JoVE Visualize What is visualize?
Stop Reading. Start Watching.
Advanced Search
Stop Reading. Start Watching.
Regular Search
Find video protocols related to scientific articles indexed in Pubmed.
Proinflammatory cytokines and C-reactive protein in uveitis associated with Behçet's disease.
Mediators Inflamm.
PUBLISHED: 04-07-2014
Show Abstract
Hide Abstract
The aim of the present study was to determine the serum cytokine profile and levels of high sensitivity C-reactive protein (hsCRP) in patients with uveitis associated with Behçet's disease (BD) and to compare them with those obtained from healthy control subjects. We determined the serum concentration of interferon-? (IFN-?), interleukin-1? (IL-1?), IL-12p70, IL-17A, tumor necrosis factor-? (TNF-?), and hsCRP in 13 patients with active uveitis associated to BD, 24 inactive BD patients, and 20 controls. In a subgroup of 10 active patients, a second serum sample was obtained when the disease was inactive. Cytokine profiles and hsCRP levels were correlated with disease activity, severity, complications, and visual outcome. Levels of IFN-? and TNF-? were significantly increased in patients with active uveitis associated to BD compared to controls (P < 0.05). IFN-?, TNF-?, and hsCRP were significantly higher during active uveitis associated to BD compared to inactive disease (P < 0.05). Furthermore, IL-17A was significantly increased in patients with active BD without pharmacological treatment compared to controls (P < 0.05). No significant correlations were found with specific cytokine profiles and disease severity, visual outcome, or complications. In summary, increased serum levels of IFN-?, TNF-?, IL-17A, and hsCRP were associated with active uveitis associated with BD and might serve as markers of disease activity.
Related JoVE Video
Early- versus late-onset systemic sclerosis: differences in clinical presentation and outcome in 1037 patients.
Medicine (Baltimore)
PUBLISHED: 03-21-2014
Show Abstract
Hide Abstract
Peak age at onset of systemic sclerosis (SSc) is between 20 and 50 years, although SSc is also described in both young and elderly patients. We conducted the present study to determine if age at disease onset modulates the clinical characteristics and outcome of SSc patients. The Spanish Scleroderma Study Group recruited 1037 patients with a mean follow-up of 5.2 ± 6.8 years. Based on the mean ± 1 standard deviation (SD) of age at disease onset (45 ± 15 yr) of the whole series, patients were classified into 3 groups: age ? 30 years (early onset), age between 31 and 59 years (standard onset), and age ? 60 years (late onset). We compared initial and cumulative manifestations, immunologic features, and death rates. The early-onset group included 195 patients; standard-onset group, 651; and late-onset, 191 patients. The early-onset group had a higher prevalence of esophageal involvement (72% in early-onset compared with 67% in standard-onset and 56% in late-onset; p = 0.004), and myositis (11%, 7.2%, and 2.9%, respectively; p = 0.009), but a lower prevalence of centromere antibodies (33%, 46%, and 47%, respectively; p = 0.007). In contrast, late-onset SSc was characterized by a lower prevalence of digital ulcers (54%, 41%, and 34%, respectively; p < 0.001) but higher rates of heart conduction system abnormalities (9%, 13%, and 21%, respectively; p = 0.004). Pulmonary hypertension was found in 25% of elderly patients and in 12% of the youngest patients (p = 0.010). After correction for the population effects of age and sex, standardized mortality ratio was shown to be higher in younger patients. The results of the present study confirm that age at disease onset is associated with differences in clinical presentation and outcome in SSc patients.
Related JoVE Video
Renal transplantation in systemic lupus erythematosus: outcome and prognostic factors in 50 cases from a single centre.
Biomed Res Int
PUBLISHED: 03-17-2014
Show Abstract
Hide Abstract
End-stage renal disease (ESRD) is an important cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE).
Related JoVE Video
14th International Congress on Antiphospholipid Antibodies Task Force Report on Catastrophic Antiphospholipid Syndrome.
Autoimmun Rev
PUBLISHED: 02-15-2014
Show Abstract
Hide Abstract
The 'Task Force on Catastrophic Antiphospholipid Syndrome (CAPS)' was developed on the occasion of the 14th International Congress on Antiphospholipid Antibodies. The objectives of this Task Force were to assess the current knowledge on pathogenesis, clinical and laboratory features, diagnosis and classification, precipitating factors and treatment of this condition in order to address recommendations for future research. This article summarizes the studies analyzed by the Task Force, its recommendations and the future research agenda.
Related JoVE Video
Registry of the Spanish network of Behçet's disease: a descriptive analysis of 496 patients.
Clin. Exp. Rheumatol.
PUBLISHED: 01-30-2014
Show Abstract
Hide Abstract
To describe the clinical features of a large cohort of 496 Spanish patients with Behçet's disease (BD) and to analyse if patient's sex influenced the initial and cumulated prevalence of disease manifestations.
Related JoVE Video
[Efficacy and safety of rituximab in the treatment of primary antiphospholipid syndrome: Analysis of 24 cases from the bibliography review.]
Med Clin (Barc)
PUBLISHED: 01-22-2014
Show Abstract
Hide Abstract
Antiphospholipid syndrome (APS) is characterized by the presence of antiphospholipid antibodies (aPL) and thrombotic and/or obstetric manifestations. Patients without another associated autoimmune disease are considered to have primary APS. Some patients develop thrombosis recurrence despite anticoagulant treatment and some clinical features do not respond to standard therapy. Rituximab may be an alternative in these cases. We review the published scientific evidence on the use of rituximab in the treatment of primary APS.
Related JoVE Video
Endogenous thrombin potential in Behçet's disease: relationship with thrombosis and anticoagulant therapy.
Clin. Exp. Rheumatol.
PUBLISHED: 01-04-2014
Show Abstract
Hide Abstract
To analyse the relationship between an automated thrombin generation test, the endogenous thrombin potential (ETP), and other hypercoagulability markers, with vascular involvement in patients with Behçet's disease (BD). Patients and methods. We analysed 56 BD patients (30 men; mean age, 34.4 ± 14.3 years) without any known thrombophilic factor, of which 17 had previously suffered from thrombosis (deep venous thrombosis in 14 and ischaemic stroke in 3), and 56 controls matched for age and sex. Additionally, we also evaluated 20 plasma samples with an international normalised ratio (INR) between 1.5 and 5.0 obtained from patients with atrial fibrillation but without a history of embolic events that were under treatment with acenocumarol. Thrombin generation was measured as ETP with a chromogenic assay in an automated analyser. Factor VIII, von Willebrand factor antigen, prothrombin fragment 1.2, D-dimer and plasmin-antiplasmin complexes were also measured.
Related JoVE Video
Epistatic interaction of ERAP1 and HLA-B in Behçet disease: a replication study in the Spanish population.
