BackgroundAcute transverse myelitis (ATM) is an infrequent but severe complication of systemic lupus erythematosus (SLE). The purpose of study was to describe clinical features and prognostic factors of patients with SLE-related ATM.MethodsIn this medical records review study, data were collected from 60 patients from 16 centers seen between 1996 and 2017 who met diagnostic criteria for SLE and myelitis as defined by the American College of Rheumatology/Systemic International Collaborating Clinics and the Working Group of the Transverse Myelitis Consortium, respectively. Objective neurological impairment was measured with American Spinal Injury Association Impairment Scale (AIS) and European Database for Multiple Sclerosis Grade Scale (EGS).ResultsAmong patients included, 95% (n = 57) were female, and the average age was 31.6 ± 9.6 years. Myelitis developed after diagnosis of SLE in 60% (n = 36). Symmetrical paraparesis with hypoesthesia, flaccidity, sphincter dysfunction, AIS = A/B, and EGS ≥ 8 was the most common presentation. Intravenous methylprednisolone was used in 95% (n = 57), and 78.3% (n = 47) received intravenous cyclophosphamide. Sensory/motor recovery at 6 months was observed in 75% (42 of 56), but only in 16.1% (9 of 56) was complete. Hypoglycorrhachia and EGS ≥ 7 in the nadir were associated with an unfavorable neurological outcome at 6 months (p < 0.05). A relapse rate during follow-up was observed in 30.4% (17 of 56). Hypoglycorrhachia and hypocomplementemia seem to be protective factors for relapse. Intravenous cyclophosphamide was associated with time delay to relapse.ConclusionsSystemic lupus erythematosus–related ATM may occur at any time of SLE course, leading to significant disability despite treatment. Relapses are infrequent and intravenous cyclophosphamide seems to delay it. Hypoglycorrhachia, hypocomplementemia, and EGS at nadir are the most important prognostic factors.
Introduction: After more than 20 years of sustained work, the Latin American Group for the Study of Lupus (GLADEL) has made a significant number of contributions to the field of lupus, not only in the differential role that race/ethnicity plays in its course and outcome but also in several other studies including the beneficial effects of using antimalarials in lupus patients and the development of consensus guidelines for the treatment of lupus in our region. Methods: A new generation of “Lupus Investigators” in more than 40 centers throughout Latin America has been constituted in order to continue the legacy of the investigators of the original cohort and to launch a novel study of serum and urinary biomarkers in patients with systemic lupus erythematosus. Results: So far, we have recruited 807 patients and 631 controls from 42 Latin-American centers including 339 patients with SLE without renal involvement, 202 patients with SLE with prevalent but inactive renal disease, 176 patients with prevalent and active renal disease and 90 patients with incident lupus nephritis. Conclusions: The different methodological aspects of the GLADEL 2.0 cohort are discussed in this manuscript, including the challenges and difficulties of conducting such an ambitious project.
