Raman spectrum of layered tilkerodeite (Pd2HgSe3) topological insulator: the palladium analogue of jacutingaite (Pt2HgSe3).

The layered mineral tilkerodeite (Pd2HgSe3), the palladium analogue of jacutingaite (Pt2HgSe3), is a promising quantum spin hall insulator for low-power nanospintronics. In this context, a fast and reliable assessment of its structure is key for exploring fundamental properties and architecture of new Pd2HgSe3-based devices. Here, we investigate the first-order Raman spectrum in high-quality, single-crystal bulk tilkerodeite, and analyze the wavenumber relation to its isostructural jacutingaite analogue. By using polarized Raman spectroscopy, symmetry analysis, and first-principles calculations, we assigned all the Raman-active phonons in tilkerodeite, unveiling their wavenumbers, atomic displacement patterns, and symmetries. Our calculations used several exchange-correlation functionals within the density functional perturbation theory framework, reproducing both structure and Raman-active phonon wavenumbers in excellent agreement with experiments. Also, it was found that the influence of the spin-orbit coupling can be neglected in the study of these properties. Finally, we compared the wavenumber and atomic displacement patterns of corresponding Raman-active modes in tilkerodeite and jacutingaite, and found that the effect of the Pd and Pt masses can be neglected on reasoning their wavenumber differences. From this analysis, tilkerodeite is found to be mechanically weaker than jacutingaite against the atomic displacement patterns of these modes. Our findings advance the understanding of the structural properties of a recently discovered layered topological insulator, fundamental to further exploring its electronic, optical, thermal, and mechanical properties, and for device fabrication.

Glucose and acetate metabolism in bovine intramuscular and subcutaneous adipose tissues from steers infused with glucose, propionate, or acetate.

We hypothesized that abomasal infusion of glucose would promote de novo fatty acid biosynthesis from glucose in vitro in bovine intramuscular (i.m.) and subcutaneous (s.c.) adipose tissues to a greater extent than ruminal infusion of acetate, propionate, or glucose. Angus crossbred steers (n = 24), 22 mo of age, were fitted with ruminal cannulas, and steers were adapted to another corn/sorghum finishing diet over a 2-wk period while recovering from the placement of the cannulas. After the adaptation period, the steers were fed the second finishing diet at 130% of their voluntary intake and were infused with isocaloric amounts (3.76 Mcal/d) of glucose, propionate, or acetate for 35 d. Glucose was infused either into the rumen or into the abomasum, whereas propionate and acetate were infused into the rumen. Acetate infusion decreased DM and DE intakes (P < 0.05). The 5th to 8th longissimus muscle section was removed immediately and transported to the laboratory within 10 min post-exsanguination in 38 °C, oxygenated Krebs Henseleit buffer containing 5 mM glucose and 5 mM acetate. Intramuscular and s.c. adipose tissues were dissected from the muscle and incubated in vitro in 5 mM glucose plus 5 mM acetate (containing [U-14C]glucose or [1-14C]acetate). Lipid content was lower (P = 0.04) in i.m. adipose tissue of the acetate-infused steers than in the other treatment groups, and i.m. adipocytes from acetate-infused steers were smaller (P = 0.01) than those from propionate-infused steers. The rate of incorporation of acetate into glyceride-fatty acids (GFA) in i.m. and s.c. adipose tissues was greater (P < 0.03) in steers receiving ruminal or abomasal infusions of glucose than in adipose tissues from steers infused with acetate. The greatest rates of GFA synthesis were observed in s.c. adipose tissue from steers infused ruminally with propionate or abomasally infused with glucose (P < 0.001). In i.m. and s.c. adipose tissues, the proportion of acetyl units from acetate incorporated into GFA was greater in steers receiving glucose infusion in the rumen or abomasum than in steers receiving acetate or propionate infusion (P < 0.05). Contrary to our hypothesis, abomasal glucose infusion did not promote greater fatty acid biosynthesis from glucose in i.m. adipose tissue than ruminal glucose infusion. However, glucose infusion caused the greatest production of acetyl units from acetate in i.m. and s.c. adipose tissues.