PLoS ONE
PUBLISHED: 01-01-2014
Show Abstract
Hide Abstract
Behçet's disease (BD) is a multifactorial disorder associated with the HLA region. Recently, the ERAP1 gene has been proposed as a susceptibility locus with a recessive model and with epistatic interaction with HLA-B51. ERAP1 trims peptides in the endoplasmic reticulum to optimize their length for MHC-I binding. Polymorphisms in this gene have been related with the susceptibility to other immune-mediated diseases associated to HLA class I. Our aim was, the replication in the Spanish population of the association described in the Turkish population between ERAP1 (rs17482078) and BD. Additionally, in order to improve the understanding of this association we analyzed four additional SNPs (rs27044, rs10050860, rs30187 and rs2287987) associated with other diseases related to HLA class I and the haplotype blocks in this gene region. According to our results, frequencies of the homozygous genotypes for the minor alleles of all the SNPs were increased among patients and the OR values were higher in the subgroup of patients with the HLA-B risk factors, although differences were not statistically significant. Moreover, the presence of the same mutation in both chromosomes increased the OR values from 4.51 to 10.72 in individuals carrying the HLA-B risk factors. Therefore, although they were not statistically significant, our data were consistent with an association between ERAP1 and BD as well as with an epistatic interaction between ERAP1 and HLA-B in the Spanish population.
Related JoVE Video
High prevalence of prothrombotic abnormalities in multifocal osteonecrosis: description of a series and review of the literature.
Medicine (Baltimore)
PUBLISHED: 10-23-2013
Show Abstract
Hide Abstract
Multifocal or multiple osteonecrosis (ON), defined by the involvement of 3 or more anatomic sites, is unusual, being observed in only 3%-10% of patients diagnosed with ON. We report the clinical characteristics of a cohort of 29 patients with multifocal ON from a single center and evaluate the prevalence of associated prothrombotic abnormalities in 26 of these patients. We conducted a retrospective study of all patients diagnosed with multifocal ON evaluated in our institution during the last 20 years. We recorded clinical manifestations and underlying diagnoses. A wide thrombophilic profile was performed, including antithrombin, protein C, protein S, lupus anticoagulant, anticardiolipin antibodies, activated protein C resistance, factor V Leiden, mutation G-20210-A of the prothrombin gene, and factor VIII. Coagulation test results were compared with those in a healthy control group and a group of patients with history of lower-extremity deep venous thrombosis. The mean age of the patients was 49.2 ± 15 years (range, 28-81 yr). The mean number of ON localizations per patient was 5.2 ± 2.3 (range, 3-11). Hips were the most commonly affected joint (82%), followed by knees (58%), shoulders (37%), and ankles (13%). Most patients had an underlying disease process, and 12 of 25 (48%) patients had coagulation test abnormalities. The most common alterations were high factor VIII levels and antiphospholipid antibody (aPL) positivity in 24% and 20% of cases, respectively. These abnormalities were more prevalent in patients with multifocal ON compared with patients in the control groups. Sixty-one percent of patients had a history of corticosteroid treatment. Patients with coagulation abnormalities had a higher number of ON localizations per patient (6.5 ± 2.7 vs. 3.88 ± 0.8; p = 0.002) and a higher prevalence of atypical ON localizations (25% vs. 0%; p = 0.05). In conclusion, in the present cohort of patients with multifocal ON, 48% of the patients had at least 1 prothrombotic factor, especially high levels of factor VIII and aPL. These findings have major implications for the diagnosis and treatment of multifocal ON and clearly indicate the need to perform a thrombophilic profile in these patients.
Related JoVE Video
Low-dose aspirin vs low-dose aspirin plus low-intensity warfarin in thromboprophylaxis: a prospective, multicentre, randomized, open, controlled trial in patients positive for antiphospholipid antibodies (ALIWAPAS).
Rheumatology (Oxford)
PUBLISHED: 10-04-2013
Show Abstract
Hide Abstract
Objectives. The objectives of this study are to examine the efficacy and safety of low-dose aspirin (LDA) vs LDA plus low-intensity warfarin (LDA + W) in the primary thrombosis prevention of aPL-positive patients with SLE and/or obstetric morbidity and the role of clinical and serological markers in the development of thrombosis.Methods. In this 5-year prospective, randomized, open, controlled trial, 166 patients with aPL were randomly assigned using a minimization protocol to receive treatment with LDA (n = 82) or LDA + W [international normalized ratio (INR) = 1.5] (n = 84). Sixty-six patients who declined randomization were followed up in an observational arm. Clinical and laboratory characteristics and medication side effects were recorded.Results. There were no differences in the number of thromboses between patients treated with LDA (4/82) or LDA + W (4/84) [hazard ratio (HR) 1.07, 95% CI 0.27, 4.3]. The incidence of thrombosis in the randomized patients was 8/166 (1.8 events/100 person-years) (HR 1.07, 95% CI 0.27, 4.3) and in the observational arm was 7/66 (4.9 events/100 person-years) (HR 2.43, 95% CI 0.87, 6.79). Sixty-five of 66 patients included in the observational arm received LDA. None of the examined clinical or serological factors appeared to predict thrombosis. Medication side effects included mild gastrointestinal symptoms in the LDA group (n = 2) and bleeding in the LDA + W group (n = 11; 1 nasal and 10 menorrhagia). The risk difference for bleeding was 13% (CI 6, 20).Conclusion. No differences in the number of thromboses were observed between patients treated with LDA vs those treated with LDA + W. More episodes of bleeding were detected in the LDA + W group. The LDA + W regime was significantly less safe and not as acceptable as LDA alone.Trial registration. ISRCTN81818945; http://isrctn.org/.
Related JoVE Video
Pediatric catastrophic antiphospholipid syndrome: Descriptive analysis of 45 patients from the "CAPS Registry".
Autoimmun Rev
PUBLISHED: 10-01-2013
Show Abstract
Hide Abstract
Given the lack of information about catastrophic antiphospholipid syndrome (APS) in pediatric patients, the objective of the current study was to describe the clinical characteristics, laboratory features, treatment, and outcome of pediatric patients with catastrophic APS and compare them with the adult patients with catastrophic APS. We identified patients who were under 18years of age at time of catastrophic APS diagnosis included in the international registry of patients with catastrophic APS (CAPS Registry). Their main demographic and clinical characteristics, laboratory features, treatment, and outcome were described and compared with those of adult patients with catastrophic APS. From the 446 patients included in the CAPS Registry as of May 2013, 45 (10.3%) patients developed 46 catastrophic events before 18years of age (one patient presented two episodes). Overall, 32 (71.1%) patients were female and the mean age was 11.5±4.6years (range, 3months-18years). A total of 31 (68.9%) patients suffered from primary APS and 13 (28.9%) from systemic lupus erythematosus (SLE). The main differences between the two groups of patients were the higher prevalence of infections as precipitating factor for catastrophic event in the pediatric population (60.9% versus 26.8% in the adult population, p<0.001) and of peripheral vessel thrombosis (52.2% versus 34.3%, p=0.017). In addition, catastrophic APS was the first manifestation of APS more frequently in pediatric patients (86.6% versus 45.2%, p<0.001). Interestingly, pediatric patients showed a trend of lower mortality, although the difference was not statistically significant (26.1% versus 40.2%; odds ratio, 1.9; 95% confidence interval, 0.96-3.79; p=0.063). No differences were found neither in the laboratory features nor in the isolated or combination treatments between groups. Catastrophic APS in pediatric patients is a rare disease. There are minimal differences in the clinical and laboratory features, treatment, and outcome of pediatric and adult catastrophic APS patients.
