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1.
Eur J Intern Med ; 2020 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-32014364

RESUMO

Systemic lupus erythematosus (SLE) is the most paradigmatic disorder within systemic autoimmune diseases. The concept and principles of treat-to-target (T2T) in SLE were established half a decade ago and, since then, remarkable advances have been made. An international consensus was organized in order to define and unify the term remission, although plurality, with subtle nuances still exists and has not been overcome. Also, lupus low disease activity state (LLDAS) was coined as an alternative and, perhaps, more realistic target. Both of them have proven to be meaningful in terms of improving several outcomes, and have opened the path for future research in clinical trials. This review arises from the need to summarize the current state of some of the recommendations of the T2T task force.

3.
Clin Exp Rheumatol ; 2020 Jan 14.
Artigo em Inglês | MEDLINE | ID: mdl-31969223

RESUMO

OBJECTIVES: Diffuse cutaneous systemic sclerosis (dcSSc) is associated with anti-topoisomerase (ATA) whereas limited cutaneous (lcSSc) and sine scleroderma (ssSSc) are mainly associated with anti-centromere antibody (ACA). Serodiscordant patients were defined as lcSSc subjects with ATA, dcSSc with ACA, and ssSSc with ATA. The aim of the present study was to compare the clinical manifestations and prognosis between serodiscordant patients and their counterparts (those with lcSSc with ACA, dcSSc with ATA and ssSSc with ACA, respectively). METHODS: From the Spanish Scleroderma Registry we selected those patients in which skin involvement (dcSSc, lcSSc or ssSSc) was detailed at baseline and last visit and ACA and ATA had been determined. Demographic, clinical characteristics, and survival data were compared according to the antibody status. RESULTS: The whole cohort comprised 901 patients and six mutually exclusive groups were defined: lcSScACA in 511 (57%) patients, lcSScATA group in 87 (10%), dcSScATA group in 172 (19%), dcSScACA group in 21 (2%), ssSScACA group in 92 (10%), and ssSScATA group in 18 (2%) patients, respectively. Interstitial lung disease (ILD) and severe ILD were more frequent in patients with dcSScATA than in those with dcSScACA. Conversely, the prevalence of isolated pulmonary hypertension (without ILD) was higher in those with dcSScACA (15% versus 2%; p=0.018). No differences were found regarding survival when comparing serodiscordant patients with the seroconcordants patients. CONCLUSIONS: In our cohort, the prevalence of serodiscordant SSc patients was low. They differed from their counterparts in some clinical manifestations. The management of patients with SSc should be guided by both serology and cutaneous subtype.

4.
Clin Exp Rheumatol ; 2020 Jan 20.
Artigo em Inglês | MEDLINE | ID: mdl-31969225

RESUMO

OBJECTIVES: To compare the main characteristics of two inception cohorts (Italian [ITC] and Spanish [SPC]) cohorts of patients with systemic lupus erythematosus (SLE) at the time of diagnosis and at one year of follow-up. METHODS: Demographic, clinical and immunological characteristics, and treatments at SLE diagnosis and at 12 months of follow-up of ITC and SPC were compared. RESULTS: One hundred and sixty-four patients in the ITC and 231 patients in the SPC were compared. the patients from ITC were younger at SLE diagnosis (41.1±15.0 years vs. 46.4±15.6 years; p<0.001) and had a higher prevalence of arthritis (62.8% vs. 45.5%; p=0.001), serositis (25.6% vs. 16.0%; p=0.026), neurological involvement (7.9% vs. 1.7%; p=0.006), and immunological abnormalities (anti-dsDNA, anti-Sm, antiphospholipid antibodies) (93.9% vs. 77.8%; p<0.001). Conversely, photosensitivity (29.5% in ITC vs. 45.9% in SPC; p=0.001) and oral ulcers (12.4% vs. 30.3%; p<0.001) were more frequent at onset of SLE in the Spanish patients. At the first 12 months of follow-up, these differences were maintained. At SLE onset, more Italian patients received glucocorticoids (85.4% vs. 50.2%; p<0.001) and immunosuppressive agents. At 12 months of follow-up, more Spanish patients were treated with antimalarials (75.6% in ITC vs. 90.0% in SPC; p<0.001). Conversely, the use of glucocorticoids was lower in SPC (89.0% in ITC vs. 57.1% in SPC; p<0.001). CONCLUSIONS: These cohorts presented different profiles in terms of pattern of organ/system involvement and disease treatment, possibly as a consequence of patient selection or different disease management approaches between Italy and Spain.