Utilização da técnica in vitro semiautomática de produção de gases na avaliação da fermentação microbiana do ceco de suínos / Use of the semi-automatic in vitro gas production technique for the evaluation of swine cecal microbial fermentation

O presente estudo teve como objetivo avaliar o os padrões de fermentação microbiana do ceco de suínos, utilizando-se um modelo aplicado na técnica in vitro de produção de gases para ruminantes. Três tipos de inóculo foram utilizados: ceco de suínos criados ao ar livre (CSF), ceco de suínos criados confinados (CSC) e líquido ruminal de bovino (LRB). Os substratos utilizados derivaram de uma dieta de suínos à base de farelo de soja e de grãos de milho, atendendo as exigências nutricionais dos suínos. Para composição dos substratos, foram estabelecidos diferentes níveis de substituição da dieta basal pela torta da amêndoa da Acrocomia aculeata (AA), coproduto da produção do biodiesel, sendo: BAS - 100% de dieta basal; M10 - 90% de dieta basal e 10% AA; M20 - 80% de dieta basal e 20% AA e o TF - feno de Tifton 85 (Cynodon spp.). A produção de gases foi avaliada nos tempos zero, três, seis, nove, 12, 16, 24, 48, 72 e 96 horas após a incubação. Foram analisados o potencial máximo de produção de gás (A) e o tempo de colonização (L). O delineamento experimental utilizado foi inteiramente ao acaso, em arranjo fatorial 4 x 3. Os dados de A e L foram submetidos à análise de variância, e as médias comparadas pelo teste de Tukey a 5%. Comparando os inóculos para A (mL/gMS), LRB e CSF apresentaram valores similares, diferindo apenas para TF. O tempo de colonização (L) entre inóculos, o CSC e o CSF apresentaram os menores tempos de colonização quando a ração foi BAS. Com os substratos M10 e M20, o inóculo LRB apresentou o menor tempo, CSF o maior tempo e CSC não diferiu de ambos. Não houve diferença significativa entre os inóculos para o substrato TF. A técnica de produção de gases utilizada para o ceco de suínos apresentou resultados semelhantes aos encontrados para ruminantes. O modelo matemático usado foi adequado para descrever a curva de fermentação no ceco de suínos, mostrando semelhanças entre as microbiotas do ceco e do rúmen.(AU)

This study aimed to evaluate the microbial fermentation patterns of the pig ceca using the technique of in vitro gas production for ruminants. Three types of inoculums were used: swine ceca raised in a free range system (CSF), swine ceca raised in a conventional confined system (CSC) and bovine rumen fluid (LRB). The substrates used were derived from pig's diet with soybean meal and corn grits, according to the nutritional requirements of swine. The substrates were composed by different set levels of substitution of basal diet by meal almond Acrocomia aculeata (AA), as: BAS - 100% of basal diet; M10 - 90% of basal diet and 10% AA (M10); M20 - 80% of basal diet and 20% AA (M20) and TF - Tifton 85 hay (Cynodon spp.).The gas production was evaluated at times 0, 3, 6, 9, 12, 16, 24, 48, 72 and 96 hours after incubation. The study analyzed the maximum potential of gas production (A) and lag time (L). The experimental design was completely randomized in a 4 x 3 factorial arrangement. The A and L data were submitted to analysis of variance and the averages compared by Tukey 5% test. Comparing the inoculum for A (ml / gDM), LRB, and CS, they showed similar values, differing only for TF. The lag time (L) between inoculum, the CSC and CSF had the lowest colonization times when the meal was BAS. With M10 and M20 substrates, the LRB inoculum showed the lowest time, CSF had the longest time and CSC did not differ from both. There was no significant difference between the inoculum for TF substrate. The gas production technique used for the ceca of pigs showed similar results to those for ruminants. The mathematical model used was adequate to describe the fermentation curve in the ceca of pigs, showing similarities between the microbiota of the ceca and the rumen.(AU)

Reduction of odours in pilot-scale landfill biocovers.

