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OBJECTIVE: One of the potential factors that cause systemic lupus erythematosus (SLE) development is autophagy. Immunity-related GTPase family M protein (IRGM) has been shown to be linked to immune-mediated diseases. The aim of the current study was to assess the role of the IRGM-autophagy gene in SLE susceptibility in an Egyptian population and its relation to lupus nephritis. METHODS: A case-control study was conducted in which a total of 200 subjects (100SLE and 100 healthy controls) were enrolled. Two single-nucleotide polymorphisms (SNPs) (rs10065172 and rs4958847) were genotyped. Genotypes and alleles analysis was conducted to compare between cases and controls, as well as a stratification analysis was conducted on the presence or absence of lupus nephritis. RESULTS: Among selected SNPs of IRGM, no association was found between both SNPs and SLE susceptibility. For rs10065172, the major expressed genotype was CC (61% and 71%) (Adj OR= 2.9, 95%= 0.545-15.5), followed by TC (34% and 27%) (Adj OR= 1.985, 95% = 0.357-11.041) in cases and controls, respectively. For rs4958847, AA and AG were comparably expressed in case [(43% and 39%) (Adj OR= 1.073, 95% = 0.483-2.382)] and control [(41% and 43%) (Adj OR= 1.24, 95% = 0.557- 2.763)], respectively. Additionally, no relationship among both SNPs and gender, lupus nephritis, disease activity, or disease duration, was observed. CONCLUSION: IRGM SNPs (rs10065172 and rs4958847) expression was comparable among SLE patients and controls of the Egyptian cohort. Genotype and allele frequency of IRGM SNPs did not differ in lupus nephritis and non-lupus nephritis patients.
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Doença de Crohn , Lúpus Eritematoso Sistêmico , Nefrite Lúpica , Humanos , Polimorfismo de Nucleotídeo Único , Predisposição Genética para Doença , Doença de Crohn/epidemiologia , Doença de Crohn/genética , Estudos de Casos e Controles , Egito , Proteínas de Ligação ao GTP/genética , Lúpus Eritematoso Sistêmico/genética , Nefrite Lúpica/genéticaRESUMO
Objective: The aim of this study was to reach a consensus on an updated version of the recommendations for the diagnosis and Treat-to-Target management of osteoporosis that is effective and safe for individuals with chronic kidney disease (CKD) G4-G5D/kidney transplant. Methods: Delphi process was implemented (3 rounds) to establish a consensus on 10 clinical domains: (1) study targets, (2) risk factors, (3) diagnosis, (4) case stratification, (5) treatment targets, (6) investigations, (7) medical management, (8) monitoring, (9) management of special groups, (10) fracture liaison service. After each round, statements were retired, modified, or added in view of the experts' suggestions, and the percent agreement was calculated. Statements receiving rates of 7-9 by more than 75% of experts' votes were considered as achieving consensus. Results: The surveys were sent to an expert panel (n = 26), of whom 23 participated in the three rounds (2 were international experts and 21 were national). Most of the participants were rheumatologists (87%), followed by nephrologists (8.7%), and geriatric physicians (4.3%). Eighteen recommendations, categorized into 10 domains, were obtained. Agreement with the recommendations (rank 7-9) ranged from 80 to 100%. Consensus was reached on the wording of all 10 clinical domains identified by the scientific committee. An algorithm for the management of osteoporosis in CKD has been suggested. Conclusion: A panel of international and national experts established a consensus regarding the management of osteoporosis in CKD patients. The developed recommendations provide a comprehensive approach to assessing and managing osteoporosis for all healthcare professionals involved in its management.
