A case of myeloperoxidase-antineutrophil cytoplasmic antibody and anticardiolipin antibody-positive pyogenic arthritis, pyoderma gangrenosum, acne and hidradenitis suppurativa (PAPASH) syndrome with colitis.

A previous case report of colitis and serine proteinase 3-antineutrophil cytoplasmic antibody positivity in pyogenic arthritis, pyoderma gangrenosum (PG), acne and hidradenitis suppurativa (PAPASH) syndrome with colitis has been published. Herein, we report a similar case of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) positivity. A 26-year-old man presented with recurrent aseptic pyogenic arthritis, acne, hidradenitis suppurativa and PG. Lower gastrointestinal endoscopy was performed, and colitis was observed. No PSTPIP1 gene mutation was found in the gene-sequencing test. Based on these findings and prior case reports, we diagnosed the patient with PAPASH syndrome, a PAPA spectrum disorder complicated by colitis. This patient had PAPASH syndrome with colitis and was MPO-ANCA and anticardiolipin antibodies-positive; it is unclear whether these antibodies play a role in this disease, but it may provide clues to further elucidate its pathogenesis.

Presencia de anticuerpos antifosfolípidos en niños con fiebre y petequias / Presence of antiphospholipid antibodies in children with fever and petechiae

Introducción: La presencia de un tiempo de cefalina (APTT) alargado en niños con fiebre y petequias es un hallazgo descrito en la bibliografía. La causa de esta alteración se desconoce, pero se postula que puede deberse a la formación de anticuerpos antifosfolípidos (Ac AFL). El objetivo de este estudio es determinar si el alargamiento del APTT se asocia con la formación Ac AFL. Pacientes y métodos: Estudio observacional, prospectivo, de casos y controles, realizado en niños que consultaron por fiebre y petequias en el servicio de urgencias de un hospital de tercer nivel durante un periodo de 13 meses. Se recogieron variables epidemiológicas, clínicas y analíticas. Se describieron las características de los grupos con APTT alargado y normal, y se comparó la asociación entre las concentraciones de los diferentes anticuerpos y el APTT. Resultados: Se incluyeron 36 pacientes, 12 casos y 24 controles. No se encontraron diferencias significativas respecto a la positividad de Ac AFL entre los casos y los controles (odds ratio [OR]= 1,67; intervalo de confianza [IC] del 95%: 0,31-9,04). No se observó ninguna asociación entre los diferentes tipos de anticuerpos y el APTT, cuyos coeficientes de regresión fueron de 0,04 seg (IC del 95%: -0,31 a 0,40) para anticardiolipina IgG, de 1,11 seg (IC del 95%: -1,24 a 3,46) para la IgM, y de -0,02 seg (IC del 95%: -0,35 a 0,31) y 0,64 seg (IC del 95%: -1,40 a 2,68) para antibeta 2 GPI, IgG e IgM, respectivamente. Conclusión: Ante los resultados de nuestro estudio, no podemos concluir que el alargamiento de APTT se relacione con la presencia de Ac AFL (AU)

Introduction: The presence of a longer time of cephalin (APTT) extended in children who come to emergency department with fever and petechiae is a result previously described in the literature. The cause of this alteration in coagulation is unknown, it is presumed that may be due to the formation of antiphospholipids antibodies. The aim of this study is to determine if the length of APTT is associated to the formation of antiphospholipids antibodies. Patients and methods: Observational, prospective case-control study in children who consulted for fever and petechiae in the emergency department of a tertiary hospital over a 13-month period epidemiological; clinical and laboratory variables were collected. The characteristics of groups with elongated and normal APTT were described and the association between concentrations of different antibodies and APTT were compared. Results: 36 patients, 12 cases and 24 controls, were included. No significant differences were found regarding the positivity of antiphospholipid antibodies between cases and controls (OR= 1.67; 95%CI: 0.31 to 9.04). No association was observed between the different types of antibodies and APTT, resulting regression coefficients in 0.04 s (95%CI: -0.31 to 0.40) for cardiolipin IgG, 1.11 s (95%CI: -1.24 to 3.46) for IgM and -0.02 s (95%CI: -0.35 to 0.31) and 0,64 s (95%CI: -1.40 to 2.68) for antibeta 2 GPI, IgG and IgM, respectively. Conclusion: Given the results of our study we can not conclude that the elongation of APTT is related with the presence of antiphospholipids antibodies (AU)