Unpleasant odours generated from waste management facilities represent an environmental and societal concern. This multi-year study documented odour and total reduced sulfur (TRS) abatement in four experimental landfill biocovers installed on the final cover of the Saint-Nicéphore landfill (Canada). Performance was evaluated based on the reduction in odour and TRS concentrations between the raw biogas collected from a dedicated well and the emitted gases at the surface. Odour analyses were carried out by the sensorial technique of olfactometry, whereas TRS analyses followed the pulse fluorescence technique. The large difference of 2-5 orders of magnitude between raw biogas (average odour concentration=2,100,000OUm(-3)) and emitted gases resulted in odour removal efficiencies of close to 100% for all observations. With respect to TRS concentrations, abatement efficiencies were all greater than 95%, with values averaging 21,000ppb of eq. SO2 in the raw biogas. The influence of water infiltration on odour concentrations was documented and showed that lower odour values were obtained when the 48-h accumulated precipitation prior to sampling was higher.

Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations (I): catastrophic APS, APS nephropathy and heart valve lesions.

The objectives of the 'Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations' were to assess the clinical utility of the international consensus statement on classification criteria and treatment guidelines for the catastrophic APS, to identify and grade the studies that analyse the relationship between the antiphospholipid antibodies and the non-criteria APS manifestations and to present the current evidence regarding the accuracy of these non-criteria APS manifestations for the detection of patients with APS. This article summarizes the studies analysed on the catastrophic APS, APS nephropathy and heart valve lesions, and presents the recommendations elaborated by the Task Force after this analysis.

Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations (II): thrombocytopenia and skin manifestations.

The objectives of the 'Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations' were to assess the clinical utility of the international consensus statement on classification criteria and treatment guidelines for the catastrophic APS, to identify and grade the studies that analyze the relationship between the antiphospholipid antibodies and the non-criteria APS manifestations, and to present the current evidence regarding the accuracy of these non-criteria APS manifestations for the detection of patients with APS. This article summarizes the studies analyzed on thrombocytopenia and skin manifestations, and presents the recommendations elaborated by the Task Force after this analysis.

Osteoarthritis and rheumatoid arthritis pannus have similar qualitative metabolic characteristics and pro-inflammatory cytokine response.

OBJECTIVE: Pannus in osteoarthritis (OA) has only recently been characterized. Little is known, however, regarding the behavior of OA pannus in vitro compared to rheumatoid arthritis (RA) pannus. The purpose of our study was to compare OA with RA pannus. METHODS: Pannus and synovial tissue co-cultures from 5 patients with OA and 5 patients with RA obtained during arthroplasty were studied. Pannus was defined as the microscopic invasive granulation tissue covering the articular surface. Tissues were cultured for 7 days and stained with Alcian Blue technique. Interleukin-1beta (IL-1beta), IL-8, IL-10, IL-12, tumor necrosis factor-alpha (TNF-alpha), and interferon gamma (IFN-gamma) were also determined in supernatants by ELISA. Cartilage oligomeric matrix protein (COMP), type II collagen, TNF-alpha, IL-10 and Ki-67 expression were also detected by immunohistochemistry. RESULTS: All patients had vascular or fibrous pannus. Synovial proliferation, inflammatory infiltrates and a decrease of extracellular matrix proteins were observed in all tissue samples. Chondrocyte proliferation was lower in OA than RA cartilage. OA synovial tissue expressed lower levels of proteoglycans than RA synoyium. Type II collagen levels were lower in OA than in RA cartilage. Significantly higher levels of IL-1beta were found in the supernatants of RA pannus compared to OA pannus (p<0.05). High but similar levels of TNF-alpha, IL-8 and TIMP-1 were detected in OA and RA pannus supernatants. IL-10, IL-12 and IFN-gamma were undetectable. CONCLUSION: RA and OA pannus had similar pro-inflammatory and anti-inflammatory cytokine profile expression. OA cartilage, synovial tissue and pannus had lower production of proteoglycans, type II collagen and IL-1beta. It remains to be elucidated why OA pannus invades the cartilage surface but does not cause the marginal erosions typically seen in RA.

