation. The diagnosis of SLE dated back to the age of 24, when multisystemic manifestations (pleuropericarditis, weight loss, alopecia, skin involvement, joint pain, kidney involvement) were observed. She had been treated with steroids since 1999; immunosuppressive drugs had been added for short periods. Developmental anomalies were present, including oligodontia, retinal problems, anomalies of the corpus callosum and pes planovalgus. Family history included multiple miscarriages, dental malformations and oligodontia and skin blistering in the first months of life. On these bases, incontinentia pigmenti (IP; or NEMO syndrome) was diagnosed and confirmed by genetic testing. The NEMO gene is implicated in immune deficiencies as well as in autoimmune diseases. This report may suggest a role for NF-kB essential modulator in the pathogenesis of SLE, in the context of the complex immunologic deficiencies increasingly associated with autoimmune diseases.

Prevention of thrombosis relapse in antiphospholipid syndrome patients refractory to conventional therapy using intravenous immunoglobulin.

OBJECTIVES: To investigate the long-term effects of megadoses of intravenous immunoglobulin (IVIG) in a small cohort of patients with relapsing primary APS resistant to conventional treatments. METHODS: Five primary APS patients, 4 women, mean age 45.1 years (range 31-76 years), were considered eligible for IVIG therapy due to relapsing thrombotic events (4 recurrent venous thromboses, 2 ischaemic cerebral strokes, 2 pulmonary thromboembolisms, 1 thrombotic event on the vena cava filter), despite conventional therapy with anticoagulants. All patients had anti-nuclear antibodies at low-medium titre without other signs or symptoms of systemic lupus erythematosus. IVIG was combined with hydroxychloroquine and, in patients with cerebral strokes, acetylsalicylic acid. Three consecutive daily infusions of IVIG were administered intravenously at a dose of 0.4 g/kg/day every month for 3 months, followed by a single monthly infusion for 9 months. RESULTS: No further thromboses occurred in the 5 treated patients (mean follow-up 89.2 months, range 61-114). Visual analogue score (VAS 0-10) improved (mean 3.5, range 3.0-5.0, before, and 7.35, range 9.9-6.0, p= 0.05) after IVIG treatment. CONCLUSIONS: In a long-term (>5 years) open study in a small cohort of high risk primary APS patients, IVIG was found to be effective in preventing recurrent thrombosis. Full understanding of the mechanisms and efficacy, as well as the optimal doses of IVIG in APS patients with recurrent thrombosis, will require appropriately designed clinical studies. Presently, IVIG use is restricted by costs and limited availability.

Pregnancy success rate and response to heparins and/or aspirin differ in women with antiphospholipid antibodies according to their Global AntiphosPholipid Syndrome Score.

BACKGROUND: The current treatment to prevent pregnancy morbidity (PM) associated with antiphospholipid antibodies (aPL) is based on the use of low dose aspirin and low molecular weight heparin (henceforth defined as standard of care (SoC) treatment). Despite the SoC, up to 30% of women with aPL continue to have pregnancy complications. The global antiphospholipid syndrome (APS) score (GAPSS) is a tool to quantify the risk for the aPL-related clinical manifestations. In this study, we investigated the individual clinical response to SoC in women with aPL after stratifying them according to their GAPSS. METHODS: One-hundred-fourty-three women (352 pregnancies) with aPL ever pregnant treated with SoC therapy were included. The patients GAPSS was then grouped according to the patients' GAPSS into low risk (< 6), medium risk (6-11), and high risk (≥12). RESULTS: The live birth rate was 70.5% (248 out of the 352 pregnancies), 45 patients (31%) experienced at least one event of PM, defined as early or late. Patients were stratified according to GAPSS values, in order to identify a low risk group (GAPSS <6, n = 72), a medium risk group (GAPSS 6-11, n = 66) and a high risk group (GAPSS ≥12, n = 5). When considering patients who ever experienced any PM while treated with SoC, all patients in the high risk group experienced PM, while patients in the medium group had a significant higher rate of PM when compared to the low risk group [29 (43.9%) patients V.s. 11 (15.3%), respectively; p < 0.001]. When analysing the number of pregnancies in the three groups, patients in the high risk group had significantly lower live birth rates, when compared to the other groups [11 (40.7%) live births vs. 100 (62.1%) and 137 (82.5%), respectively; p < 0.05]. Furthermore, patients with medium risk group also had significantly lower live birth rates, when compared to the lower risk group (p < 0.001). CONCLUSIONS: GAPSS might be a valuable tool for to identify patients with a higher likelihood of response to SoC.