Antiphospholipid antibodies in Mexican HIV-positive patients

Several studies have shown that HIV patients tend to develop autoimmune diseases, and have numerous antibodies, such as antiphospholipid antibodies. Antiphospholipid antibodies are the serological markers used in the diagnosis of the antiphospholipid syndrome. However, antiphospholipid antibodies also appear to exist in infectious diseases. Objective: TomeasurethetitersofantiphospholipidantibodiesinhealthyandinHIVpositiveMexican mestizo patients, and correlate them with the patient clinical manifestations to identify possible findings compatible with an autoimmune disease. Material and methods: A case control study was conducted on 50 healthy mixed race Mexican subjects andin50 randomly selectedHIV-positive patients from the (..) (AU)

Varios estudios han demostrado que los pacientes con VIH tienden a desarrollar enfermedades autoinmunes, presentando diversos anticuerpos como los anticuerpos antifosfolípidos. Los anticuerpos antifosfolípido son los marcadores serológicos que se emplean en el diagnóstico del síndrome antifosfolípido. Sin embargo los anticuerpos antifosfolípido también suelen existir en enfermedades infecciosas específicas. Objetivo: Medir los títulos de anticuerpos antifosfolípido en pacientes mexicanos mestizos sanos y VIH positivos y correlacionarlos con las manifestaciones clínicas de los pacientes para identificar posibles hallazgos compatibles con alguna enfermedad autoinmune. Material y métodos: Se trata de un estudio de casos y controles en el que se evaluaron (..) (AU)

Plasma exchange and immunoadsorption effectively remove antiphospholipid antibodies in pregnant patients with antiphospholipid syndrome.

Conventional therapy with aspirin and/or heparin is at times incapable of preventing complications in high risk pregnancies of patients with antiphospholipid syndrome (APS). In those cases, a so-called second-line treatment protocol is used in addition to conventional therapy strategies. This manuscript is a report on three APS pregnant patients who were successfully treated with plasma exchange (PE) (two cases) or with immunoadsorption (IA) (one case) as a second-line treatment strategy. The efficacy of these procedures in removing anticardiolipin (aCL) and anti-ß(2)glycoprotein I (aß(2)GPI) antibodies from blood was evaluated. Serum samples were collected before and after 87 apheretic treatment sessions. Serum IgG/M aCL and IgG/M aß(2)GPI antibodies were determined using an "in-house" enzyme-linked immunosorbent assay and showed that all three patients had medium/high IgG aCL and aß(2)GPI titers. All three women had a successful pregnancy. A significant decrease in IgG aCL (P = 0.0001) and aß(2)GPI (P = 0.0001) antibody titers was observed after PE and IA sessions. There was moreover a significant, steady fall in serum IgG aCL pretreatment levels during the course of all three pregnancies (P = 0.0001, P = 0.0001, P = 0.001). The fall in IgG aß(2)GPI was significant in two of the patients (P = 0.0001, P = 0.0001) both with high antibody titers, but not in one with medium antibody titers, who was treated with PE (P = 0.17).

Estrategias terapéuticas en el síndrome antifosfolipídico / Therapeutic strategies in antiphospholipid syndrome

El síndrome antifosfolipídico (SAF) se caracteriza por la asociación de los anticuerpos antifosfolipídicos (AAF) con trombosis de repetición, abortos o pérdidas fetales recurrentes y trombocitopenia. Los AAF más estudiados son los anticuerpos anticardiolipina, el anticoagulante lúpico y los anticuerpos anti-ß2-glucoproteína I. El SAF puede presentarse de forma aislada, denominándose SAF primario, o bien asociado a otras enfermedades autoinmunes sistémicas, fundamentalmente a lupus eritematoso sistémico. Más recientemente, se ha descrito un subgrupo de SAF en el que los pacientes desarrollan múltiples trombosis durante un corto espacio de tiempo, que se ha denominado SAF catastrófico. Aunque parece clara la asociación entre la presencia de AAF y trombosis, la actitud terapéutica no debe ir dirigida primariamente a la eliminación o a la reducción de los niveles de estos anticuerpos mediante recambios plasmáticos, gammaglobulinas intravenosas o inmunodepresores (excepto en el SAF catastrófico), ya que no existe una clara correlación entre los niveles de los AAF y los episodios trombóticos. El tratamiento de estos pacientes debe basarse en el uso de antiagregantes plaquetarios o anticoagulantes(AU)