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BACKGROUND: Psychiatric disorders, including schizophrenia, could herald other manifestation( s) of systemic lupus erythematosus (SLE) potentially hindering timely and optimal management. Moreover, schizophrenia is among the described 'extra-criteria' manifestations of anti-phospholipid syndrome (APS). Hence, screening schizophrenia patients for SLE and APS may pose diagnostic and therapeutic implications. OBJECTIVES: Examine schizophrenia patients with no overt connective tissue disease(s) manifestation( s) for clinical and/or serologic evidence of SLE and/or APS. METHODS: The study included 92 schizophrenia patients (61 (66.3%) males) and 100 age- and gender- matched healthy controls. Both groups were tested for anti-nuclear antibodies (ANAs), antidouble stranded deoxyribonucleic acid (anti-dsDNA) antibodies, complement 3 (C3) and C4, and criteria anti-phospholipid antibodies (aPL) (anticardiolipin Immunoglobulin (Ig) G and IgM, antibeta- 2-glycoprotein I IgG and IgM, and lupus anticoagulant (LAC)). RESULTS: The patients' mean age and disease duration were 28.8 ± 8.1 and 5.7 ± 2.2 years, respectively. The prevalence of ANA positivity, height of titre, and pattern was comparable between patients and controls (p = 0.9, p = 0.8 and p = 0.1, respectively). Anti-dsDNA antibodies and hypocomplementemia were absent in both groups. A significantly higher frequency of positive LAC was observed among patients compared with controls (7.6% vs. 1%, p = 0.02), whereas other aPL were comparable between both groups. None of the patients or controls demonstrated clinically meaningful (medium or high) aPL titres. CONCLUSION: In our study, schizophrenia was solely associated with LAC. Thus, in the absence of findings suggestive of SLE or APS, routine screening for both diseases is questionable.
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Síndrome Antifosfolipídica , Lúpus Eritematoso Sistêmico , Esquizofrenia , Anticorpos Antinucleares , Anticorpos Antifosfolipídeos , Estudos de Casos e Controles , Egito , Feminino , Humanos , Imunoglobulina G , Imunoglobulina M , Inibidor de Coagulação do Lúpus , Masculino , PrevalênciaRESUMO
The study aimed to evaluate the association of demographic, clinical, and histopathologic characteristics with renal and disease outcomes. Persistent lack of partial or complete remission despite sequential induction therapy, chronic kidney disease (CKD) or endstage renal disease (ESRD), and/or mortality were determined as poor renal outcomes. Disease damage was investigated through the Systemic Lupus International Collaborating Clinics/ American College of Rheumatology Damage Index (SDI). Of 201 biopsy-proven lupus nephritis patients, a poor outcome was present in 56 (27.9%) patients, with nine (4.5%), 22 (10.9%), and 29 (14.4%) patients demonstrating lack of response, CKD, and ESRD, respectively, and the prevalence of mortality was 5.5% (11/201). The outcome was poor among males [29/201 (14.4%)] [P = 0.008; odds ratio (OR): 2.8; 95% confidence interval (CI): 1.2-6.4], yet comparable between adult- and juvenile-onset patients [80/201 (39.8%) (≤16 years)] (P = 0.6; OR: 0.8; 95% CI: 0.4-1.6). Hypertension (P <0.001; OR: 6.3; 95% CI: 2.6-14.9), elevated creatinine (P <0.001; OR: 5.2; 95% CI: 2.6-10.3), and hematuria (P <0.001; OR: 3.7; 95% CI: 1.9-7.5) at presentation, and fibrinoid necrosis [P <0.001; odds ratio (OR): 4.1; 95% confidence interval (CI): 2.1-8.1], wire loops (P = 0.006; OR: 2.4; 95% CI: 1.2-4.6), crescents (P <0.001; OR: 5.4 95% CI: 2.8-10.5), interstitial fibrosis (P = 0.001; OR: 2.7; 95% CI: 1.4-5.1), and acute vascular lesions (P = 0.004; OR: 3.6; 95% CI: 1.4-9.4) on biopsy were associated with a poor outcome. Chronic glomerular (P = 0.003) and acute vascular lesions (P <0.001), and a higher chronicity index (r = 0.1; P = 0.006) on biopsy, and frequent renal (r = 0.3; P <0.001) and extra-renal flares (r = 0.2; P <0.001) were associated with higher SDI scores. Among the studied renal and extra-renal parameters, independent predictors of higher disease damage solely included frequent renal flares (áµ= 1; P <0.001). To conclude, a poor renal outcome (27.9%) was associated with distinct features. Disease damage was associated with frequent renal flares.