Related JoVE Video
HLA and non-HLA genes in Behçets disease: a multicentric study in the Spanish population.
Arthritis Res. Ther.
PUBLISHED: 09-19-2013
Show Abstract
Hide Abstract
According to genome wide association (GWA) studies as well as candidate gene approaches, Behçets disease (BD) is associated with human leukocyte antigen (HLA)-A and HLA-B gene regions. The HLA-B51 has been consistently associated with the disease, but the role of other HLA class I molecules remains controversial. Recently, variants in non-HLA genes have also been associated with BD. The aims of this study were to further investigate the influence of the HLA region in BD and to explore the relationship with non-HLA genes recently described to be associated in other populations.
Related JoVE Video
Neuropsychiatric systemic lupus erythematosus: magnetic resonance imaging findings and correlation with clinical and immunological features.
Autoimmun Rev
PUBLISHED: 07-01-2013
Show Abstract
Hide Abstract
Neuropsychiatric (NP) syndromes are a major cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). The aims of this work were to describe the brain abnormalities in a group of SLE patients during their first episode of NP manifestations using a conventional magnetic resonance imaging (MRI) technique and to investigate the possible correlation between these findings and the clinical and immunological characteristics of these patients. We performed an observational retrospective cross-sectional study that included all patients with NP symptoms who underwent MRI at the Hospital Clinic of Barcelona between the years 2003 and 2012 because of suspecting NP syndromes due to SLE (NPSLE). We studied 43 patients in which 11 types of NPSLE were present, being headache the most frequent, followed by cerebrovascular disease, epileptic crises and cranial neuropathy. A statistically significant association was found between myelopathy and low complement (C4) levels (p=0.035) and disease activity measured as SLE Disease Activity Index (SLEDAI) >4 (p=0.00006). Eighteen (41.9%) patients presented MRI abnormalities. We found an association between myelopathy and the presence of inflammatory or mixed (vascular and inflammatory) type lesions (p=0.003). This pattern was also associated with a high SLEDAI score (p=0.002) and low complement (CH50) levels (p=0.032). We found no relationship between MRI changes and age, time of evolution, or the presence of antiphospholipid or anti-dsDNA antibodies. These results suggest that MRI, although it is the imaging modality of choice in the present moment, by itself does not establish or exclude the diagnosis of NPSLE. In addition, the presence of certain disease activity features (SLEDAI and low complement levels) seems to be associated with the presence of an inflammatory pattern on MRI.
Related JoVE Video
A systemic sclerosis and systemic lupus erythematosus pan-meta-GWAS reveals new shared susceptibility loci.
Hum. Mol. Genet.
PUBLISHED: 06-04-2013
Show Abstract
Hide Abstract
Systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) are two archetypal systemic autoimmune diseases which have been shown to share multiple genetic susceptibility loci. In order to gain insight into the genetic basis of these diseases, we performed a pan-meta-analysis of two genome-wide association studies (GWASs) together with a replication stage including additional SSc and SLE cohorts. This increased the sample size to a total of 21,109 (6835 cases and 14,274 controls). We selected for replication 19 SNPs from the GWAS data. We were able to validate KIAA0319L (P = 3.31 × 10(-11), OR = 1.49) as novel susceptibility loci for SSc and SLE. Furthermore, we also determined that the previously described SLE susceptibility loci PXK (P = 3.27 × 10(-11), OR = 1.20) and JAZF1 (P = 1.11 × 10(-8), OR = 1.13) are shared with SSc. Supporting these new discoveries, we observed that KIAA0319L was overexpressed in peripheral blood cells of SSc and SLE patients compared with healthy controls. With these, we add three (KIAA0319L, PXK and JAZF1) and one (KIAA0319L) new susceptibility loci for SSc and SLE, respectively, increasing significantly the knowledge of the genetic basis of autoimmunity.
Related JoVE Video
Rituximab use in the catastrophic antiphospholipid syndrome: Descriptive analysis of the CAPS registry patients receiving rituximab.
Autoimmun Rev
PUBLISHED: 05-08-2013
Show Abstract
Hide Abstract
The catastrophic variant of the antiphospholipid syndrome (APS) is characterized by thrombosis in multiple organs developing over a short period of time. First-line treatment for the catastrophic APS is the combination of anticoagulation plus corticosteroids plus plasma exchange and/or intravenous immunoglobulin. Despite this regimen, the mortality remains high and new treatment options are needed. By a systematic review of the Catastrophic APS Registry (CAPS Registry), we identified 20 patients treated with rituximab. The purpose of this study is to describe the clinical manifestations, laboratory features, and outcomes of rituximab-treated CAPS patients. In addition, the rationale for using rituximab in catastrophic APS is discussed.
Related JoVE Video
Catastrophic antiphospholipid syndrome: diagnosis and management in pregnancy.
Clin. Lab. Med.
PUBLISHED: 04-19-2013
Show Abstract
Hide Abstract
Catastrophic antiphospholipid syndrome (CAPS) is a potentially lethal variant of antiphospholipid syndrome, characterized by multiorgan thrombosis in a short period of time, affecting mainly small vessels. In approximately 50% of CAPS cases, the catastrophic event is preceded by a trigger, mainly infections, or surgery, anticoagulation withdrawal, lupus flares, neoplasm, or pregnancy and puerperium. Treatment of CAPS is based on expert opinion and relies on a combination of several strategies, including anticoagulation, steroids, plasma exchange sessions, and/or intravenous immunoglobulins.
Related JoVE Video
Discontinuation of anticoagulation or antiaggregation treatment may be safe in patients with primary antiphospholipid syndrome when antiphospholipid antibodies became persistently negative.
Immunol. Res.
PUBLISHED: 04-10-2013
Show Abstract
Hide Abstract
Recommended treatment for patients with primary antiphospholipid syndrome (APS) after a thrombotic event is long-term anticoagulation. Few patients with APS and previously positive antiphospholipid antibodies (aPL) may become negative over time. It is still not exactly known how to treat these patients when aPL become persistently negative. We described the follow-up of 11 primary APS patients whose aPL become persistently negative and in whom thromboprophylaxis was discontinued. The primary end-point was the recurrence of thrombosis in patients with previous thrombotic event and a first thrombotic event in women with previous obstetric APS. Ten (90%) patients were female. Seven (64%) patients had a history of deep venous thrombosis, two of them with pulmonary embolism, and four (36%) women had recurrent miscarriage. Lupus anticoagulant (LAC) was the aPL most frequently detected (82%). Two patients had both LAC and IgG anticardiolipin antibodies. No new thrombotic episode was observed after a median follow-up period of 20 months. Anticoagulation or antiaggregation could be discontinued in some peculiar patients with low-risk primary APS whose aPL become persistently negative. However, studies including a larger number of patients are required to confirm these results.
Related JoVE Video
Can inherited thrombophilia modulate the clinical phenotype of patients with antiphospholipid syndrome?
Clin. Exp. Rheumatol.
PUBLISHED: 03-17-2013
Show Abstract
Hide Abstract
The current case-control study was aimed to determine the prevalence and the clinical significance of inherited thrombophilia - factor V Leiden and G20210A prothrombin polymorphisms - in patients with antiphospholipid syndrome (APS).