The classical clinical picture of the antiphospholipid syndrome (APS) is characterized by venous or arterial thromboses, fetal losses and thrombocytopenia, in the presence of antiphospholipid antibodies (aPL), namely lupus anticoagulant, anticardiolipin antibodies or antibodies directed to various proteins, mainly â2 glycoprotein I, or all three. Apart from being “primary” (without any discernable underlying systemic autoimmune disease), or associated to another disease (usually to systemic lupus erythematosus), it may also occur rapidly over days or weeks when it has been termed “catastrophic” APS. Therapy should not primarily be directed at effectively reducing the aPL levels and the use of immunotherapy (including high dose steroid administration, immunosuppression or plasma exchange) is generally not indicated, unless in the catastrophic APS. Treatment of APS patients should be based on the use of antiaggregant and anticoagulant therapy(AU)

Clinical significance of antiphospholipid syndrome nephropathy (APSN) in patients with systemic lupus erythematosus (SLE) / Significado clínico de la nefropatía del síndrome antifosfolípido en pacientes con lupus eritematoso sistémico (LES)

La nefropatía del síndrome anti fosfolípido (NSAF) es actualmente una alteración patológica bien definida, caracterizada por la presencia de lesiones renales vaso-oclusivas, trombosis aguda arterial y arteriolar, y que ocasiona zonas de atrofia isquémica cortical. El objetivo del presente trabajo fue analizar la prevalencia y el significado clínico de la NSAF en pacientes con glomerulonefritis (GN) secundaria a Lupus Eritematoso Sistémico (LES). Se analizaron retrospectivamente las biopsias renales de 162 pacientes con GN secundaria a LES, buscando intencionadamente los datos histopatológicos de la NSAF. Se registraron los datos clínicos y serológicos al momento de la biopsia renal y durante el período de seguimiento promedio de 7 años. En los casos en que se obtuvo una biopsia renal subsecuente se analizó el desarrollo de la NASF. Resultados: Encontramos datos de NSAF en 17 pacientes (10.4%); 12 de ellos tenían lesiones proliferativas focales o difusas. Los índices histopatológicos de actividad y de cronicidad fueron más altos en los pacientes con la NSAF cuando se compararon con los pacientes sin NSAF. Los pacientes con nefropatía anti fosfolípido tuvieron con mayor frecuencia hipertensión arterial, creatinina sérica elevada, síndrome nefrótico, GN rápidamente progresiva y muerte, en comparación con los pacientes con GN lúpica sin NSAF. Se detectaron anticuerpos anticardiolipina en 52% de los pacientes con NSAF en quienes se realizó el examen al momento de la biopsia, en comparación con 27% de los pacientes sin NSAF. Se realizó biopsia renal subsecuente en 18 pacientes; quienes tuvieron NSAF en la primera biopsia tuvieron mayor incremento en la esclerosis glomerular en la segunda biopsia, al compararlo con quienes no tuvieron NSAF en la biopsia inicial. Conclusiones: La nefropatía del antifosfolípido es un factor de riesgo para hipertensión arterial, síndrome nefrótico y GN rápidamente progresiva en los pacientes con GN lúpica. La NSAF debiera considerarse en los criterios de clasificación del síndrome anti fosfolípido, y sería recomendable realizar estudios con tratamiento anticoagulante en estos pacientes (AU)