A, Centurión O, Duarte M.Manifestaciones clínicas y laboratoriales en el Lupus Eritematoso Sistémico. Mem. Inst. Investig. Cienc. Salud. 2016;14(1):94-109. R E S U M E NEl Lupus eritematoso sistémico (LES) es una enfermedad autoinmune compleja que se caracteriza por su capacidad de afectar a diversos órganos, lo que determina las diferentes manifestaciones clínicas objetivadas durante la evolución de la enfermedad. De forma asociada se ha descrito que estas manifestaciones presentan una variación geográfica o étnica, siendo por lo general menos grave en pacientes con ascendencia europea que en aquellos que presentan ascendencia africana, asiática o hispana. Alteraciones, tanto del sistema inmune adaptativo (células T y B) como del innato (Toll like receptorx-TLR), contribuyen al desarrollo del LES. Las células B tienen su papel en la producción de los autoanticuerpos (i.e. anticuerpos anti-ADN y anticuerpos antinucleosoma) y de determiandas citocinas. Las pruebas de laboratorio son de gran valor cuando se evalúa a un paciente con sospecha de enfermedad autoinmune. Los resultados pueden confirmar el diagnóstico, estimar la severidad de la enfermedad, evaluar el pronóstico y son de suma utilidad para el seguimiento de la actividad del LES.Palabras clave: manifestaciones clínicas, lupus eritematoso sistémico, análisis laboratoriales Clinical and laboratory manifstations in Systemic Lupus Erythematosus A B S T R A TSystemic Lupus Erythematosus (SLE) is a complex autoimmnune disease characterized by its ability to affect different organs, which determines different clinical manifestations observed during the course of the disease. It has been described that these manifestations have geographic or ethnic varations being generally less serious in patients of European descent than in those with African, Asian or Hispanic descents. Alterations of both the adaptative (T and B cells) and innate (Toll like receptorx-TLR) immnune systems contribute to the development of SLE. B cells have a role in the production of autoantibodies (i.e. anti-DNA and anti-nucleosome antibodies) and some cytokines. Laboratory tests are invaluable when evaluating a patient with suspected autoimmune disease. The results can confirm the diagnosis, estimate the severity of the disease, assess prognosis and are extremely useful for monitoring the activity of SLE.
D engue is among the most important arboviral diseases worldwide; it is reported in 128 countries with an estimated 50 million to 100 million cases annually. 1 It represents a major public health problem in Paraguay. Dengue virus (DENV) infections may result in subclinical illness or evolve to a potentially fatal disease, known as severe dengue. Studies indicate that DENV infections can trigger a flare of systemic lupus erythematosus (SLE) and that co-occurrence can lead to severe cases. [2][3][4] The purpose of this report is to present a case of a patient with SLE and a delayed diagnosis of dengue.
BackgroundSurvival of biological therapies (BT) may be considered as an indicator of efficacy and safety of the drug. BT survival have been studied mainly in adult’s patients, whereas only few studies have been focused on paediatric population to date.ObjectivesTo analyse and compare BT survival in adults and juvenile onset arthritis patients from BIOBADAGUAY registry.MethodsPatients with a chronic inflammatory arthritis enrolled in the Paraguayan-Uruguayan biological register (BIOBADAGUAY) between2015 and 2017 where included. For this study, patients were divided in two groups: 1. Adults with anychronic inflammatory arthritis and 2. Patients with juvenile idiopathic arthritis (JIA). To compare the groups according to BT, only the first biotherapy was considered.Survival analysis was performed using Kaplan-Meier estimators and proportional hazard regression model. First we analysed global BT survival in both groups; secondly we compare BT survival between groups.ResultsFrom 778 BTs(etanercept n=184, adalimumab n=440, rituximab n=44, infliximab n=27, tocilizumab n=75, and others n=8), 556 where identify as first line BTs. Of these, only adalimumab and etanercept were included in the study due to sufficient number prescriptions in both groups for the analysis.We found a mean survival times for adults of 289 (±20.7 