Related JoVE Video
Vitamin K-dependent proteins GAS6 and Protein S and TAM receptors in patients of systemic lupus erythematosus: correlation with common genetic variants and disease activity.
Arthritis Res. Ther.
PUBLISHED: 03-11-2013
Show Abstract
Hide Abstract
INTRODUCTION: Growth arrest-specific gene 6 protein (GAS6) and protein S (ProS) are vitamin K-dependent proteins present in plasma with important regulatory functions in systems of response and repair to damage. They interact with receptor tyrosine kinases of the Tyro3, Axl and MerTK receptor tyrosine kinase (TAM) family, involved in apoptotic cell clearance (efferocytosis) and regulation of the innate immunity. TAM-deficient mice show spontaneous lupus-like symptoms. Here we tested the genetic profile and plasma levels of components of the system in patients with systemic lupus erythematosus (SLE), and compare them with a control healthy population. METHODS: Fifty SLE patients and 50 healthy controls with matched age, gender and from the same geographic area were compared. Genetic analysis was performed in GAS6 and the TAM receptor genes on SNPs previously identified. The concentrations of GAS6, total and free ProS, and the soluble forms of the three TAM receptors (sAxl, sMerTK and sTyro3) were measured in plasma from these samples. RESULTS: Plasma concentrations of GAS6 were higher and, total and free ProS were lower in the SLE patients compared to controls, even when patients on oral anticoagulant treatment were discarded. Those parameters correlated with SLE disease activity index (SLEDAI) score, GAS6 being higher in the most severe cases, while free and total ProS were lower. All 3 soluble receptors increased its concentration in plasma of lupus patients. CONCLUSIONS: The present study highlights that the GAS6/ProS-TAM system correlates in several ways with disease activity in SLE. We show here that this correlation is affected by common polymorphisms in the genes of the system. These findings underscore the importance of mechanism of regulatory control of innate immunity in the pathology of SLE.
Related JoVE Video
Immunosuppression and Chagas disease: a management challenge.
PLoS Negl Trop Dis
PUBLISHED: 01-17-2013
Show Abstract
Hide Abstract
Immunosuppression, which has become an increasingly relevant clinical condition in the last 50 years, modifies the natural history of Trypanosoma cruzi infection in most patients with Chagas disease. The main goal in this setting is to prevent the consequences of reactivation of T. cruzi infection by close monitoring. We analyze the relationship between Chagas disease and three immunosuppressant conditions, including a description of clinical cases seen at our center, a brief review of the literature, and recommendations for the management of these patients based on our experience and on the data in the literature. T. cruzi infection is considered an opportunistic parasitic infection indicative of AIDS, and clinical manifestations of reactivation are more severe than in acute Chagas disease. Parasitemia is the most important defining feature of reactivation. Treatment with benznidazole and/or nifurtimox is strongly recommended in such cases. It seems reasonable to administer trypanocidal treatment only to asymptomatic immunosuppressed patients with detectable parasitemia, and/or patients with clinically defined reactivation. Specific treatment for Chagas disease does not appear to be related to a higher incidence of neoplasms, and a direct role of T. cruzi in the etiology of neoplastic disease has not been confirmed. Systemic immunosuppressive diseases or immunosuppressants can modify the natural course of T. cruzi infection. Immunosuppressive doses of corticosteroids have not been associated with higher rates of reactivation of Chagas disease. Despite a lack of evidence-based data, treatment with benznidazole or nifurtimox should be initiated before immunosuppression where possible to reduce the risk of reactivation. Timely antiparasitic treatment with benznidazole and nifurtimox (or with posaconazole in cases of therapeutic failure) has proven to be highly effective in preventing Chagas disease reactivation, even if such treatment has not been formally incorporated into management protocols for immunosuppressed patients. International consensus guidelines based on expert opinion would greatly contribute to standardizing the management of immunosuppressed patients with Chagas disease.
Related JoVE Video
Current and future treatments for Behçets uveitis: road to remission.
Int Ophthalmol
PUBLISHED: 01-09-2013
Show Abstract
Hide Abstract
Behçets disease (BD) is a multisystem inflammatory disorder of uncertain origin, although it remains defined within the spectrum of systemic immune-mediated vasculitic disorders and also represents a spectrum of putative autoimmune disease. Major symptoms include oral aphthous ulcers, genital ulcerations, skin lesions, and ocular lesions. Despite afflicting many systems, ocular complications of BD are some of the more devastating for the patient and their quality of life. Eye involvement, which affects 60-80 % of BD patients, is characterized in its more severe form by posterior or panuveitis including occlusive retinal vasculitis. While pathogenesis of BD remains complex, association with Class I MHC (HLA-B*51) predisposing to inflammation with engagement of the innate-immune system (neutrophils, NK cells), and perpetuated by the adaptive T cell responses against infectious- and/or auto-antigens. Despite the choice of conventional immunosuppressive therapies available, only recently with the advent of biologic therapy has visual prognosis and outcomes been substantially and favorably altered. For example, both interferon-? (IFN-?) and tumour necrosis factor (TNF)-? antagonists deliver promising results and for the first time improve prognosis. With IFN-? therapy, durable remissions of uveitis can be achieved and lead to drug-free remission. Similarly, anti-TNF therapy with infliximab is reported to be rapidly effective in inducing and maintaining remission. Most recently, rising evidence reports on the use of adalimumab, etanercept, and golimumab, while use of anti-interleukin (IL)-1 agents (anakinra, canakinumab, gevokizumab), IL-6 blockers (tocilizumab), and rituximab (depleting anti-CD20 antibody) is also increasing. The aim of this review is to provide evidence for the role of conventional therapies combined with evidence for advantages and disadvantages of biologic therapies in the treatment of ocular BD. Although randomized controlled trials remain sparse, evidence remains strong and enticing that biologic agents are invaluable for the treatment of sight-threatening Behçets uveitis and makes it an exciting time for Behçets specialists worldwide.
Related JoVE Video
Risk of thromboembolic events after recurrent spontaneous abortion in antiphospholipid syndrome: a case-control study.
Ann. Rheum. Dis.
PUBLISHED: 09-06-2011
Show Abstract
Hide Abstract
To investigate whether patients having antiphospholipid syndrome (APS) as the only aetiological factor for recurrent spontaneous abortion (RSA) are at increased risk of thrombosis later in life.
Related JoVE Video
Therapeutic plasma exchange for the management of refractory systemic autoimmune diseases: report of 31 cases and review of the literature.
Autoimmun Rev
PUBLISHED: 05-01-2011
Show Abstract
Hide Abstract
Therapeutic plasma exchange (TPE) represents a treatment option in patients with systemic autoimmune diseases because most of their clinical manifestations are related to the presence of antibodies or immune complex deposition.
Related JoVE Video
Management of refractory cases of catastrophic antiphospholipid syndrome.