Antiphospholipid syndrome nephropathy (APSN) is now a well-recognized vaso-occlusive renal lesion associated with acute thrombosis and chronic arterial and arteriolar lesions, leading to zones of cortical ischemic atrophy. Our objective was to evaluate the prevalence and clinical significance of APSN in patients with Systemic Lupus Erythematosus (SLE). Methods Kidney biopsy specimens obtained from 162 patients with lupus glomerulonephritis were retrospectively examined for the presence of APSN. Clinical and laboratory data obtained at the time of kidney biopsy and during a mean follow-up of 7 years were recorded. In cases for which serial kidney biopsy specimens were available, the evolution of APSN was examined. Results We found APSN in 17 (10.4%) patients with lupus glomerulonephritis (GN), 12 with focal or proliferative lesions. Both activity and chronicity indexes were higher in patients with APSN when compared with lupus nephritis without APSN. Patients with APSN had a higher frequency of hypertension and elevated serum creatinine levels at the time or kidney biopsy, as well as a higher frequency of rapidly progressive GN, nephrotic syndrome and death at the end of the follow-up. Anticardiolipin antibodies were found in 52% of those with APSN and in 27% of those without APSN. Serial kidney biopsy specimens were available from 18 patients. An increase of glomerular sclerosis was found in the second biopsy particularly in those patients with APSN in the first biopsy. Conclusions APSN is a risk factor that contributes to an elevated prevalence of hypertension, elevated serum creatinine, nephrotic syndrome and increased glomerular sclerosis. APSN should be included in the classification criteria of APS, and the use of appropriate anticoagulant therapy should be tested (AU)

A protocol for determination of anticardiolipin antibodies by ELISA.

The anticardiolipin (aCL) test has been widely used by physicians since the mid-1980s for diagnosing patients with antiphospholipid syndrome (APS). Establishment of this diagnosis has enabled effective management of patients with recurrent thrombosis or recurrent pregnancy losses. The test was first established in 1983 as a radioimmunoassay and soon thereafter converted into ELISA. There have been numerous efforts to standardize the aCL test, but precise reproducible measurement of aCL levels is difficult and the use of semiquantitative measurements (high, medium and low) is recommended as this is probably sufficient for clinical diagnosis. Using validated ELISAs for measuring aCL Abs offers greater reproducibility, would reduce interlaboratory variations and limit discrepancies in results between different laboratories. This article details a procedure that takes approximately 2 h and summarizes the information available on the aCL ELISA test.

Utilidad clínica de las pruebas inmunológicas especializadas en reumatología en un hospital de segundo nivel de atención en México / Clinical utility of specialized immunologic testing in rheumatology in a secondary level hospital in Mexico

Introducción: El laboratorio en reumatología tiene un papel importante en la evaluación, el diagnóstico y el seguimiento de diversos padecimientos. Los anticuerpos antinucleares (ANA), los anticuerpos anti-ADN de cadena sencilla o doble (ss o dsAnti-ADN) y anticuerpos anticardiolipínicos (AcACL) se usan frecuentemente y su utilidad diagnóstica es bien conocida en centros de atención de tercer nivel. Nuestro hospital es un centro de atención de segundo nivel que implementó hace 2 años estas pruebas. Después de 1 año de su introducción, decidimos evaluar la frecuencia en su uso, quién solicita estas pruebas, su utilidad diagnóstica en lupus eritematoso generalizado (LEG) y síndrome antifosfolipídico (SAF). Pacientes y método: Se evaluó a todos los pacientes con cuadro clínico de estas enfermedades y solicitud de estas pruebas del 1 de septiembre de 2005 al 30 de junio de 2006. De manera prospectiva, los analizó un evaluador con un formato estandarizado que contenía información clínica, diagnóstico inicial, datos del médico solicitante, servicio, diagnóstico tras resultado y los cambios en la terapéutica. Análisis estadístico: se utilizó estadística descriptiva y tablas de 2 × 2 para evaluar la utilidad diagnóstica en las indicaciones más comunes. Resultados: De un total de 286 solicitudes recibidas, se analizaron 157. Reumatología y medicina interna enviaron 63 y 31 solicitudes respectivamente. Con respecto a los ANA en LEG, se calculó la sensibilidad (70%); la especificidad (92%); el valor predictivo positivo (VPP) (81%); el valor predictivo negativo (VPN) (86%); la razón de verosimilitud positiva (RVsP) (8,73) y la razón de verosimilitud negativa (RVsN) (0,33). En relación con los anti-ADN en LEG, la sensibilidad (78%); la especificidad (50%); el VPP (80%); el VPN (46%); la RVsP (1,56) y la RVsN (0,44). Respecto a los AcACL en SAF, la sensibilidad (78%); la especificidad (92%), el VPP (78%), el VPN (92%), la RVsP (10) y la RVsN (0,24). Conclusiones: En nuestro hospital hay poca frecuencia en la solicitud de estos estudios. La sensibilidad y la especificidad parecen no estar acordes con lo publicado. Es necesaria la elaboración de lineamientos que en nuestro medio regulen la solicitud de estudios especializados en reumatología y aumenten su utilidad clínica (AU)