5.
Demography ; 2020 Jan 27.
Artigo em Inglês | MEDLINE | ID: mdl-31989536

RESUMO

This study investigates the effect of violent crime on school district-level achievement in English language arts (ELA) and mathematics. The research design exploits variation in achievement and violent crime across 813 school districts in the United States and seven birth cohorts of children born between 1996 and 2002. The identification strategy leverages exogenous shocks to crime rates arising from the availability of federal funds to hire police officers in the local police departments where the school districts operate. Results show that children who entered the school system when the violent crime rate in their school districts was lower score higher in ELA by the end of eighth grade, relative to children attending schools in the same district but who entered the school system when the violent crime rate was higher. A 10% decline in the violent crime rate experienced at ages 0-6 raises eighth-grade ELA achievement in the district by 0.03 standard deviations. Models that estimate effects by race and gender show larger impacts among Black children and boys. The district-wide effect on mathematics achievement is smaller and statistically nonsignificant. These findings extend our understanding of the geography of educational opportunity in the United States and reinforce the idea that understanding inequalities in academic achievement requires evidence on what happens inside as well as outside schools.

6.
Medicine (Baltimore) ; 99(4): e18589, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31977850

RESUMO

To date, there is no clear agreement regarding which is the best method to detect a connective tissue disease (CTD) during the initial diagnosis of interstitial lung diseases (ILD). The aim of our study was to explore the impact of a systematic diagnostic strategy to detect CTD-associated ILD (CTD-ILD) in clinical practice, and to clarify the significance of interstitial pneumonia with autoimmune features (IPAF) diagnosis in ILD patients.Consecutive patients evaluated in an ILD Diagnostic Program were divided in 3 groups: IPAF, CTD-ILD, and other ILD forms. Clinical characteristics, exhaustive serologic testing, high resolution computed tomography (HRCT) images, lung biopsy specimens, and follow-up were prospectively collected and analyzed.Among 139 patients with ILD, CTD was present in 21 (15.1%), 24 (17.3%) fulfilled IPAF criteria, and 94 (67.6%) were classified as other ILD forms. Specific systemic autoimmune symptoms such as Raynaud phenomenon (19%), inflammatory arthropathy (66.7%), and skin manifestations (38.1%) were more frequent in CTD-ILD patients than in the other groups (all P < .001). Among autoantibodies, antinuclear antibody was the most frequently found in IPAF (42%), and CTD-ILD (40%) (P = .04). Nonspecific interstitial pneumonia, detected by HRCT scan, was the most frequently seen pattern in patients with IPAF (63.5%), or CTD-ILD (57.1%) (P < .001). In multivariate analysis, a suggestive radiological pattern by HRCT scan (odds ratio [OR] 15.1, 95% confidence interval [CI] 4.7-48.3, P < .001) was the strongest independent predictor of CTD-ILD or IPAF, followed by the presence of clinical features (OR 14.6, 95% CI 4.3-49.5, P < .001), and serological features (OR 12.4, 95% CI 3.5-44.0, P < .001).This systematic diagnostic strategy was useful in discriminating an underlying CTD in patients with ILD. The defined criteria for IPAF are fulfilled by a considerable proportion of patients referred for ILD.


Assuntos
Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/diagnóstico , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Autoanticorpos/imunologia , Biópsia , Doenças do Tecido Conjuntivo/patologia , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X
7.
J Rheumatol ; 47(1): 89-98, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30770503