Autoimmun Rev
PUBLISHED: 05-01-2011
Show Abstract
Hide Abstract
The catastrophic variant of the antiphospholipid syndrome (APS) is the most severe form of APS with acute multiple organ involvement and small vessel thrombosis. Refractory catastrophic APS may be defined as patients who did not respond to first-line therapies (anticoagulation, glucocorticoids and plasma exchange and/or intravenous immunoglobulins) and died in the acute phase of the episode or patients with recurrent episodes of catastrophic APS. The purpose of this review is to focus on the current management of these refractory patients and some of the potential new therapeutic approaches.
Related JoVE Video
Epidemiology and management of refractory lupus nephritis.
Autoimmun Rev
PUBLISHED: 05-01-2011
Show Abstract
Hide Abstract
Although the survival of patients with lupus nephritis (LN) has improved considerably in recent years, refractory LN appears in a substantial proportion of patients and, therefore, treatment of LN remains a real challenge today. We will use the term "refractory" LN, for those cases with none or partial response to first-line therapies. In this sense, numerous epidemiological factors, including racial, socioeconomic, histological and serological parameters, may influence treatment response and, therefore, may have an impact on the outcome of renal involvement. Initial conventional therapy will depend somewhat on these epidemiological factors. If this initial therapy fails, fortunately today we have alternative therapies that include the multitarget therapy and the use of biologics. Published evidence about these therapies is presented in this review. Important terms in the management of LN, such as the definition of complete response, partial response, sustained response and renal flare as well as the discrimination of different types of flare, are also discussed here according to the European consensus statement on the terminology used in the management of lupus glomerulonephritis.
Related JoVE Video
[Uveitis diagnosis characterization at a referral centre in the area of Barcelona, Spain].
Med Clin (Barc)
PUBLISHED: 04-04-2011
Show Abstract
Hide Abstract
To describe the uveitis pattern in our geographic area. Recent demographic, environmental and scientific changes can determine uveitis pattern changes, which we aim to investigate.
Related JoVE Video
Efficacy of cyclophospamide in the treatment of interstitial lung disease associated with systemic sclerosis.
Arch. Bronconeumol.
PUBLISHED: 01-13-2011
Show Abstract
Hide Abstract
Cyclophosphamide (CYC) stabilizes the parameters of lung function tests (LFT) of patients with (SSc) and interstitial lung disease (ILD) treated for 12 months. There is little information about long-term treatment (24 months). The aim of this study is to analyze the effect of intravenous CYC in LFT parameters in patients with SSc and ILD treated for 24 months.
Related JoVE Video
Antiphospholipid syndrome: long-time research on pathogenic mechanisms has finally lead to new therapeutic strategies.
Expert Opin. Ther. Targets
PUBLISHED: 11-10-2010
Show Abstract
Hide Abstract
The antiphospholipid syndrome is characterized by the presence of arterial or venous thrombosis or recurrent miscarriages in a patient with positive laboratory tests for antiphospholipid antibodies (anticardiolipin antibodies and/or lupus anticoagulant and/or anti-beta2-glycoprotein I). Despite the strong association between antiphospholipid antibodies and thrombosis and obstetric morbidity, their pathogenic role in the development of these clinical features has not been fully elucidated. However, the knowledge of new pathogenic mechanisms might identify novel therapeutic targets and therefore may improve the management of these patients.
Related JoVE Video
[Biological therapies in systemic lupus erythematosus].
Rev Med Chil
PUBLISHED: 11-04-2010
Show Abstract
Hide Abstract
The immunosuppressive agents used in patients with systemic lupus erythematosus (SLE) have significantly improved prognosis. However, it is necessary to develop more specific immunosuppressive treatments with less toxicity. Better understanding of the mechanisms involved in the loss of tolerance in autoimmune diseases has contributed to the development of potential new treatments called biologic therapies. The targets of these biological therapies are directed toward the B cell depletion, interference in the co-stimulation signals and the blockade of cytokines. Therapies using anti-CD20 monoclonal antibodies have shown satisfactory results especially in patients with SLE refractory to conventional treatment. The biological therapies provide encouraging results that represent a possible option in the treatment of refractory patients as well as a potential therapy in the future management of SLE.
Related JoVE Video
Solid cancer, antiphospholipid antibodies, and venous thromboembolism.
Autoimmun Rev
PUBLISHED: 09-19-2010
Show Abstract
Hide Abstract
The pathogenic role of antiphospholipid antibodies (aPL) in the development of venous thromboembolism (VTE) in patients with malignancies has not been established. From May 2006 to April 2008, 258 consecutive patients with solid-organ malignancies who developed VTE (VTE+) were recruited. A group of 142 patients matched for age, sex and tumor type cancer patients without VTE (VTE-) and an age-and-sex matched group of 258 healthy subjects were also included. A second blood sample was taken in positive aPL patients at least 12 weeks later. Twenty-one (8.1%) VTE+ patients, 2 (1.4%) VTE- patients (p=0.006) and 2 (0.8%) healthy subjects (p<0.001) were positive for aPL. Persistent aPL positivity was observed in only 4 out of 15 available VTE+ patients. No differences in demographic characteristics, clinical pattern and outcome were observed in VTE+ patients according to aPL status. The low prevalence and transience of aPL positivity in patients with solid-organ malignancies with VTE argues against a pathogenic role in the development of thrombosis in this setting. The published evidence of the relationship between cancer, aPL, and thrombosis is reviewed.
Related JoVE Video
Catastrophic antiphospholipid syndrome: updated diagnostic algorithms.
Autoimmun Rev
PUBLISHED: 07-20-2010
Show Abstract
Hide Abstract
The catastrophic antiphospholipid syndrome (APS) is the most severe form of APS with acute multiple organ involvement and small vessel thrombosis. During the 13th International Congress on Antiphospholipid Antibodies (aPL) a "Catastrophic APS Task Force" was developed to discuss the challenges in the diagnosis and management of catastrophic APS. The purpose of this paper is to summarize the diagnostic challenges (false-positive/negative aPL results and overlap with other thrombotic microangiopathies) and propose updated diagnostic algorithms for catastrophic APS. Important steps of the diagnostic algorithms include: a) history of APS or persistent aPL-positivity; b) three or more organ new thrombosis developing in less than a week; c) biopsy diagnosis of microthrombosis; and d) other explanations for multiple organ thromboses and/or microthrombosis.
Related JoVE Video
Effects of infliximab in the treatment of refractory posterior uveitis of Behçets disease after withdrawal of infusions.
Int Ophthalmol
PUBLISHED: 05-02-2010
Show Abstract
Hide Abstract
To determine the efficacy of infliximab treatment in refractory posterior uveitis in Behçets disease after withdrawal of infusions. Four patients with posterior uveitis secondary to Behçets disease were treated with infliximab until complete remission and were followed after withdrawal of infusions. Intra-ocular inflammation was assessed using the binocular indirect ophthalmoscopy score, best-corrected visual acuity (BCVA) and foveal thickness measured by optic coherence tomography (OCT). All the patients included in the study were treated with infliximab for a minimum of 12 months and were in complete remission. None of the patients were taking steroids or immunosuppressants. Main follow-up after withdrawal of infusions was 7.5 months. Two out of four patients (50%) maintained complete remission of posterior uveitis. BCVA was stable in seven eyes. OCT showed worsening in macular edema in the two eyes of the patients with reactivation. Infliximab is an efficient long-term treatment for refractory posterior uveitis. Repeated infusions are required to maintain long-term remission which may be sustained on discontinuation of the drug.