Effects of anti-CD20 monoclonal antibody as a rescue treatment for ANCA-associated idiopathic systemic vasculitis with or without overt renal involvement.

BACKGROUND: Cyclophosphamide (CYC) is thought to be the most effective treatment for antineutrophil cytoplasmatic antibody (ANCA)-associated idiopathic systemic vasculitis with severe organ or life threatening presentation. The key mechanism of action of CYC is suppression of the B lymphocyte activity. However, a considerable minority of patients either remains refractory to conventional therapy or experiences dose-limiting side effects. METHODS: In the present study, rituximab (4 weekly doses of 375 mg/m2 and 2 more doses at 1-month interval) was intravenously administered as a rescue therapy to 7 patients (4 affected by idiopathic systemic microscopic polyangiitis, 2 by Wegener's granulomatosis, and 1 affected by Churg Strauss syndrome). The study group was made up of 3 women and 4 men, mean age 61.5 years (39-71), intolerant or refractory to more conventional therapy. Four patients had histologically confirmed paucimmune necrotizing glomerulonephritis. RESULTS: Significant decreases were observed in levels of serum creatinine, proteinuria, erythrocyte sedimentation rate, C-reactive protein, and ANCA titers within the first 12 months of follow-up. Arthralgia and weakness rapidly disappeared in all patients. Four out of five patients reported a decrease in the degree of paresthesia, paralleled by an improvement in the electrodiagnostic parameters. A significant improvement was observed in both Birmingham Vasculitis Activity Score and Vasculitis Damage Index. Side effects were negligible. CONCLUSION: In this sample of patients with idiopathic systemic vasculitis that was refractory or intolerant to conventional treatment, rituximab was found to be a safe and effective rescue therapy.

HCV infection: pathogenesis, clinical manifestations and therapy.

Chronic hepatitis C virus (HCV) infection is a worldwide public health problem with a global prevalence of 2-3%. It is believed that about 170 million people are currently infected (about 3% of the world's population), and a further 3-4 million are infected each year. HCV is the main reason for liver transplantation in the developed world, and the main cause of liver-related morbidity and mortality in a number of countries, including Italy. It is not only a frequent cause of chronic liver diseases such as hepatitis, cirrhosis and hepatocellular carcinoma, but is also involved in the pathogenesis of various autoimmune and rheumatic disorders (arthritis, vasculitis, sicca syndrome, porphyria cutanea tarda, lichen planus, nephropathies, thyroid diseases, and lung fibrosis), as well as in the development of B-cell lymphoproliferative diseases. Furthermore, patients suffering from C hepatitis tend to produce rheumatoid factor, cryoglobulins and a large series of autoantibodies (ANA, anti-SSA/SSB, SAM, ATG, aCL). The use of glucocorticoids or immuno-suppressant agents in HCV infected individuals, which are needed to treat autoimmune and rheumatic disorders, leads to a risk of worsening the clinical outcome of HCV. Under these conditions, the viral infection often needs to be treated with antiviral agents, mainly pegylated interferon combined with ribavirin. However, cyclosporine A seems to be safe and effective in patients with autoimmune disease (AD) and concomitant chronic HCV infection as is documented by the reduction in viremia and transaminases, particularly in patients with high baseline levels. Finally, HCV is the main trigger of mixed cryoglobulinemia. An attempt at viral eradication is therefore indicated in most patients, and is particularly effective in the case of mild or moderate manifestations. In severe cases, rituximab is an apparently safe and effective alternative to conventional immunosuppression and, specifically, it controls B-cell proliferation.

Anti-GM1 and anti-sulfatide antibodies in patients with systemic lupus erythematosus, Sjögren's syndrome, mixed cryoglobulinemia and idiopathic systemic vasculitis.