Introduction: Laboratory tests have an important role in rheumatology for evaluation, diagnosis and follow up in several diseases. Specialized tests such as antinuclear antibodies (ANA), anti single or double stranded DNA antibodies (anti DNA) and anticardiolipin antibodies (AcACL) are frequently used and its diagnostic performance is well known in tertiary care centers. Our setting is a secondary care center that implemented these tests two years ago. After one year of implementation, we decided to evaluate the frequency of use, who orders these tests, and their diagnostic properties for the diagnosis of systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APLS). Patienst and method: All patients with clinical charts and a request for these tests were evaluated from September 1, 2005 to June 30, 2006. These evaluations were done prospectively by a single, trained evaluator following a standardized format looking at pretest clinical information such as pretest diagnosis, physician’s level of training, service and posttest results as well as therapeutic changes after results. Statistical analysis: descriptive statistics and 2 by 2 tables to estimate diagnostic performance of most common indications. Results: Two hundred and eighty six requests were reviewed and only 157 were evaluated. Rheumatology and Internal Medicine services sent 63 and 31 requests for these tests respectively. Diagnostic properties of ANA for SLE were sensitivity (sen) 70%, specificity (spec): 92%, positive predictive value (PPV): 81%, negative predictive value (NPP): 86%, positive likelihood ratio (PLR): 8.73 and negative likelihood ratio (NLR): 0.33. Anti double stranded DNA Sen: 78%, spec: 50%, PPV: 80%, NPP: 46%, PLR: 1.56, NLR: 0.44. ACACL Sen: 78% spec: 92%, PPV: 78%, NPV 92%, PLR: 10, NLR 0.24. Conclusions: These specialized tests are not frequently used in our setting. Their diagnostic properties are not as accurate as those published in medical literature. Guidelines are needed in our hospital to improve their diagnostic performance (AU)

Vasculite livedóide com anticorpo antifosfolipídio / Livedoid vasculitis with antiphospholipid antibodies

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The authors report a case of a 79-year-old woman presenting livedoid vasculitis. This is a painfull clinical entity, characterized by purpuric lesions with shaped ulcers and atrophie blanche. Raised anticardiolipin antibody titers are associated at the present case. The ethiopatogenic mecanisms and the different denominations for the disease are discussed

Anticuerpos anticardiolipinas dependientes de Beta2 glicoproteína I y significado clínico / Beta2 glycoprotein I dependent anticardiolipin antibodies and clinical features

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Development of an in vitro miniature model to simulate immunoadsorption in patients with systemic lupus erythematosus.

In SLE, immunoadsorption is used as an adjuvant therapy; however, adsorption profiles and binding mechanisms have not yet been completely investigated. Using a minicolumn filled with the sorbent IMPH with or without the ligand phenylalanine, we developed a model simulating clinical conditions in a reduced scale with a constant ratio of plasma to column volume and a constant plasma flow at room temperature. By desorbing the column, the adsorption efficacy for different antibodies could be measured directly. We demonstrate that the adsorption rate can be increased by a low plasma flow and by covering the column surface. Double perfusion of the same column did not increase the amount of adsorbed antibodies. We further demonstrate that the carrier material without a ligand is unable to bind antibodies or protein. In the IMPH sorbent anti-dsDNA antibodies were significantly better adsorbed than total IgG or total protein. After a single perfusion of 21 samples, we estimated a mean anti-dsDNA antibody adsorption rate of 22.5% (+/-13.6). A group of ten responders with a medium adsorption rate of 35.4% (+/-6.5) clearly differed from a second group of eleven nonresponders (10.9% +/- 4.2). Anti-cardiolipin antibodies (ACA) were adsorbed in a wide range (IgG type, 2.5-52.7%, IgM type, 1.1-37.8%) while anti-Ro (SSA) antibody adsorption was negligible. This in vitro minimodel provides a precise simulation of therapeutic immunoadsorption and helps to analyze the binding characteristics of the sorbent IMPH and shows its effectiveness in several antibody subsets of different patients.