Related JoVE Video
Antiphospholipid syndrome: frequency, main causes and risk factors of mortality.
Nat Rev Rheumatol
PUBLISHED: 04-13-2010
Show Abstract
Hide Abstract
The presence of antiphospholipid antibodies has been shown to be related to an increased risk of thrombotic events. In patients with definite antiphospholipid syndrome (APS), that is, those who have had thrombosis and at least two positive determinations of antiphospholipid antibodies, secondary thromboprophylaxis with long-term anticoagulation therapy results in a low rate of recurrent thrombotic events, ranging from 0.016 to 0.031 events per patient per year. Thrombotic complications are, however, the most common cause of death in APS. The mortality rate in a large European cohort of patients with APS during a 5-year study period was 5.3%, and up to 40% of the deaths in this cohort were attributed to severe thrombotic events such as myocardial infarction, stroke and pulmonary embolism. Catastrophic APS is an unusual form of the disease, being observed in less than 1% of reported cases of APS, which is associated with a much higher mortality rate than classical APS. The combined use of anticoagulation, corticosteroids, plasma exchange and intravenous immunoglobulin therapy could result in a dramatic reduction in mortality, by approximately 20%, in patients with catastrophic APS.
Related JoVE Video
Successful treatment with posaconazole of a patient with chronic Chagas disease and systemic lupus erythematosus.
Am. J. Trop. Med. Hyg.
PUBLISHED: 03-30-2010
Show Abstract
Hide Abstract
American Trypanosomiasis or Chagas disease (CD) is a neglected disease that affects Latin American people worldwide. Two old antiparasitic drugs, benznidazole and nifurtimox, are currently used for specific CD treatment with limited efficacy in chronic infections and frequent side effects. New drugs are needed for patients with chronic CD as well as for immunosuppressed patients, for whom the risk of reactivation is life-threatening. We describe a case of chronic CD and systemic lupus erythematosus (SLE) that required immunosuppression to control the autoimmune process. It was found that benznidazole induced a reduction, but not an elimination, of circulating Trypanosoma cruzi levels, whereas subsequent treatment with posaconazole led to a successful resolution of the infection, despite the maintenance of immunosuppressive therapy.
Related JoVE Video
Recent trends in the management of antiphospholipid syndrome (Hughes syndrome).
Drugs Today
PUBLISHED: 03-05-2010
Show Abstract
Hide Abstract
Antiphospholipid syndrome (Hughes syndrome) is characterized by the presence of arterial or venous thrombosis or recurrent miscarriages in a patient with positive laboratory tests for antiphospholipid antibodies (anticardiolipin antibodies and/or lupus anticoagulant and/or anti-beta(2) glycoprotein I). Despite the strong association between antiphospholipid antibodies, and thrombosis and obstetric morbidity, their pathogenic role in the development of these clinical features has not been fully elucidated. At present, patients with thrombosis are treated with long-term oral anticoagulation as standard. The approach for women with obstetric manifestations is based on the use of aspirin plus heparin. However, the knowledge of new pathogenic mechanisms might identify novel therapeutic targets and, therefore, may improve the management of these patients.
Related JoVE Video
Clot lysis time and thrombin activatable fibrinolysis inhibitor in severe preeclampsia with or without associated antiphospholipid antibodies.
J. Reprod. Immunol.
PUBLISHED: 02-14-2010
Show Abstract
Hide Abstract
We investigated clot lysis time, thrombin activatable fibrinolysis inhibitor antigen (TAFI) levels and TAFI gene polymorphisms in pregnant patients with severe preeclampsia, with or without associated antiphospholipid syndrome (APS). The study groups included 82 pregnant patients without antiphospholipid antibodies with severe preeclampsia (PE group) and 10 pregnant APS patients who developed severe preeclampsia (APS-PE group). Controls included 76 primary pregnant APS patients (APS group) and 89 healthy pregnant patients (NOR group) with uneventful term pregnancy and delivery. Patients in the APS-PE, APS and NOR groups were sampled during each trimester of pregnancy and at 4-6 months and 12 months after delivery. Patients in the PE group were sampled during the third trimester and after delivery. Significantly prolonged clot lysis time after delivery was found in the PE, APS-PE and APS groups compared to the NOR group. The PE and APS-PE groups had longer clot lysis time than the APS group. Levels of TAFI were found to be higher after delivery in patients of the PE and APS-PE groups compared to the APS and NOR groups. Allele distribution of the TAFI gene polymorphisms was similar among the four study groups. We conclude that increased TAFI antigen levels and impaired fibrinolysis are pathogenetic factors in preeclampsia, regardless of whether or not preeclampsia is associated with the presence of antiphospholipid antibodies.
Related JoVE Video
Chronic thromboembolic pulmonary hypertension in Behçets disease: effectiveness of endarterectomy.
Clin. Exp. Rheumatol.
PUBLISHED: 01-27-2010
Show Abstract
Hide Abstract
Behçets disease is a systemic vasculitis characterised by recurrent mouth and genital ulcers and uveitis. About 25% of patients suffer from vascular involvement. We describe a patient with Behçets disease who suffered recurrent pulmonary embolism and developed severe chronic thromboembolic pulmonary hypertension. The patient was successfully treated with pulmonary endarterectomy that normalised pulmonary haemodynamics. Chronic thromboembolic pulmonary hypertension is a potential complication of Behçets disease that may be amenable to pulmonary endarterectomy.
Related JoVE Video
Genotypes coding for low serum levels of mannose-binding lectin are underrepresented among individuals suffering from noninfectious systemic inflammatory response syndrome.
Clin. Vaccine Immunol.
PUBLISHED: 12-30-2009
Show Abstract
Hide Abstract
Gene polymorphisms, giving rise to low serum levels of mannose-binding lectin (MBL) or MBL-associated protease 2 (MASP2), have been associated with an increased risk of infections. The objective of this study was to assess the outcome of intensive care unit (ICU) patients with systemic inflammatory response syndrome (SIRS) regarding the existence of functionally relevant MBL2 and MASP2 gene polymorphisms. The study included 243 ICU patients with SIRS admitted to our hospital, as well as 104 healthy control subjects. MBL2 and MASP2 single nucleotide polymorphisms were genotyped using a sequence-based typing technique. No differences were observed regarding the frequencies of low-MBL genotypes (O/O and XA/O) and MASP2 polymorphisms between patients with SIRS and healthy controls. Interestingly, ICU patients with a noninfectious SIRS had a lower frequency for low-MBL genotypes and a higher frequency for high-MBL genotypes (A/A and A/XA) than either ICU patients with an infectious SIRS or healthy controls. The existence of low- or /high-MBL genotypes or a MASP2 polymorphism had no impact on the mortality rates of the included patients. The presence of high-MBL-producing genotypes in patients with a noninfectious insult is a risk factor for SIRS and ICU admission.
Related JoVE Video
Thrombin activatable fibrinolysis inhibitor and clot lysis time in pregnant patients with antiphospholipid syndrome: relationship with pregnancy outcome and thrombosis.