OBJECTIVES: Over the last two decades, increasing interest has been focused on the association between autoimmune polyneuropathies and anti-neuronal autoantibodies in immune-mediated polyneuropathy. The possible appearance of these autoantibodies in systemic diseases that are not limited to the nervous system has not been fully addressed yet. METHODS: We evaluated 32 patients with systemic lupus erythematosus, 34 patients with hepatitis C virus-associated mixed IgM-k/IgG cryoglobulinemia, 19 with small vessel ANCA-associated vasculitis, and 20 patients with Sjögren's syndrome by means of an immunoenzyme method of anti-neuronal autoantibody detection. RESULTS: As compared to normals, a significant increase (p < 0.001) in plasma titers of both IgM and IgG anti-GM1 ganglioside and IgM and IgG anti-sulfatide was observed in patients with systemic lupus erythematosus, mixed cryoglobulinemia and Sjög-ren's syndrome. Idiopathic systemic vasculitis patients were found to have significantly increased levels of anti-sulfatide IgG autoantibodies (p < 0.001). Clinical and electrophysiologic studies revealed that abnormal titers of anti-neuronal antibodies were associated with evidence of neuropathy in patients with systemic lupus erythematosus and ANCA-related vasculitis (p < 0.05) as well as in patients with mixed cryoglobulinemia and Sjögren's syndrome (p < 0.001). CONCLUSION: Anti-GM1 and anti-sulfatide antibodies are frequently found in patients with small vessel ANCA-associated vasculitis and other multi-organ immune-mediated diseases. Upon detection of these antibodies, accurate neurologic examination should be carried out due to the significant association that can be found between these serologic abnormalities and the involvement of the peripheral nervous system as also detected by electrophysiologic studies. This study supports the unexpected possibility that anti-neuronal reactivity may be a direct trigger of neurologic injury in these systemic disorders.

Mass spectrometry analysis of IgA1 hinge region in patients with IgA nephropathy.

BACKGROUND: Physicochemical alterations of the IgA molecule are supposed to play a pathogenetic role in IgA nephropathy (IgAN). The present study was carried out to analyze the structural variety of O-glycans on the IgA1 hinge region in IgAN. Sera from 9 IgAN patients and 9 healthy controls were individually examined to evaluate the IgA1 content and binding lectins (jacalin and Helix aspersa), using enzyme-linked immunosorbent assay (ELISA) techniques. The IgA1 from pooled sera were separated by affinity chromatography (jacalin), and the fragment containing the hinge region was prepared by pyridylethylation and trypsin treatment. The IgA fragments containing the hinge glycopeptide (33-mer hinge peptide core (HP) + O-glycans) were separated by jacalin affinity chromatography. Because we used jacalin, we only analyzed the Gal-3GalNAc residue containing IgA. The molecular weight (MW) of the IgA1 fragments was estimated using an ion trap mass spectrometer equipped with an electrospray ion source (ESI/MS). RESULTS: IgA1 concentration in pathological sera was higher than in the control serum (p<0.01). Compared with controls, serum IgA1 from IgAN patients showed significantly greater binding to the 2 lectins, jacalin (p<0.01) and Helix aspersa (HA, p<0.001), which are specific for O-linked Gal-beta1,3-GalNAc and GalNAc, respectively. Analyses of pooled sera showed that the number of O-glycosidic chains was comparable in IgAN and normal sera. With regards to the individual residues, we found that IgAN sera contained less sugar and galactose and sialic acid moieties than sera from control subjects, was reduced in IgAN sera, while terminal N-acetylgalactosamine levels were higher when compared with normal serum. CONCLUSIONS: Abnormalities of hinge region O-linked glycans were confirmed using advanced spectrometry technology. The pathogenetic implications for aggregation and defective removal of IgA1 are discussed.

[Antiphospholipid syndrome and kidney]. / Sindrome da anticorpi anti fosfolipidi e rene.