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Falência Renal Crônica , Nefrite Lúpica , Insuficiência Renal Crônica , Adulto , Masculino , Humanos , Nefrite Lúpica/diagnóstico , Nefrite Lúpica/epidemiologia , Nefrite Lúpica/complicações , Estudos Retrospectivos , Egito/epidemiologia , Rim/patologia , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/epidemiologia , Falência Renal Crônica/etiologia , Insuficiência Renal Crônica/complicações , BiópsiaRESUMO
The nuclear receptor coactivator 5 (NCOA5) has been linked to several inflammatory disorders, including Behçet's disease (BD). We evaluated the expression of NCOA5 messenger RNA (mRNA) using real-time reverse transcription-polymerase chain reaction, and analyzed the rs2903908 T > C of NCOA5 using TaqMan allelic discrimination assay in 49 Egyptian BD patients and 50 controls. The NCOA5 mRNA levels were higher in patients compared to controls (p = .02), female patients compared to males (p = .037), and in patients with ocular involvement (p = .049). Non-CC genotype carriers had a higher frequency of articular manifestations compared with CC carriers (p = .047). Genotypes CC + CT were associated with reduced risk of cutaneous involvement (OR = 0.27, p = .04). CC carriers with active BD or cutaneous manifestations displayed significantly lower NCOA5 mRNA expression than TT carriers. Our results demonstrate that NCOA5 is linked to BD clinical findings and activity.
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Síndrome de Behçet , Coativadores de Receptor Nuclear , Polimorfismo de Nucleotídeo Único , Feminino , Humanos , Masculino , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/genética , Estudos de Casos e Controles , Egito/epidemiologia , Frequência do Gene , Predisposição Genética para Doença , Genótipo , Coativadores de Receptor Nuclear/genética , Coativadores de Receptor Nuclear/metabolismo , RNA Mensageiro/genética , RNA Mensageiro/metabolismoRESUMO
IgA vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is the most common cause of systemic vasculitis in childhood. Given its potential life-threatening systemic complications, early and accurate diagnosis as well as management of IgAV represent a major challenge for health care professionals. This study was carried out to attain an evidence-based expert consensus on a treat-to-target management approach for IgAV using Delphi technique. The preliminary scientific committee identified a total of 16 key clinical questions according to the patient, intervention, comparison, and outcomes (PICO) approach. An evidence-based, systematic, literature review was conducted to compile evidence for the IgAV management. The core leadership team identified researchers and clinicians with expertise in IgAV management in Egypt upon which experts were gathered from different governorates and health centers across Egypt. Delphi process was implemented (two rounds) to reach a consensus. An online questionnaire was sent to expert panel (n = 26) who participated in the two rounds. After completing round 2, a total of 20 recommendation items, categorized into two sections were obtained. Agreement with the recommendations (rank 7-9) ranged from 91.7-100%. Consensus was reached (i.e. ⩾75% of respondents strongly agreed or agreed) on the wording of all the 20 clinical standards identified by the scientific committee. Algorithms for the diagnosis and management have been suggested. This was an expert, consensus recommendations for the diagnosis and treatment of IgAV and IgA vasculitic nephritis, based on best available evidence and expert opinion. The guideline presented a strategy of care with a pathway to achieve a state of remission as early as possible. PLAIN LANGUAGE SUMMARY: Given its potential life-threatening systemic complications, early and accurate diagnosis of immunoglobulin A vasculitis represents a major challenge for health care professionals. This work provided cornerstone principles for the management of the condition. Adopting PICO approach and implementing Delphi process a consensus was reached on evidence-based treat-to-target treatment recommendations. This will endorse enhancement and consistency of care of this cohort of patients in standard practice.
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Unusual presentations of sarcoidosis pose a diagnostic challenge and warrant attention. Hematologic associations: Case 1 (37-years-old male): Pancytopenia: myelofibrosis (leading to sepsis and mortality) following a two-year quiescent course of biopsy-proven-sarcoidosis. Case 2 : (38-years-old male): Presentation with thrombocytopenia (5 × 103/cmm): immune thrombocytopenic purpura (histologically associated with megakaryocytic emperipolesis). Biopsied enlarged lymph nodes demonstrated sarcoidosis. Hematologic sarcoid involvement is usually due to granulomatous bone marrow (3.9%) or splenic infiltration (6-30%); however, the presented manifestations are scarcely reported with a potential significance that is yet to be elucidated. Case 3: Neurologic presentation: 48-years-old female: presentation with bilateral sensorineural hearing loss and facial palsy. Brain magnetic resonance imaging showed leptomeningeal thickening. Biopsied enlarged lymph nodes showed sarcoidosis. Case 4: Neurologic and renal manifestations: 13-years-old male (family history of sarcoidosis): Presenting with acute headache, investigations showed elevated serum creatinine (2.1 mg/dL) and angiotensin converting enzyme, and computed tomography chest and abdominal findings characteristic of sarcoidosis. Associated benign increased intracranial and acute tubulointerstitial nephritis (with eosinophils) were diagnosed upon concordant workup. Of sarcoidosis neurologic affection (5-10%), cranial nerve(s) involvement is among the most common (25-50% of neurosarcoid affection), particularly that of the facial nerve (Case 3). Leptomeningeal enhancement is among the most common neurosarcoid radiologic findings (30-40%). Whereas benign increased intracranial tension (Case 4) is much less reported. Among sarcoidosis renal involvement (35-50%), interstitial nephritis usually presents with granulomatous renal lesions, yet its sole association with sarcoidosis is unusual (Case 4). The portrayed atypical hematologic, neurologic, and renal manifestations further emphasize the masquerading nature of sarcoidosis.