Anti-citrullinated peptide antibodies in lupus patients with or without deforming arthropathy.

The objective was to study the association of antibodies against cyclic citrullinated peptides (anti-CCP) in patients with lupus articular damage. We studied 34 systemic lupus erythematosus patients (30 women) with (n = 14) or without (n = 20) deforming arthropathy. Anti-DNA and arthritis were mandatory inclusion criteria for both groups. As controls, 34 patients with rheumatoid arthritis and nine patients with rheumatoid arthritis and systemic lupus erythematosus (rhupus) were included. Anti-CCP and rheumatoid factor were determined by ELISA and nephelometry respectively. All patients had recent x-ray films of the hands that were evaluated according to Sharp's method. Systemic lupus erythematosus patients had a mean 6.50 +/- 0.86 (SD, range 5-8) American College of Rheumatology (ACR) criteria, rheumatoid arthritis patients met 5.38 +/- 0.60 (range 4-6) ACR criteria for rheumatoid arthritis and rhupus patients had 5.78 +/- 0.44 (range 5-6) criteria for rheumatoid arthritis and 5.11 +/- 0.78 (range 4-6) for systemic lupus erythematosus. Systemic lupus erythematosus patients, with or without deforming arthropathy, had normal serum anti-CCP concentrations. In contrast, rheumatoid arthritis and rhupus patients had 30- and 23-fold higher than normal amounts of anti-CCP (p < 0.001, both comparisons). Rheumatoid arthritis (97%) and rhupus (100%) patients were more frequently positive for anti-CCP than SLE patients with (7%) or without (5%) deforming arthropathy (p < 0.001, both comparisons). Patients with lupus deforming arthropathy were more frequently positive for rheumatoid factor (65%) than patients with non-deforming arthritis (15%) (p = 0.005). Patients with lupus deforming arthropathy had similar frequency of erosions and mean Sharp's score than rhupus patients. Anti-CCP antibodies do not associate with lupus arthropathy, whether deforming, non-deforming or erosive.

Anaerobic biodegradation of an organic by-products leachate by interaction with different mine tailings.

Deinking by-products from paper recycling have been used as cover materials on acid-producing tailings. Due to residual cellulose, anaerobic degradation leads to the production of an organic-rich leachate, which may percolate through the tailings. This study aims at describing the influence of the tailings' characteristics on the attenuation of organic matter in leachate and at discussing the degradation mechanisms. To this end, leachate was mixed with different types of tailings, including three unoxidized tailings with varying acid generation potentials and one tailing in three states of oxidation. Regularly, selected biochemical parameters were analyzed to access the evolution of organic components. The results show that when leachate from deinking by-products was placed in contact with tailings, phase and acid-base balance reactions took place in the beginning. Subsequently, oxidation-reduction reactions dominated the chemistry of the system. The type, the constituents, and the state of oxidation of the mine tailings condition the mechanisms of biodegradation of organic components. Methanogenesis was predominant in the control sample (pure organic leachate) but was absent in all leachate-tailings mixtures. No biodegradation was observed in the liquid phase for oxidized tailings and the organic concentration remained constant for unoxidized tailings, independently of the acid-generation potential. The biodegradation efficiency was optimal when the leachate was in contact with a mixture of oxidized and unoxidized tailings due to sulfate-reduction.

Heterogeneity of antibodies to beta2-glycoprotein 1 from patients with systemic lupus erythematosus.