The diagnosis of antiphospholipid syndrome (APS) relies on clinical and laboratory criteria, which have been recently outlined in specific consensus conferences. Renal involvement in APS is not infrequent and includes different clinical patterns. For clinical purposes a distinction can be made between large vessel and microvascular involvement. Renal artery stenosis is frequent in APS. In case of microvascular involvement with an acute clinical course a differential diagnosis with other thrombotic microangiopathic diseases has to be made, taking in account thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, malignant hypertension, drug nephrotoxicity (cyclosporin) and others. The disease is often chronic, with hypertension, different degrees of renal insufficiency and mild proteinuria. In patients with systemic lupus erythematosus and antiphospholipid antibodies the prognosis of kidney disease is generally poorer than in lupus alone. Finally, the kidney is almost invariably a target in catastrophic antiphospholipid syndrome. Anticoagulation is the therapy of choice, especially in arterial stenosis and acute disease, but is probably also indicated in chronic and subacute patterns. The role of immunomodulatory therapy has to be assessed.

Efficacy of belimumab on renal outcomes in patients with systemic lupus erythematosus: A systematic review.

Both BLISS-52 and BLISS-76 international phase III trials in Systemic Lupus Erythematosus (SLE) met their primary outcomes; however, they were not designed to assess the efficacy of belimumab for the treatment of lupus nephritis (LN). LN is a frequent cause of SLE-associated morbidity and mortality, and emerging evidence suggests a potential therapeutic role for agents that target B lymphocyte stimulator (BLyS). We conducted a systematic review to identify data on the effect of belimumab on LN. A total of 2004 patients with SLE were identified from 11 studies. Three hundred and twenty-six patients had LN at baseline and 234 (71.8%) of those received belimumab. Thirteen patients out of 234 (5.5%) received belimumab for active LN. Due to the heterogeneous definitions of treatment response, clinical presentation and renal involvement, it was not possible to compare results using a single outcome parameter. However, the majority of these studies defined clinical response in terms of rates of renal flare, renal remission, and/or renal organ disease improvement. One hundred twenty-nine (55.1%) of the 234 patients with LN at baseline showed an improvement in renal parameters after treatment with belimumab. In patients with baseline proteinuria>0.2g/24h, (n=687), those receiving belimumab had a median reduction in proteinuria during follow-up as high as 38%. When focusing on patients with proteinuria≥1g/24h (n=228), 70.7% of those treated with belimumab (n=157) achieved a renal response. In the pooled population of patients receiving belimumab, we found an overall annual renal flare rate of 1.7% [24/1448, mean observation time 1,1years (0,5-3)]. Despite the limitations of the studies included in this analysis, available data are promising and provide preliminary support for targeting BlyS to induce or maintain a renal response. Further trials should examine whether belimumab (alone or following rituximab) represents an additional therapeutic option in the treatment of LN.

The adjusted Global AntiphosPholipid Syndrome Score (aGAPSS) for risk stratification in young APS patients with acute myocardial infarction.

BACKGROUND: Young adults with acute myocardial infarction are a critical group to examine for the purpose of risk factor stratification and modification. In this study we aimed to assess the clinical utility of the adjusted Global AntiphosPholipid Syndrome Score (aGAPSS) for the risk stratification of acute myocardial infarction in a cohort of young patients with antiphospholipid syndrome (APS). METHODS: The analysis included 83 consecutive APS patients (≤50years old) who presented with arterial or venous thromboembolic events. Data on cardiovascular risk factors and antiphospholipid antibodies (aPL) positivity were retrospectively collected. The aGAPSS was calculated by adding the points corresponding to the risk factors, based on a linear transformation derived from the ß-regression coefficient as follows: 3 for hyperlipidaemia, 1 for arterial hypertension, 5 for aCL IgG/IgM, 4 for anti-b2 glycoprotein I IgG/IgM and 4 for LA. RESULTS: Higher aGAPSS values were observed in patients with acute myocardial infarction when compared to the others [mean aGAPSS 11.9 (S.D. 4.15, range 4-18) Vs. mean aGAPSS 9.2 (S.D. 5.1, range 1-17); T test: p<0.05]. Significantly higher aGAPSS values were also seen in patients with acute coronary syndrome compared to patients with a history of peripheral or cerebrovascular arterial thrombotic events [mean aGAPSS 11.9 (S.D. 4.15, range 4-18) Vs. mean aGAPSS 6.7 (S.D. 5.7, range 1-17); T test: P<0.005]. CONCLUSIONS: The aGAPSS is based upon a quantitative score and could aid risk stratifying APS patients younger than 50years for the likelihood of developing coronary thrombotic events and may guide pharmacological treatment for high-risk patients.