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ABSTRACT Background: MicroRNAs (miRNAs) are non-coding RNAs that regulate gene expression post-transcriptionally. Accumulating evidence indicates that the miR-30 family takes part in the development of multiple tissues and organs, and is a potential contributor to various dis eases, including autoimmune disorders such as systemic lupus erythematosus (SLE). The aim of this study was to evaluate the expression of miR-30e-5p, a member of the miR-30 fam ily, and investigate its potential relationship to clinical characteristics and possible disease activity in an Egyptian SLE cohort. Methods: Serum samples from 40 SLE patients and 37 age and gender matched healthy sub jects were tested for miR-30e-5p expression level using the Taqman quantitative reverse transcription-polymerase chain reaction. Analysis was performed using the 2 - AACT method. Results: The mean age of the patients was 28.7 ± 7.9 years, with a mean disease duration of 6.4 ±5.3 years. The median fold change in serum miR-30e-5p among our SLE cohort was significantly higher 1.748 (0.223-20.485) compared to the control group 0.877 (0.058-3.522) (P = 0.02). Receiver operating characteristic curve analysis revealed that miR-30e-5p expres sion level can discriminate SLE patients from controls at a cut-off value >1.06 with the area under the curve (AUC) = 0.676 (95% CI: 0.559-0.794, P = 0.02), with 64.3% sensitivity and 61.5% specificity. There was no correlation between any of the demographic features, clinical manifestations (apart from serositis, P = 0.013) or disease activity and miR-30e-5p levels. Conclusion: Our study demonstrated elevated miR-30e-5p expression levels in serum sam ples of SLE patients. Apart from serositis, it was not associated with any other disease characteristics.
RESUMEN Antecedentes: Los microARN (miRNA) son ARN no codificantes que regulan la expresión de los genes después de la transcripción. Las pruebas acumuladas indican que la familia de miR-30 participa en el desarrollo de múltiples tejidos y órganos, y es un posible contribuyente a diversas enfermedades, incluidos los trastornos autoinmunes como el lupus eritematoso sistémico (LES). El objetivo de este estudio fue evaluar la expresión del miR-30e-5p, un miembro de la familia miR-30, e investigar su posible relación con las características clínicas y la posible actividad de la enfermedad en una cohorte egipcia de LES. Métodos: Se analizaron muestras de suero de 40 pacientes con LES y 37 sujetos sanos de edad y sexo similares para determinar el nivel de expresión de miR-30e-5p, utilizando la reacción en cadena de la polimerasa de transcripción inversa cuantitativa Taqman. El análisis se llevó a cabo empleando el método 2-AACT. Los resultados: La edad media de los pacientes fue de 28,7 ± 7,9 años, mientras que la duración media de la enfermedad fue de 6,4 ± 5,3 años. La mediana del cambio de pliegue del suero miR-30e-5p entre nuestra cohorte de LES fue significativamente mayor, 1,748 (0,223-20,485), en comparación con el grupo de control, 0,877 (0,058-3,522) (p = 0,02). El análisis de la curva característica de funcionamiento del receptor reveló que el nivel de expresión del miR-30e-5p puede discriminar a los pacientes con LES de los controles en un valor de corte > 1,06, con el área bajo la curva (AUC) = 0,676 (IC del 95%: 0,559-0,794; p = 0,02), una sensibilidad del 64,3% y una especificidad del 61,5%. No hubo asociación entre ninguna de las características demográficas, manifestaciones clínicas (aparte de la serositis, p = 0,013) o actividad de la enfermedad y los niveles de miR-30e-5p. Conclusión: Nuestro estudio demostró niveles elevados de expresión de miR-30e-5p en mues tras de suero de pacientes con LES. Aparte de la serositis, no se asoció con ninguna otra característica de la enfermedad.