We studied antibodies to beta2-glycoprotein 1 (anti-beta2GP1) from 72 patients with systemic lupus erythematosus (SLE) with or without antiphospholipid syndrome (APS) or with or without anticardiolipin antibodies (aCL). Fifteen patients had APS and positive antiphospholipid antibodies [clinical APS(+)/aPL(+)], 12 patients had APS, negative serum IgG and IgM aCL, antiphosphatidylethanolamine, anti-phosphatidylserine and no lupus anticoagulant [clinical APS(+)/ aPL(-)]. A third group included 16 patients without APS but high aCL levels [clinical APS(-)/ aPL(+)]. In a fourth group we studied 29 patients without clinical manifestations of APS or aCL [clinical APS(-)/aPL(-)]. One hundred anticardiolipin and VDRL-negative normal sera were studied as controls. IgG antibodies to cardiolipin proper in a bovine beta2GP-free system, to human beta2GP1 immobilized on cardiolipin or to human beta2GP1 alone were detected in all sera by ELISA using irradiated and nonirradiated plates from two manufacturers. Sera from APS(+)/aPL(+) patients showed IgG binding to CL, CL + beta2GP1 and beta2GP1 in irradiated and nonirradiated plates. APS(+)/ aPL(-) sera had more significant IgG binding to beta2GP1 than normal controls when studied in both irradiated or nonirradiated plates (P = 0.001). This binding was inhibited by solid-phase cardiolipin in a dose-dependent manner. Sera from the APS(-)/aPL(+) subgroup had comparable IgG activity in both the CL and CL + beta2GP1 assays, while no anti-beta2GP1 activity was detected in these sera. Sera from the clinical APS(-)/aPL(-) patients were negative in the three ELISA systems. Antibodies to human beta2GP1 from SLE patients recognize various epitopes. Those from APS(+)/ aPL(+) patients appear to react with an epitope boosted by cardiolipin in addition to another one present in the native protein. In contrast, anti-beta2GP1 from patients with APS(+)/aPL(-) are blocked by cardiolipin, suggesting that their epitope is the phospholipid-binding site.

The anti-phospholipid antibody syndrome: clinical and serological aspects.

The association of thromboses and/or cytopenias with anti-phospholipid antibodies (aPL), the anti-phospholipid syndrome (APS), is well recognized. The syndrome may be primary or occur within systemic lupus erythematosus (SLE). The notion of the syndrome occurring within SLE is important since patients found to have aPL may be at risk for developing APS manifestations, those who develop some manifestations may be at risk for developing others and, finally, SLE patients with this syndrome may need special treatment. There are subtle differences between the primary and the secondary forms, mostly due to the frequently higher and more persistent autoantibody levels in the primary and the influence of lupus in the secondary. These syndromes may be related to various antigen/antibody systems in which phospholipids participate either directly or through their effect on the proteins that bind them. Similar clinical manifestations also occur in patients who have serum antibodies to such proteins (e.g. beta2-glycoprotein-I) in the absence of phospholipid. Some of these antibodies may even be more important pathogenically than the antibodies against cardiolipin that were originally described. Testing for the latter is, however, still the first choice when suspecting an antiphospholipid syndrome. If this is negative in this situation, a search for the other autoantibodies is indicated.

Serum anti-beta2-glycoprotein-I and anticardiolipin antibodies during thrombosis in systemic lupus erythematosus patients.