Immunoglobulin G from patients with antiphospholipid syndrome impairs the fibrin dissolution with plasmin.

Immunoglobulin G (IgG) isolated from normal human blood plasma stabilizes the structure of perfused crosslinked fibrin and prolongs the time for its dissolution with plasmin, when the fibrin surface is exposed to 500 s(-1) shear rate flow. The IgG from patients suffering in antiphospholipid syndrome with thrombotic complications exerts even stronger antifibrinolytic effect. A patient, whose IgG does not affect the fibrin dissolution with plasmin, displays a bleeding tendency. The shear stress-induced disassembly of the fibrin clots containing IgGs with antifibrinolytic potency occurs at a much more advanced stage of fibrin digestion, as evidenced by the electrophoretic pattern of the ureatreated samples. The antifibrinolytic effects are also produced under static conditions and these are caused by the variable portion of the IgG molecules (fragment Fab), whereas the constant part (fragment Fc) has no inhibitory effect. The IgGs with antifibrinolytic properties do not affect directly the plasmin activity in amidolytic assay, but the IgGs from APS patients obliterate the competition of the fibrin and the peptidyl-p-nitroanilide for the protease in the same assay system suggesting interference of the IgGs with the plasmin action on the fibrin substrate. Thus, the correlation of the clinical symptoms with the effect of the isolated IgG on the dissolution of perfused fibrin clots supports a physiological and a pathological role of IgG in the fibrinolytic process related to the variability of the cross-reactions of immunoglobulins with fibrin, fibrin degradation products or fibrin-plasmin complexes.

Antiphospholipid syndrome associated with intestinal amoebiasis.

We present a case of intestinal amoebiasis with subsequent development of antiphospholipid syndrome, manifested by deep vein thrombosis and pulmonary emboli. Anticardiolipin antibodies (aCL) of IgM type at medium titer and aCL IgG antibody at low titer were determined during the days after the onset of infection. To our knowledge this is the first case of antiphospholipid syndrome associated with amoebiasis to be presented in the literature.

Polyclonal anticardiolipin antibodies purified from sera of patients with active systemic lupus erythematosus induce apoptosis of the cultured glomerular mesangial cells.

OBJECTIVE: To test the effect of anticardiolipin antibodies (aCL) on cultured glomerular mesangial cells with regard to their expression of apoptosis-related genes. METHODS: aCL purified from active lupus sera by cardiolipin micelles were incubated with cultured rodent mesangial cells (RMC). Morphological changes of the RMC were observed. The genomic DNA was extracted for the detection of apoptosis. The total cell RNA was extracted for detection of Fas, c-myc, p53, and bcl-2 transcripts by reverse transcription-polymerase chain reaction. RESULTS: aCL (100 GPL-U/0.1 mg protein/ml) bound to RMC more prominent than human IgG (100 microg/ml). The antibodies suppressed RMC proliferation in a dose-dependent manner. The RMC were undergoing apoptosis as evidenced by morphologic changes, fluoresceinannexin V staining and appearance of nucleosome-sized DNA fragments. RMC spontaneously express p53 and c-myc but not Fas or bcl-2. aCL (100 GPL-U/ml) enhanced the expression of Fas but not other apoptosis-related genes and suppressed the intracellular tyrosine phosphorylation. CONCLUSIONS: Binding of aCL can induce apoptosis of the RMC. The aCL may be implicated in the pathogenesis of lupus nephritis.

Affinity purified human antiphospholipid antibodies bind normal term placenta.

Antiphospholipid antibodies (aPL) are associated with an increased incidence of fetal loss, but the pathophysiology remains unclear. One mechanism may involve the binding of aPL directly to the placenta where they may initiate placental thrombosis and infarction. We have developed an immunofluorescent technique to detect human aPL binding to human placenta. Endogenous immunoglobulins were eluted by extensive washing and residual staining was prevented by incorporating multiple blocking steps. APL were affinity purified on both cardiolipin and phosphatidylserine liposomes from the sera of six patients with aPL (five antiphospholipid syndrome (APS) patients and one post bone marrow transplant patient). Heterogeneous binding to normal term placenta, involving either the trophoblast microvillous surface, stromal and peri-vascular regions was demonstrated by affinity purified aPL from five of six patients. Preliminary sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE) and western blotting studies have demonstrated that aPL bind a number of placental proteins. beta2GPI was not the predominant protein bound by aPL using this technique. This study provides further evidence for the involvement of aPL in mediating placental damage.