Am. J. Reprod. Immunol.
PUBLISHED: 11-10-2009
Show Abstract
Hide Abstract
Antiphospholipid syndrome (APS) pregnancies are associated with thrombotic obstetric complications, despite treatment. This study evaluated Thrombin Activatable Fibrinolysis Inhibitor (TAFI) levels, TAFI gene polymorphisms and Clot Lysis Time (CLT) in pregnant patients with APS in relation to pregnancy outcome and thrombosis.
Related JoVE Video
Etiopathogenesis of Behcets disease.
Autoimmun Rev
PUBLISHED: 10-01-2009
Show Abstract
Hide Abstract
Bechets disease (BD) is an inflammatory, multi systemic disease with spontaneous remissions and relapses similar to various autoimmune diseases. BD leads to organ damage, including the eyes, skin, joints, etc., which produces various clinical manifestations. The central histopathologic characteristic is systemic vasculitis with perivascular inflammatory infiltrates. The etiopathogenesis is unknown, although immunological abnormalities, possibly induced by susceptible microbiological pathogens, have been postulated.
Related JoVE Video
Factors influencing polyautoimmunity in systemic lupus erythematosus.
Autoimmun Rev
PUBLISHED: 09-29-2009
Show Abstract
Hide Abstract
Since characterization of the extent to which particular combinations of autoimmune diseases (ADs) occur in excess of that expected by chance may offer new insights into possible common pathophysiological mechanisms, polyautoimmunity (i.e., ADs co-occurring within patients) in systemic lupus erythematosus (SLE) and its associated factors were investigated.
Related JoVE Video
Catastrophic antiphospholipid syndrome: treatment, prognosis, and the risk of relapse.
Clin Rev Allergy Immunol
PUBLISHED: 06-06-2009
Show Abstract
Hide Abstract
The "catastrophic" variant of the antiphospholipid syndrome (APS) is characterized by multiple vascular occlusive events, usually affecting small vessels and developing over a short period of time. Although patients with catastrophic APS represent less than 1% of all patients with APS, they are usually in a life-threatening situation with a 50% mortality rate. The purpose of this paper is to review the treatment strategies and prognostic factors in patients with catastrophic APS. A detailed description of the clinical and laboratory features of the syndrome can be found in the other articles of this issue.
Related JoVE Video
Morbidity and mortality in the antiphospholipid syndrome.
Curr Opin Pulm Med
PUBLISHED: 05-14-2009
Show Abstract
Hide Abstract
Thrombotic complications are the most common cause of death and serious morbidity in antiphospholipid syndrome (APS). Functional prognosis is poor in a significant number of patients with APS. Moreover, catastrophic APS is an unusual form of presentation that represents less than 1% of APS cases reported but with a high mortality rate (around 50%). There is a growing awareness to determine optimal prognostic markers and therapeutic measures to prevent these important complications in the APS.
Related JoVE Video
Thromboprophylaxis and obstetric management of the antiphospholipid syndrome.
Expert Opin Pharmacother
PUBLISHED: 03-17-2009
Show Abstract
Hide Abstract
There is consensus in treating patients with antiphospholipid syndrome (APS) and first venous thrombosis with oral anticoagulation to a target international normalized ratio of 2.0 - 3.0. A recent systematic review recommended a target international normalized ratio > 3.0 in the group of patients with APS and arterial thrombosis. The approach for women with obstetric manifestations of APS is based on the use of aspirin plus heparin. In addition, we summarize the evidence-based information about management of catastrophic APS and some difficult cases, such as seronegative APS, patients who do not display formal classification criteria for APS, and recurrent thrombotic events despite optimal anticoagulation.
Related JoVE Video
Heart valve surgery in patients with the antiphospholipid syndrome: analysis of a series of nine cases.
Eur J Cardiothorac Surg
PUBLISHED: 02-09-2009
Show Abstract
Hide Abstract
Antiphospholipid syndrome (APS) is a rare coagulation disorder associated with recurrent arterial and venous thrombotic events. Heart valve abnormalities are commonly found in patients with APS.
Related JoVE Video
Factor XIII-A subunit Val34Leu polymorphism is associated with the risk of thrombosis in patients with antiphospholipid antibodies and high fibrinogen levels.
Thromb. Haemost.
PUBLISHED: 02-05-2009
Show Abstract
Hide Abstract
Recent reports have described the factor XIII A subunit (FXIII-A) Val34Leu polymorphism as a protective factor against venous and arterial thrombosis. The aim of this study was to investigate the association between the FXIII-A Val34Leu polymorphism, its interaction with fibrinogen concentration, and thrombosis in patients with antiphospholipid antibodies (aPL). We included 172 consecutive patients with aPL: 88 with primary antiphospholipid syndrome (APS), 38 with APS associated with systemic lupus erythematosus (APS-SLE), 32 with SLE and aPL but without APS (SLE-aPL), and 14 asymptomatic individuals with aPL (A-aPL). The FXIII-A Val34Leu polymorphism was assessed by polymerase chain reaction techniques. We found no significant differences in FXIII-A Leu34 allele frequencies between primary APS (allele frequency 0.22), APS-SLE (0.23), SLE-aPL (0.22) and A-aPL (0.32) patients, or between patients with (0.21) and without thrombosis (0.26). FXIII-A Leu34 allele frequencies were significantly lower in patients with thrombosis and those in the upper fibrinogen tertile (>3.40 g/l) (allele frequency 0.07) compared with patients without thrombosis in the upper fibrinogen tertile (0.29) and patients with (0.29) and without (0.25) thrombosis in the mid- and lower fibrinogen tertiles. The FXIII-A Leu34 allele had a protective effect against thrombosis in patients in the upper fibrinogen tertile (odds ratio [OR] = 0.20, 95% confidence interval [CI] 0.07-0.60) but not in those in the other tertiles (OR = 1.20, 95% CI 0.67-2.16). The FXIII-A Leu34 allele seems to have a protective effect on the development of thrombosis in patients with aPL, but only in those with high plasma fibrinogen values.
Related JoVE Video
Behçet disease-associated uveitis successfully treated with golimumab.
Ocul. Immunol. Inflamm.
Show Abstract
Hide Abstract
Over the past decade, the off-label use of biologic agents such as TNF-? antagonists, including infliximab and adalimumab, has improved the treatment armamentarium for refractory immune-mediated uveitis, with particular success in Behçet disease-associated uveitis. Golimumab is a novel fully human anti-TNF-? monoclonal antibody that has been approved for the treatment of rheumatoid arthritis, psoriatic arthritis, and ankylosing spondylitis, with very promising results. Herein, the authors present the use of GLM in a case of Behçet uveitis refractory to other TNF-? blockers. There are only two reports in the literature about the use of GLM in uveitis, describing four patients with JIA-associated uveitis and a case of idiopathic retinal vasculitis. To the authors knowledge, this is the first report about the use of GLM in Behçet uveitis.
Related JoVE Video
Association of allelic variants of factor V Leiden, prothrombin and methylenetetrahydrofolate reductase with thrombosis or ocular involvement in Behçets disease: a systematic review and meta-analysis.