RESUMO

OBJECTIVE: Monotherapy is an option as first-line therapy for pulmonary arterial hypertension (PAH). However, combination therapy is a beneficial alternative. Our objective was to evaluate the efficacy of monotherapy versus combination therapy in patients with systemic sclerosis (SSc)-associated PAH. METHODS: All patients with SSc-associated PAH from the Spanish Scleroderma Registry (RESCLE) were reviewed. Patients were split into 3 groups: monotherapy versus sequential combination versus upfront combination therapy. The primary endpoint was death from any cause at 1, 3, and 5 years from PAH diagnosis. RESULTS: Seventy-six patients (4.2%) out of 1817 had SSc-related PAH. Thirty-four patients (45%) were receiving monotherapy [endothelin receptor antagonist (n = 22; 29%) or phosphodiesterase-5 inhibitors (n = 12; 16%)], 25 (33%) sequential combination, and 17 (22%) upfront combination therapy. A lower forced vital capacity/DLCO in the sequential combination group was reported (2.9 ± 1.1 vs 1.8 ± 0.4 vs 2.3 ± 0.8; p = 0.085) and also a higher mean pulmonary arterial pressure in combination groups (37.2 ± 8.7 mmHg vs 40.8 ± 8.8 vs 46 ± 15.9; p = 0.026) at baseline. Treatment regimen (p = 0.017) and functional class (p = 0.007) were found to be independent predictors of mortality. Sequential combination therapy was found to be an independent protective factor (HR 0.11, 95% CI 0.03-0.51; p = 0.004), while upfront combination therapy showed a trend (HR 0.68, 95% CI 0.23-1.97; p = 0.476). Survival from PAH diagnosis among monotherapy, sequential, and upfront combination groups was 78% versus 95.8% versus 94.1% at 1 year, 40.7% versus 81.5% versus 51.8% at 3 years, and 31.6% versus 56.5% versus 34.5% at 5 years (p = 0.007), respectively. Side effects were not significantly different among groups. CONCLUSION: Combination sequential therapy improved survival in our cohort.

8.
Reumatol Clin ; 2019 Nov 26.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-31784396

RESUMO

OBJECTIVE: Pregnancy and puerperium are considered a risk situation in women with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). Therefore, specialized assessment is essential both preconception and during pregnancy and the puerperium. Likewise, it is very important that different specialists in autoimmune diseases and high-risk pregnancies participate in the follow-up of these patients in a coordinated manner. The Spanish Society of Gynaecology and Obstetrics, the Spanish Society of Internal Medicine, and the Spanish Society of Rheumatology have set up a working group for the preparation of three consensus documents. METHODS: The stages of the work were: distribution of work in three groups corresponding to the three periods related to pregnancy (preconception, during pregnancy and childbirth and puerperium), identification of key areas, exhaustive review of the literature and formulation of recommendations. RESULTS: This first document includes the 48 recommendations that address aspects related to infertility, the need for and treatments for gonadal preservation and preconception assessment. CONCLUSIONS: These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with SLE/APS during pregnancy.

9.
Reumatol Clin ; 2019 Dec 09.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-31831386

RESUMO

OBJECTIVE: In order to agree on the fundamental aspects related to the management of pregnancy in patients with systemic lupus erythematosus and antiphospholipid syndrome, the Spanish Societies of Gynaecology and Obstetrics, Internal Medicine and Rheumatology have set up a working group for the preparation of three consensus documents. METHODS: Each of the Scientific Societies involved proposed five representatives based on their experience in the field of pregnancy control in patients with autoimmune diseases. The recommendations were developed following the Delphi methodology. RESULTS: This third document contains the recommendations regarding the management of delivery, puerperium and lactation, including medication use during these periods and the care of the newborn. In addition, a section on contraception is included. CONCLUSIONS: These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with systemic lupus erythematosus/antiphospholipid syndrome during pregnancy.