Primary antiphospholipid syndrome presented by total infarction of right kidney with nephrotic syndrome.

We report the case of a young woman with primary antiphospholipid syndrome (APS), which presented with acute renal failure, hypoproteinemia, hypoalbuminemia and nephrotic proteinuria. Investigations showed total infarction of right kidney by extensive arterial and vein thrombosis and presence of anticardiolipin antibodies IgG isotype (anti-beta2-glycoprotein I-positive). She was submitted to right nefrectomy and initiated anticoagulant therapy. After nefrectomy, the postoperative period was marked by the development of arterial hypertension and persistence of nephrotic syndrome. Hypertension was treated with antihypertensive drugs (IECA, beta-blocker and calcium antagonist). As the nephrotic syndrome persisted despite anticoagulant and antihypertensive therapy, the patient was treated with oral corticosteroids. Her renal function improved, hypoproteinemia and hypoalbuminemia corrected to normal values and proteinuria decreased to subnephrotic value. We discuss the unusual presentation of this case of primary antiphospholipid syndrome with total unilateral renal thrombosis and nephrotic syndrome that respond to anticoagulant, antihypertensive and corticosteroid therapy.

A chemically defined, toleragen-based approach for targeting anti-beta2-glycoprotein I antibodies.

Antiphospholipid syndrome is characterized by a prothrombotic state and the presence of beta2-glycoprotein I (beta2-GPI)-dependent antiphospholipid antibodies. The feasibility of a B cell tolerance-based approach for specific reduction of anti-beta2-GPI antibodies was investigated. Anti-beta2-GPI antibodies isolated from a patient with antiphospholipid syndrome were used to screen peptide libraries expressed in phage, resulting in the identification of a phage that specifically bound anti-beta2-GPI antibodies. The phage-displayed peptide was identified and chemically optimized to generate a synthetic 14-mer peptide with an internal thioether linkage (LJP 685) that retained the binding profile of the original phage. LJP 685 was conjugated to a defined, non-immunogenic organic platform to generate a tetravalent presentation of LJP 685 for use as a toleragen. Tetravalent LJP 685 induced a dose-dependent reduction in antibody levels in mice previously immunized and boosted with LJP 685 coupled to the carrier keyhole limpet hemocyanin. These experiments support the technical feasibility of a tolerance-based approach for reducing anti-beta2-GPI antibodies in vivo.

Anticardiolipin antibody in recurrent spontaneous aborting and fertile women

Objective: to determine the association between the presence of anticardiolipin antibody and a history of recurrent spontaneous abortion. Study design: clinical controlled study Location: Department of Gynecology and Obstetrics - University of Campinas (UNICAMP) Subjects: 52 individuals with recurrent spontaneous abortion were included in Group 1 and 104 individuals with at least one live born child in Group 2. Elapsed time from last delivery to blood sampling varied from six months to two years. Method: Between November 1993 and November 1994, patients'blood samples were screened for anticardiolipin antibody by ELISA, as described by Triplett, Barna and Unger (1993). Analysis: Chi-square and Fisher's Exact tests were used for statistical analysis. Student's "t" test was used to compare the means. Results: There was no statistical difference in the presence of the anticardiolipin antibody between Group I (zero and 2.9 per cent) and Group 2 (7.7 and 5.8 per cent). Concluison: There was no association between the presence of anticardiolipin antibody and recurrent spontaneous abortion.

Síndrome de Sneddon e anticorpo anticardiolipina / Sneddon's syndrome and antibody anticardiolipin

Os autores apresentam o caso de uma paciente com 34 anos de idade, com cinco eventos de abordamento, fetal, livedo reticular, acidente vascular cerebral e positividade para os anticorpos anticardiolipina idiotipos IgG e IgM. A paciente preenche os critérios par síndrome Sneddon, associado às manifestaçoes clínicas e positividade para os anticorpos anticardiolipina. Chama-se atençao para a investigaçao dos anticorpos anticardiolipina na síndrome de Sneddon.