Autoimmun Rev
Show Abstract
Hide Abstract
Thrombosis is frequent in patients with Behçets disease (BD), although the exact cause remains uncertain. Some single nucleotide polymorphism (SNP) (G1691A in factor V gene, also called factor V Leiden [FVL], G20210A in prothrombin gene and C677T in methyltetrahydrofolate reductase [MTHFR] gene) have been associated with thrombosis and ocular involvement in BD with controversial results.
Related JoVE Video
Influence of the STAT3 genetic variants in the susceptibility to psoriatic arthritis and Behcets disease.
Hum. Immunol.
Show Abstract
Hide Abstract
Signal-transducer and activator of transcription protein 3 (STAT3) gene encodes a transducer and transcription factor that plays an important role in many cellular processes such as cell growth, apoptosis and immune response. Several STAT3 genetic variants have been associated to different autoimmune diseases. Our aim was to reveal the possible STAT3 influence in other immune-mediated diseases such as psoriatic arthritis (PsA) and Behcet disease (BD).
Related JoVE Video
Pars plana vitrectomy for vitreoretinal complications of Behçet uveitis.
Eur J Ophthalmol
Show Abstract
Hide Abstract
Purpose. To evaluate the role of pars plana vitrectomy (PPV) for the treatment of vitreoretinal complications of ocular Behçet disease (BD).?Methods. Retrospective review of the medical records of all patients who underwent PPV for vitreoretinal complications of Behçet uveitis in the Ophthalmology Department of the Hospital Clinic of Barcelona, Barcelona, Spain, from January 1994 until December 2011. Preoperative and postoperative best-corrected visual acuities (BCVA), ocular findings, treatments, surgical indications, and outcomes were evaluated in all patients.?Results. Twelve patients (14 eyes) were included in the study. Indications for PPV were the following: vitreous hemorrhage in 4 eyes (28.5%), persistent vitreous opacities in 3 eyes (21.4%), rhegmatogenous retinal detachment in 2 eyes (14.3%), macular hole in 2 eyes (14.3%), persistent cystoid macular edema in 2 eyes (14.3%), and combined tractional and rhegmatogenous retinal detachment in 1 eye (7.1%). At last examination (median follow-up 105 months, range 6-209 months), BCVA increased 3 Snellen lines or more in 6 eyes (42.9%). Immunosuppressive treatment could be reduced in 5 patients (41.6%). No serious ocular inflammatory episodes presented during the follow-up in those operated patients. ?Conclusions. In this retrospective series with long-term follow-up, PPV can treat secondary vitreoretinal complications of Behçet uveitis, restoring or stabilizing vision without major adverse events.
Related JoVE Video
Relapsing catastrophic antiphospholipid syndrome potential role of microangiopathic hemolytic anemia in disease relapses.
Semin. Arthritis Rheum.
Show Abstract
Hide Abstract
To analyze the clinical and laboratory characteristics of patients with catastrophic antiphospholipid syndrome (APS) who suffer relapses.
Related JoVE Video
Adalimumab for the treatment of Behçets disease: experience in 19 patients.
Rheumatology (Oxford)
Show Abstract
Hide Abstract
To describe the experience of two tertiary Spanish centres (Hospital Clínico San Cecilio, Granada and Hospital Clínic, Barcelona) with the use of adalimumab for the treatment of severe clinical manifestations in patients with Behçets disease (BD) in whom immunosuppressive therapy had failed.
Related JoVE Video
Update on the catastrophic antiphospholipid syndrome and the "CAPS Registry".
Semin. Thromb. Hemost.
Show Abstract
Hide Abstract
Although less than 1% of patients with the antiphospholipid syndrome (APS) develop the catastrophic variant known as catastrophic antiphospholipid syndrome (CAPS), its potentially lethal outcome emphasizes its importance in clinical medicine today. However, the rarity of this variant makes it extraordinarily difficult to study in any systematic way. To collate all the published case reports as well as the newly diagnosed cases from all over the world, an international registry of patients with CAPS ("CAPS Registry") was created in 2000 by the European Forum on Antiphospholipid Antibodies (www.med.ub.es/MIMMUN/FORUM/CAPS.HTM). Currently, this database documents the entire clinical, laboratory, and therapeutic data of more than 350 fully registered patients.
Related JoVE Video
Venous thromboembolic disease in systemic autoimmune diseases: an association to keep in mind.
Autoimmun Rev
Show Abstract
Hide Abstract
Systemic autoimmune diseases are conditions of unknown etiology, characterized by the simultaneous or successive involvement of most organs and systems, as well as the presence of autoantibodies as biological markers. Venous thromboembolic disease has a higher incidence in this population when compared to healthy individuals. This responds to the increase in congenital and acquired risk factors in this group. One of the main risk factors is linked to the presence of antiphospholipid antibodies, whose prevalence is increased among patients with such conditions.
Related JoVE Video
Related JoVE Video
Cardiac valve replacement in patients with antiphospholipid syndrome.
Arthritis Care Res (Hoboken)
Show Abstract
Hide Abstract
To analyze the results of cardiac valve replacement in a multicenter cohort of patients with antiphospholipid syndrome (APS) and to identify prognostic factors of poor outcome.
Related JoVE Video
Val247Leu ?2-glycoprotein-I allelic variant is associated with antiphospholipid syndrome: systematic review and meta-analysis.
Autoimmun Rev
Show Abstract
Hide Abstract
Previous studies have suggested that the possession of the Val/Val genotype of the Val247Leu polymorphism of the ?(2)-glycoproteinI (?(2)-GPI) gene may be associated with antiphospholipid syndrome (APS), and, among patients with APS, with the production of anti-?(2)-GPI antibodies or the development of thrombosis. Given the controversial results reported, the aim of this work is to combine previous findings by means of a systematic review and a meta-analysis.
Related JoVE Video
Lymphoplasmacytic lymphoma causing light chain cast nephropathy.
Nephrol. Dial. Transplant.
Show Abstract
Hide Abstract
Plasma cell dyscrasias are frequently associated with kidney disease through the production of monoclonal immunoglobulin but with a diverse set of pathologic renal patterns. While almost all patients with a renal biopsy showing a cast nephropathy have myeloma, kidney involvement associated with pathological immunoglobulin light chains and lymphoma is rare. To our knowledge, this is the first report of a cast nephropathy associated with lymphoplasmacytic lymphoma. We emphasize the relation between light chain deposition and renal dysfunction in this disease with production of light chains. A therapeutic approach that decreases light chain production appears to be warranted in these patients.
Related JoVE Video

What is Visualize?

JoVE Visualize is a tool created to match the last 5 years of PubMed publications to methods in JoVE's video library.

How does it work?

We use abstracts found on PubMed and match them to JoVE videos to create a list of 10 to 30 related methods videos.

Video X seems to be unrelated to Abstract Y...

In developing our video relationships, we compare around 5 million PubMed articles to our library of over 4,500 methods videos. In some cases the language used in the PubMed abstracts makes matching that content to a JoVE video difficult. In other cases, there happens not to be any content in our video library that is relevant to the topic of a given abstract. In these cases, our algorithms are trying their best to display videos with relevant content, which can sometimes result in matched videos with only a slight relation.