PURPOSE: Antibodies to beta2-glycoprotein-I are more strongly associated with clinical antiphospholipid syndrome than are anticardiolipin antibodies. We previously found a decrease in anticardiolipin antibodies at the time of thrombosis in 6 patients with systemic lupus erythematosus (SLE). We therefore sought to determine the prevalence and levels of antibodies to beta2-glycoprotein-I and to cardiolipin before, during, and after thrombosis in patients with SLE, and to compare them with patients who did not have thrombosis. METHODS: We studied 24 patients with SLE who had at least one episode of thrombosis and 102 patients with SLE without thrombosis. Serum anticardiolipin antibodies were measured by conventional enzyme-linked immunosorbent assay (ELISA) using newborn calf serum as the blocking agent. Serum anti-beta2-glycoprotein-I antibodies were measured by ELISA on nonirradiated plates, using purified human beta2-glycoprotein-I without phospholipid. RESULTS: All patients with thrombosis had anti-beta2-glycoprotein-I antibodies, compared with only 17% of controls (P <0.0001). We observed a significant decrease in serum anti-beta2-glycoprotein-I levels at the time of thrombosis, as compared with previous and subsequent samples. The prevalence and levels of IgG and IgM anticardiolipin antibodies were similar in patients with and without thrombosis. A decrease in IgG or IgM anticardiolipin titers occurred during thrombosis in 6 patients. Anticoagulant, corticosteroid, and immunosuppressive treatments did not appear to affect anti-beta2-glycoprotein-I levels at the time of thrombosis. CONCLUSION: Anti-beta2-glycoprotein-I antibodies are strongly associated with thrombosis in patients with SLE. The decrease of anti-beta2-glycoprotein-I levels at the time of thrombosis may indicate a pathogenic role. This antibody may also be a marker of predisposition for thrombosis in these patients.

Autoantibodies in systemic lupus erythematosus.

Cationic germ line gene-encoded anti-DNA antibodies appear to cause inflammatory lesions via deposition and in situ formation of immune complexes, but also perhaps through apoptosis of glomerular mesangial cells. Somatic mutation is not critical for the expression of the electrical net charge of anti-DNA. In one transgenic mouse model, the nephrogenicity of anti-DNA was dependent on the expression of interleukin alpha by epidermal cells. Anti-P autoantibodies are present in the serum of healthy children but are masked by IgG antibodies. Some anti-P antibodies appear to be a subset of antilymphocyte autoantibodies. It was confirmed that anti-Ro/SS-A antibodies react with structurally unrelated conformational epitopes. The combined results of immunofluorescence and enzyme-linked immunosorbent assay in the detection of antineutrophil cytoplasmic antibody has a 99.5% specificity for vasculitis among patients with connective tissue diseases. Antinucleosome antibodies are highly prevalent in patients with systemic lupus erythematosus and show an inverse correlation with nucleosome plasma levels. Transfected T-cell receptor alpha chains specific for nucleosomal autoepitopes from a Th clone that accelerates lupus nephritis, recognized the nucleosomal epitopes presented by antigen presenting cell-bearing 1-A molecules, as well as human DR molecules, via the classical major histocompatibility complex class II groove. IgG antibodies against (beta 2 -glycoprotein (GP)-1 are significantly associated with thrombosis in patients with antiphospholipid syndrome, with a specificity and sensitivity that ranges from 85% to 98% and 32% to 54%, respectively. The lupus anticoagulant activity, or the lack of it, of anti-beta 2-glycoprotein-1 appears to reside on the epitope specificity. Beta 2-glycoprotein-1 binds to apoptotic cells; in turn, anti-beta 2-glycoprotein-1 facilitate apoptotic cell clearance by macrophages. The recently described anti-A2/RA33 autoantibody appears to recognize an epitope capable of distinguishing patients with lupus or with rheumatoid arthritis from those with mixed connective tissue disease.

Hidden anti-phospholipid antibodies in normal human sera circulate as immune complexes whose antigen can be removed by heat, acid, hypermolar buffers or phospholipase treatments.