10.
Front Immunol ; 10: 2755, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31849952

RESUMO

Behçet's disease (BD) is an immune-mediated vasculitis related to imbalances between the innate and adaptive immune response. Infectious agents or environmental factors may trigger the disease in genetically predisposed individuals. HLA-B51 is the genetic factor stronger associated with the disease, although the bases of this association remain elusive. NK cells have also been implicated in the etiopathogenesis of BD. A family of NK receptors, Killer-cell Immunoglobulin-like Receptor (KIR), with a very complex organization, is very important in the education and control of the NK cells by the union to their ligands, most of them, HLA class I molecules. This study aimed to investigate the contribution of certain KIR functional polymorphisms to the susceptibility to BD. A total of 466 BD patients and 444 healthy individuals were genotyped in HLA class I (A, B, and C). The set of KIR genes and the functional variants of KIR3DL1/DS1 and KIR2DS4 were also determined. Frequency of KIR3DL1*004 was lower in patients than in controls (0.15 vs. 0.20, P = 0.005, Pc = 0.015; OR = 0.70; 95% CI 0.54-0.90) in both B51 positive and negative individuals. KIR3DL1*004, which encodes a misfolded protein, is included in a common telomeric haplotype with only one functional KIR gene, KIR3DL2. Both, KIR3DL1 and KIR3DL2 sense pathogen-associated molecular patterns but they have different capacities to eliminate them. The education of the NK cells depending on the HLA, the balance of KIR3DL1/KIR3DL2 licensed NK cells and the different capacities of these receptors to eliminate pathogens could be involved in the etiopathogenesis of BD.

11.
Reumatol Clin ; 2019 Nov 25.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-31780250

RESUMO

OBJECTIVE: In order to agree on the fundamental aspects related to the management of pregnancy in patients with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS), the Spanish Societies of Gynaecology and Obstetrics, Internal Medicine and Rheumatology set up a working group for the preparation of three consensus documents. METHODS: Each of the Scientific Societies involved proposed five representatives based on their experience in the field of pregnancy control in patients with autoimmune diseases. The recommendations were developed following the Delphi methodology. RESULTS: This second document contains the recommendations regarding the management of pregnancy in women with SLE and APS, including complications such as lupus activity, congenital heart block, thrombotic and obstetric manifestations of APS and placental vascular disease. CONCLUSIONS: These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with SLE/APS during pregnancy.

12.
J Rheumatol ; 2019 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-31676692

RESUMO

OBJECTIVE: In patients with antinuclear antibodies (ANA) and undifferentiated features of systemic autoimmune disease, the coexistence of monospecific anti-dense fine speckled-70 (anti-DFS70) antibodies is associated with a lower risk of progression to overt disease. Therefore, they might help in correctly classifying ANA positive patients and avoiding unnecessary follow-up diagnostic procedures. The aim of this study was to analyze the economic impact of the introduction of the anti-DFS70 antibody test in a hospital setting. METHODS: A case-control study was performed to detect monospecific anti-DFS70 antibodies in ANA positive subjects with undifferentiated features (cases; n=124) and with a defined systemic autoimmune disease (controls; n=290). Based on current clinical practice, a decision tree was developed to represent the disease course of patients with undifferentiated features in the subsequent three years. A budget impact analysis was performed to estimate the effect of implementing the screening for anti- DFS70 antibodies in the case group on the total costs. A sensitivity analysis was conducted to calculate the impact of the uncertainty of the input parameters on the results. RESULTS: Among the 124 patients in the case group, 5 (4.0%) tested positive for anti-DFS70 antibodies versus 4/290 (1.4%) in the control group (p=NS). The mean cost per patient under the current clinical practice decreased from 3,274€ to 3,192€ in our scenario. The budget impact reports cost savings of 10,288€. CONCLUSION: The introduction of anti-DFS70 antibody test would avoid unnecessary follow-up diagnostic procedures and minimize the use of health resources generated by suspicion of a potential systemic autoimmune disease.

13.
Presse Med ; 48(11 Pt 2): 347-353, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31694791

RESUMO

Plasma exchange is a well-established therapeutic procedure commonly used in many autoimmune disorders. The beneficial effects of plasma exchange are thought to occur through the elimination of pathogenic mediators found in plasma, including autoantibodies, complement components, and cytokines. The catastrophic antiphsopholipid syndrome (CAPS) is a life-threatening variant of the antiphospholipid syndrome (APS) where several thrombosis take place in a short period of time in patients with circulating antiphospholipid antibodies. The triple therapy with anticoagulation, corticosteroids and plasma exchange or intravenous immunoglobulins has been proposed in CAPS. CAPS is a rare disease precluding the conduction of formal clinical trials. However, the observation of a better clinical course of patients who received this treatment supports their use. Plasma exchange has become an established therapeutic procedure in CAPS but there are no studies regarding the better approach and thus its use relies on the experience of the physicians in charge. The current article aims to review potential mechanisms of action of plasma exchange and the technical aspects of this procedure and will focus on its current role in CAPS, the experience published in treating this condition and the treatment protocol that we use in our institution.