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Humanos , Feminino , Adulto , Reação em Cadeia da Polimerase , Doenças da Pele e do Tecido Conjuntivo , Ácidos Nucleicos, Nucleotídeos e Nucleosídeos , Processos Patológicos , Serosite , Condições Patológicas, Sinais e Sintomas , Elementos Antissenso (Genética) , RNA Antissenso , Doenças do Tecido Conjuntivo , MicroRNAs , Lúpus Eritematoso SistêmicoRESUMO
OBJECTIVE: To investigate the characteristics, evolution, and visual outcome of non-infectious uveitis. METHODOLOGY: Records of 201 patients with non-infectious uveitis (136 (67.7%) males and 84 (41.8%) juvenile-onset (≤ 16 years)) were retrospectively reviewed. Data were analyzed through Kruskal-Wallis and Mann-Whitney, chi-square (χ2) tests, and logistic regression. RESULTS: The median disease and follow-up durations were 36 (interquartile range (IQR) 24-70) and 24 (IQR 10-36) months, respectively. Fifty-eight (28.9%) patients had persistently idiopathic uveitis, and 143 (71.1%) were associated with rheumatic diseases, of whom uveitis heralded, coincided with, and succeeded the rheumatic manifestation(s) in 62/143 (43.4%), 37/143 (25.9%), and 44/143 (30.7%) patients, respectively. Established rheumatic diseases were Behçet's disease (103/201 (51.2%)), juvenile idiopathic arthritis (13/201 (6.5%)), sarcoidosis (8/201 (4%)), seronegative spondyloarthropathy (7/201 (3.5%)), and Vogt-Koyanagi-Harada (7/201 (3.5%)), and other diagnoses were present in 5/201 (2.5%) patients. Patients with idiopathic uveitis were characterized by a juvenile-onset (p < 0.001), lower male predominance (p = 0.01), prevalent granulomatous (p < 0.001), and anterior (p = 0.001) uveitis. The median visual acuity at last visit was 0.3 (IQR 0.05-0.6). Visual loss was present in 45/201 (22.3%) patients (36/201 (17.9%) unilateral and 9/201 (4.4%) bilateral). Apart from a longer disease duration (p = 0.002), lower educational level (p = 0.03), and prevalent panuveitis (p < 0.001), visual loss was not associated with any other studied ocular or extra-ocular characteristics. CONCLUSION: Behçet's disease (51.2%) and idiopathic uveitis (28.9%) were the most prevalent causes of non-infectious uveitis in our study. Visual loss (22.3%) was associated with a longer disease duration, lower education level, and prevalent panuveitis. Key Points ⢠Most common causes of uveitis referred to rheumatologists were Behçet's disease and idiopathic uveitis. ⢠Several rheumatic diseases initially presented only with uveitis, more commonly in adult and male patients. ⢠Panuveitis was more frequent among patients with an established rheumatic disease, whereas granulomatous uveitis was uncommon. ⢠Longer disease duration and presence of panuveitis were independently associated with visual loss.
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Artrite Reumatoide , Síndrome de Behçet , Uveíte , Adulto , Síndrome de Behçet/complicações , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/epidemiologia , Egito/epidemiologia , Humanos , Masculino , Estudos Retrospectivos , Uveíte/complicações , Uveíte/diagnóstico , Uveíte/epidemiologiaRESUMO
PURPOSE: To evaluate the choroidal thickness (CT) in the macular area in patients with lupus nephritis and to compare the results with both non-nephritic patients and normal control. To assess the relation of CT to serum microRNA146, disease duration, activity index, and medications. PATIENTS AND METHODS: Thirty-five SLE patients and thirty normal healthy controls were enrolled for this cross-sectional prospective study. All participants have undergone optical coherence tomography using RTVue OCT (Optovue Inc., Fremont, CA, USA). The scan used was the macular cross 6-mm line. We measured CT from the posterior edge of the retinal pigment epithelium (RPE) to the choroid-sclera junction at subfovea, and 750 µm both temporal and nasal to the fovea. RESULTS: The mean central subfoveal CT in patients was 275.7 ± 41.0 µm (214-374 µm), and the mean central subfoveal CT in the control group was 364.5± 23.0 µm (323-411µm). There was a significant thinning at all three points in patients compared to the control group (p<0.001, Mann-Whitney U-test). In the patients group, subfoveal choroid in non-nephritic subgroup showed significant thinning compared to nephritic subgroup (p=0.032, Mann-Whitney U-test). Drusen-like deposits (DLDs) were detected in 22.9% (8/35) of patients and none in control (p=.023). MiRNA146 showed a significant positive correlation with nephritic lupus patients (r=0.036, P=0.04). CONCLUSION: The choroidal thickness was significantly thicker among the nephritic lupus patients as compared to the non-nephritic subgroup. Both SLE patients' subgroups are thinner than normal control. Subfoveal choroidal thickening can be considered a biomarker in nephritic lupus especially in conjunction with an increase in miRNA146a. All SLE patients are at risk of small Drusen-like deposits.