Heat treatment of normal human serum reveals otherwise masked anti-cardiolipin antibodies (aCL). We studied the mechanism of masking and the nature of the inhibitor of these aCL IgG. Other forms of treatment, besides heating for 30 min at 56 degrees C, can also unmask hidden aCL IgG. These include acid pH, hypermolar buffers and phospholipase digestion. When unmasked, these aCL recognize other anionic and zwitterionic phospholipids, but do not react with DNA, cell antigens or IgG. Using thin layer chromatography we demonstrate that the heat-labile inhibitor(s) of these aCL are phosphatidylserine, phosphatidylethanolamine, phosphatidylglycerol and phosphatidylcholine. These antibodies are not beta2-glycoprotein-I dependent and actually compete with this protein for phospholipid binding. The hidden antibodies are comprised of two populations of IgG autoantibodies: one reactive with cardiolipin, phosphatidylserine, phosphatidylinositol, phosphatidylglycerol, phosphatidylethanolamine and sphingomyelin, and the other reactive almost exclusively with phosphatidylcholine and phosphorylcholine on enzyme-linked immunosorbent assay plates or when exposed by bromelain on the erythrocyte surface. Our data suggest that hidden aCL are natural oligoreactive IgG anti-phospholipid autoantibodies that circulate masked by their antigen.

Autoantibodies in systemic lupus erythematosus.

Research on pathogenic and nonpathogenic anti-DNA antibodies has shown that the charge and affinity for double-stranded DNA may not be adequate predictors of their pathogenicity. In turn, anti-DNA-induced renal damage could be caused by differences in fine specificities. Penetration of anti-double-stranded DNA monoclonal antibodies into live cells appears to require DNA or a DNA-like membrane receptor. Anti-U1 ribonucleoproteins penetrate live cells and induce apoptosis. HLA-DRB1*1501 or HLA-DRB1*1503, DQA1*0102, and DQB1*0602 haplotypes were increased in patients with systemic lupus erythematosus with anti-ribosomal protein P (anti-P) antibodies, particularly in whites, blacks, and Mexican-Americans. Anti-Ro/Sjögren's syndrome antigen A antibodies had a strong predictive diagnostic value for systemic lupus erythematosus among patients positive for antinuclear antibodies and negative for double-stranded DNA but had no utility among those positive for both antinuclear antibodies and anti-DNA antibodies. True antiphospholipid murine monoclonal antibodies display an unusual number of cationic residues in the H-chain CD3 region, whereas anti-beta 2-glycoprotein-I monoclonal antibodies lack them. The association of IgG anti-beta 2-glycoprotein-I with a history of thrombosis in systemic lupus erythematosus has been largely confirmed. Apoptotic thymocytes bind antiphospholipid antibodies in a beta 2-glycoprotein-I-dependent manner and may constitute a source of immunogen in autoimmune disease.

The antiphospholipid/cofactor syndromes. II. A variant in patients with systemic lupus erythematosus with antibodies to beta 2-glycoprotein I but no antibodies detectable in standard antiphospholipid assays.

OBJECTIVE: After the initial description of the anticardiolipin syndrome inpatients with systemic lupus erythematosus (SLE), it became clear that phospholipids other than cardiolipin could also be involved, that there could also be a primary syndrome, and that protein cofactors participate in in vitro reactivity of the autoantibodies. We describe 5 patients with SLE with clinical manifestations of antiphospholipid syndrome (APS) but persistently negative antiphospholipid antibodies (aPL). In all their tested sera we found antibodies to phospholipid-free beta 2-glycoprotein I (a beta 2-GPI). METHODS: We studied 5 patients with SLE with at least 2 clinical manifestations of APS with no serum aPL detected in routine assays. IgG and IgM a beta 2-GPI were studied by ELISA and by Western blot. We also tested for antibodies to phosphatidylcholine, phosphatidylethanolamine, and phosphatidylserine by ELISA. We studied 54 normal sera as controls. RESULTS: Four patients had livedo reticularis, 2 had thrombocytopenia, 2 had hemolytic anemia, and one each had recurrent venous thromboses, repeated fetal loss, pulmonary arterial hypertension, and transverse myelitis. No patient had serum aPL, but all had high titers of IgG a beta 2-GPI (p < 0.001 vs controls). Reactivity found in ELISA was confirmed by Western blot. CONCLUSION: We describe a variant of APS in patients with SLE with negative aPL but serum antibodies to beta 2-GPI.