Assuntos
Síndrome Antifosfolipídica/terapia , Troca Plasmática/métodos , Corticosteroides/uso terapêutico , Anticorpos Antifosfolipídeos/sangue , Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/imunologia , Terapia Combinada/métodos , Hidratação/métodos , Humanos
14.
Artigo em Inglês | MEDLINE | ID: mdl-31628810

RESUMO

OBJECTIVE: To analyse the clinical utility of trabecular bone score (TBS) evaluation for fracture risk assessment in glucocorticoid (GC)-treated patients compared with BMD assessment. METHODS: One hundred and twenty-seven patients on GC treatment were included [mean age 62 (18) years, 63% women] in this cross-sectional study. The medical history, anthropometric data, lumbar and femoral BMD (DXA) [considering osteoporosis (OP): T-score ⩽-2.5], TBS (considering degraded microarchitecture: <1.230) and dorsolumbar X-ray [to assess vertebral fractures (VF)] were evaluated. BMD and TBS sensitivity, specificity, and positive and negative predictive values (PPV, NPV) were evaluated to determine the diagnostic accuracy of the two methods. RESULTS: All patients were receiving GC treatment for autoimmune diseases during 47.7 (68.9) months at a mean daily dose of 14.5 mg; 17% had VF, 28% any type of fragility fracture (VF + non-VF), 29% OP and 52% degraded microarchitecture. Degraded microarchitecture was significantly more frequent than densitometric OP in patients with VF (76% vs 38%) and with any fragility fracture (69% vs 36%). For VF, TBS and BMD sensitivity, specificity, PPV, and NPV were 0.76, 0.53, 0.25 and 0.92, and 0.38, 0.72, 0.22 and 0.85, respectively. Specificity increased to 0.89 for VF and 0.9 for any fragility fracture on combining BMD+TBS. TBS had better ability than BMD to discriminate between patients with fracture, especially VF (area under the curve = 0.73). CONCLUSION: TBS seems to have greater discriminative power than BMD for fracture risk assessment in GC-treated patients, confirming the utility of this method as a complementary tool in the diagnosis of GC-induced OP.

15.
Artigo em Inglês | MEDLINE | ID: mdl-31580459

RESUMO

OBJECTIVES: To compare clinical features, laboratory data and fetal-maternal outcomes between 1000 women with obstetric APS (OAPS) and 640 with aPL-related obstetric complications not fulfilling Sydney criteria (non-criteria OAPS, NC-OAPS). METHODS: This was a retrospective and prospective multicentre study from the European Registry on Obstetric Antiphospholipid Syndrome. RESULTS: A total of 1650 women with 5251 episodes, 3601 of which were historical and 1650 latest episodes, were included. Altogether, 1000 cases (OAPS group) fulfilled the Sydney classification criteria and 650 (NC-OAPS group) did not. Ten NC-OAPS cases were excluded for presenting thrombosis during follow-up. All cases were classified as category I (triple positivity or double positivity for aPL) or category II (simple positivity). Overall, aPL laboratory categories showed significant differences: 29.20% in OAPS vs 17.96% in NC-OAPS (P < 0.0001) for category I, and 70.8% in OAPS vs 82% in NC-OAPS (P < 0.0001) for category II. Significant differences were observed when current obstetric complications were compared (P < 0.001). However, major differences between groups were not observed in treatment rates, livebirths and thrombotic complications. In the NC-OAPS group, 176/640 (27.5%) did not fulfil Sydney clinical criteria (subgroup A), 175/640 (27.34%) had a low titre and/or non-persistent aPL positivity but did meet the clinical criteria (subgroup B) and 289/640 (45.15%) had a high aPL titre but did not fulfil Sydney clinical criteria (subgroup C). CONCLUSION: Significant clinical and laboratory differences were found between groups. Fetal-maternal outcomes were similar in both groups when treated. These results suggest that we could improve our clinical practice with better understanding of NC-OAPS patients.