SD) weeks for etanercept and 287 (±8.6 SD) weeks for adalimumab. In JIA patients the mean survival were 243 (±26.0 SD) and 216 (±24.0 SD) weeks for etanercept and adalimumab respectivelyWhen comparing survival between groups, we found that JIA presented more discontinuation of BT when compare with adult patients (p=4.4 × 10–4, HR=0.51 [95%CI, 0.35–0.73]). Similar results were observed when analysing only etanercept (p=3.92 × 10–2; HR=0.50 [95% CI, 0.26–0.97]) or adalimumab (p=1.20 × 10–3; HR=0.48 [95% CI, 0.30–0.75])treatments. Then we analysed withdrawn motive in JIA patients, and found that remission was the principal reason of discontinuation in this group of patients.Finally, we stratified survival analysis by discontinuation according to adverse events, and found that JIA group presented a lower risk of discontinuation due to adverse events than adults (p=1.96 × 10–1; HR=2.18 [95% CI, 0.67–7.07]).ConclusionsIn our study we have analysed mean BT survival between adults and JIA at the BIOBADAGUAY registry. When we compared both groups of patients it was observed that JIA patients presented more BT discontinuation but due to remission.Disclosure of InterestNone declared
BackgroundBIOBADAGUAY is the Paraguayan/Uruguayan registry of adverse events (AE) in patients with inflammatory rheumatic conditions under biologic therapy (BT)ObjectivesTo determine the frequency and severity of AE in patients under BT from the BIOBADAGUAY registryMethodsProspective, observational study of undetermined length to verify the efficacy, safety and survival of the BT. The methodology applied is available at https//biobadaguay.ser.es. For the present study epidemiological and clinical variables, BT, type and severity of AE were analysed. The incidence rate (IR) was calculated as the total number of adverse events per 1000 patients/year and the incidence rate ratio (IRR) was analysed using the Poisson regression model.Results778 BT were analysed (56.6% adalimumab, 23.7% etanercept, 9.6% tocilizumab, 5.7% rituximab, 3.5%infliximabm, 0.5%golimumab, 0.38%infliximab biosimilar, 0.13% abatacept). In these, 330 AE were observed, 256 (77.6%) mild and 74 (24.4%) severe. The global IR of AE was 143.9 (95% CI,128.8–160.8) and 32.6 (95% CI,25.3–40.5), 111.6 (95% CI, 98.4–126.2) for severe and mild respectively. Infection was the most frequent AE in 175 (53.0%) and 39 (22.3%) of them were severe. Infection’s IR was 76.3 (95% CI,65.4–88.5), 59.3 (95% CI,49.76–70.2) and 17.0 (95% CI,12.1–23.3) for global, severe and mild respectively. Out of the 39 severe AE, respiratory infections were the most frequent in 43.6% of the cases. 5 tuberculosis, 6 malignancies and 6 deaths were observed.When analysing the IR according to diagnosis, Idiopathic Juvenile Arthritis (JIA) was associated with a higher IR of global AE when comparing to the other diagnosis (IRR=2.3 [95% CI,1.6–3.4] p=4.27 × 10–6). RA diagnosis was significantly associated with a higher risk of severe AE (IRR=2.20 [95% CI,1.2–4.1] p=1.17 × 10–2). tocilizumab was significantly associated with a higher incidence of global AE, (IRR=2.69 [95% CI, 1.90–3.82] p=3.13 × 10–8) and severe ones (IRR=3.34 [95% CI,1.81–6.1] p=1.10 × 10–4). Adalimumab was significantly associated with a lower rate of global AE(IRR=0.6 [95% CI, 0.4–0.8] p=1.86 × 10–4).Abstract AB0452 – Table 1Incidence rate and incidence rate ratio of adverse events according to diagnosis and biological therapyVariableIRIRRP value Psoriasic Arthritis88 [52,2–139,1]0,59 [0,33–1,04]7,03e-02Ankylosis Spondylitis102,3 [68,0–147,8]0,68 [0,40–1,17]1,63e-01Rheumatoid Arthritis134,2 [115,6–155,0]0,85 [0,62–1,15]2,81e-01Idiopatic Juvenile Arhritis286,4 [229,4–353,2]2,34 [1,63–3,37]4,27e-06Adalimumab113,5 [96,9–132,1]0,57 [0,43–0,77]1,86e-04Etanercept190,4 [152,7–234,6]1,44 [1,03–2,02]3,30e-02Rituximab66,9 [26,9–137,8]0,45 [0,18–1,13]8,93e-02Infliximab75,0 [24,3–175,0]0,51 [0,21–1,23]1,35e-01Tocilizumab340,0 [261,8–434,2]2,69 [1,90–3,82]3,13e-08IR: incidence, IRR: incidence ratio (per 1000 patients year)ConclusionsAE were mild in general and infections were the most frequent. In the present study, it was found that JIA and treatment with tocilizumab presented a higher IRR of AE while RA presented a higher rate of severe...
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