17.
Rheumatol Int ; 39(11): 1875-1882, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31522232

RESUMO

The objective of this study was to evaluate the impact of digital ulcers (DUs) in daily life of systemic sclerosis (SSc) Spanish patients. We developed a multicenter observational study to compare functional disability in SSc patients with active DUs vs. those without DUs. An additional correlation between perception of patients and physicians on disability due to DUs was performed. A total of 199 patients were enrolled, 70 (35%) with DUs. Patients with DUs were younger (48 vs. 58 years; p < 0.001) and had more frequently the diffuse subtype of SSc (45 vs. 24%; p = 0.004) than patients without DUs. Patients with DUs showed significantly higher scores in the Cochin Hand Function Scale overall (p < 0.002) and for each of its five dimensions. They also showed higher scores in the Systemic Sclerosis Health Assessment Questionnaire items related to hand function such as, dress and self-care (p < 0.013), eat (p < 0.013) and grip (p < 0.03), and higher Visual Analogic Scale scores for pain (p < 0.013), trouble related with Raynaud's Phenomenon (p < 0.001) and sense of severity (p < 0.004). Impact on daily activities was significantly higher in patients with DUs (p = 0.002), with a non-significant trend to experience higher impact on work productivity (p = 0.07). A high correlation was found between DUs patients and physicians opinion on the impact of DUs (daily life: Pearson R = 0.86; work productivity: Pearson R = 0.87). Study findings show an impaired hand function and increased disability for daily life activities and work productivity in SSc patients with DUs compared with patients without DUs in Spanish population.

18.
Clin Ther ; 41(9): 1839-1862, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31405527

RESUMO

PURPOSE: The treatment of thrombosis in patients with antiphospholipid syndrome (APS) usually requires long-term anticoagulation with vitamin K antagonists. The effectiveness of direct oral anticoagulants (DOACs) in APS has not been fully addressed. The purpose of this research was to analyze the efficacy (thrombotic event-free time) and tolerability (bleeding events) of DOACs in patients with APS. METHODS: We performed a descriptive analysis of a systematic review of data from patients with APS treated with DOACs reported in the literature, via EMBASE, PubMed, and the European League Against Rheumatism and American College of Rheumatology congresses. After systematic review, a meta-analysis of data from clinical trials was performed. FINDINGS: A total of 728 patients, accounting for 731 courses of treatment with DOACs, were identified. The majority (48.3%) presented with triple anti-phospholipid antibody positivity. The prevalence of thrombosis during DOAC treatment was 13.9%. Analysis of risk factors for recurrent thrombosis suggested that a higher mean (SD) number of prior thrombotic events (1.80 [0.87] vs 1.67 [1.45]; P = 0.012), history of combined arterial and venous thrombosis (27.3% vs 9.2% [P < 0.0001]; odds ratio [OR] = 3.72 [95% CI, 1.91-7.25]), previous treatment with LMWH (9.8% vs 1.1% [P = 0.04]; OR = 9.95 [95% CI, 1.08-91.97]), use of immunosuppressant treatment (41.7% vs 12.7% [P = 0.03]; OR = 4.9 [95% CI, 1.21-19.76]), and no reason to switch anticoagulant treatment other than patient's decision (32% vs 2.8% [P = 0.001]; OR = 16.24 [95% CI, 3.16-83.52]) were associated with a high risk for re-thrombosis. Meta-Analysis did not show statistically relevant difference in risk of thrombosis or bleeding comparing warfarin with DOACs. IMPLICATIONS: The findings from this systematic literature review and meta-analysis suggest that patients treated with DOACs and having risk factors such as history of recurrent thrombosis, a history of combined arterial and venous thrombosis, or a need for immunosuppressant treatment, may have higer ratio of thrombotic recurrence. There are limited data to inform decisions on the use of DOACs in patients with APS with different or no risk factors.

19.
Clin Rheumatol ; 38(9): 2529-2539, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31020474

RESUMO

INTRODUCTION: Research describes higher incidence of neurodevelopmental disorders and learning disabilities in offspring of women affected by lupus. Factors implied are pregnancy and delivery adversities and exposure to maternal antibodies and cytokines. Little is known about the offspring immunological condition or the relation between offspring and maternal condition. OBJECTIVES: This study was conducted in order to analyze immunological configuration, psychopathology, and neuropsychological performance of young offspring of women with lupus, in comparison with healthy controls and in relation to maternal psychophysical condition. METHODS: Twenty-one offspring aged 8-17 of 17 women with lupus and 34 controls were recruited. Pregnancy conditions, stress factors, and immunological, psychopathological, and neuropsychological characteristics were compared. Immunological tests included standard lupus screening, lupus-related autoantibodies, antibodies against GluN2 subunit of the N-methyl-D-aspartate receptor (NMDAR) (anti-DWEYS Ab), and levels of ten cytokines (IL-1ß, IL-2, IL-4, IL-5, IL-6, IL-8, IL-10, GMCSF, IFN-γ, TNF-α). RESULTS: Offspring had lower leukocyte count (p = 0.001) and higher levels of anti-dsDNA Ab (p = 0.022), anti-DWEYS-GluN2 Ab (p < 0.001), and eight cytokines (IL-1ß, IL-2, IL-4, IL-5, IL-6, IL-10, TNF-α-all p < 0.001-and IFN-γ, p = 0.026) than controls. Their cytokine levels did not differ from their mothers'; 23.9% of offspring met the criteria for a clinical psychiatric diagnosis. No differences were found in intelligence measures. Various neuropsychological scores correlated inversely with maternal psychophysical health. CONCLUSIONS: Offspring's profile suggests proinflammatory and autoimmune activation. Their rate of psychiatric diagnosis appears higher than in the general population, and their cognitive performance is related to maternal psychophysical health. Longitudinal research might investigate whether immunological and psychosocial conditions influence psychopathology and cognition. Graphical abstract The hypothesized sequence for physical and neuropsychological development for the SLE offspring.

20.
Autoimmun Rev ; 18(4): 406-414, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30772493

RESUMO

AIM: To analyse the clinical features, laboratory data and foetal-maternal outcomes, and follow them up on a cohort of 1000 women with obstetric antiphospholipid syndrome (OAPS). METHODS: The European Registry of OAPS became a registry within the framework of the European Forum on Antiphospholipid Antibody projects and was placed on a website in June 2010. Thirty hospitals throughout Europe have collaborated to carry out this registry. Cases with obstetric complaints related to antiphospholipid antibodies (aPL) who tested positive for aPL at least twice were included prospectively and retrospectively. The seven-year survey results are reported. RESULTS: 1000 women with 3553 episodes were included of which 2553 were historical and 1000 were latest episodes. All cases fulfilled the Sydney classification criteria. According to the laboratory categories, 292 (29.2%) were in category I, 357 (35.7%) in IIa, 224 (22.4%) in IIb and 127 (12.7%) in IIc. Miscarriages were the most prevalent clinical manifestation in 386 cases (38.6%). Moreover, the presence of early preeclampsia (PE) and early foetal growth restriction (FGR) appeared in 181 (18.1%) and 161 (16.1%), respectively. In this series, 448 (44.8%) women received the recommended OAPS treatment. Patients with recommended treatment had a good live-birth rate (85%), but worse results (72.4%) were obtained in patients with any treatment (low-dose aspirin (LDA) or low-molecular-weight heparin (LMWH) not on recommended schedule, while patients with no treatment showed a poor birth rate (49.6%). CONCLUSION: In this series, recurrent miscarriage is the most frequent poor outcome. To avoid false-negative diagnoses, all laboratory category subsets were needed. OAPS cases have very good foetal-maternal outcomes when treated. Results suggest that we were able to improve our clinical practice to offer better treatment and outcomes to OAPS patients.


Assuntos
Síndrome Antifosfolipídica/epidemiologia , Complicações na Gravidez/epidemiologia , Aborto Habitual/tratamento farmacológico , Aborto Habitual/epidemiologia , Aborto Habitual/etiologia , Adulto , Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/tratamento farmacológico , Síndrome Antifosfolipídica/imunologia , Aspirina/uso terapêutico , Estudos de Coortes , Europa (Continente)/epidemiologia , Feminino , Seguimentos , Heparina de Baixo Peso Molecular/uso terapêutico , Humanos , Recém-Nascido , Gravidez , Complicações na Gravidez/tratamento farmacológico , Complicações na Gravidez/imunologia , Resultado da Gravidez , Sistema de Registros , Estudos Retrospectivos , Adulto Jovem
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