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OBJECTIVES: To study the visual disability predictors in Behçet's disease (BD). METHODS: A cross-sectional observational study including 54 patients. Sociodemographic and cumulative clinical characteristics were obtained. Ophthalmic examination focused on disease activity and degree of visual loss. RESULTS: Presenting features included oral ulcers, orogenital ulcers, uveitis, and vascular involvement. Ocular involvement was also present in a majority of the cases 74.1%. The age at onset had no significant effect on diagnostic time lag (P = .9), unlike rural residency (P = .02). Laterality, ocular activity, and interventions significantly affected final visual acuity (PË0.001). A diagnostic time lag ≥ 9 months significantly affected final visual acuity (P = .039). CONCLUSION: BD is associated with considerable vision loss at a young age. Panuveitis, bilaterality, ocular activity, and interventions are significant predictors. A diagnostic time lag ≥ 9 months is associated with poor visual outcomes and is significantly associated with rural residency.
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Síndrome de Behçet/complicações , Transtornos da Visão/etiologia , Adolescente , Adulto , Auditoria Clínica , Estudos Transversais , Egito , Feminino , Hospitais Universitários , Humanos , Masculino , Pessoa de Meia-Idade , Acuidade VisualRESUMO
INTRODUCTION: The aim of this study was to investigate the characteristics and outcome of systemic lupus erythematosus (SLE) among elderly-onset patients. METHODS: This study included 575 SLE patients managed at Cairo, Alexandria, and Helwan universities from August 2014 to 2018: of whom 49 (8.5%), 420 (73%), and 106 (18.4%) were elderly- (> 50 years), adult- (17-50 years), and juvenile- (≤ 16 years) onset patients, respectively. Cumulative characteristics were recorded. Disease activity at the last visit was investigated through the Systemic Lupus Erythematosus Disease Activity Index-2K (SLEDAI-2K), whereby lupus low disease activity (LLDA) was defined as a SLEDAI-2K score ≤ 4. The disease outcome was assessed through investigating disease damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)) and the prevalence of mortality. Quantitative and categorical data were compared using Kruskal-Wallis and Mann-Whitney tests, and chi-square (χ2) test, respectively. RESULTS: Late-onset SLE (LSLE) patients demonstrated the lowest prevalence of constitutional and mucocutaneous manifestations (p < 0.001), serositis (p = 0.006), nephritis (p < 0.001), neuropsychiatric involvement (p < 0.001), and hypocomplementinemia (p < 0.001), but showed the highest prevalence of comorbidities and multimorbidity (comorbidities ≥ 2) (p < 0.001), and positive anti-ds DNA antibodies (p < 0.001). Elderly-onset patients demonstrated the lowest SLEDAI-2K and SDI scores, achieved LLDA the most (p < 0.001), and developed any damage (SDI ≥ 1) the least (p < 0.001). The prevalence of mortality was comparable across the three age groups (p = 0.6). CONCLUSIONS: Late-onset SLE patients (8.5%) showed the lowest prevalence of major organ involvement and the highest prevalence of comorbidities, and demonstrated more favorable disease activity and damage indices.Key Points⢠The disease characteristics and outcome among LSLE patients are characterized by being controversial, with studies from the Middle East being limited. Our cohort constituted of 8.5% elderly-onset SLE patients-who were characterized by the lowest prevalence of major organ involvement and the lowest activity and damage indices-making the disease pattern more favorable in this age group, despite being characterized by the highest prevalence of comorbidities.
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Lúpus Eritematoso Sistêmico/epidemiologia , Adolescente , Adulto , Idade de Início , Idoso , Criança , Comorbidade , Egito/epidemiologia , Feminino , Glucocorticoides/administração & dosagem , Humanos , Imunossupressores/administração & dosagem , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Índice de Gravidade de Doença , Adulto JovemRESUMO
This perspective article aims to highlight the importance of values-driven personal reflection and collaboration for effective translational medicine training. We frame the dilemma in translational medicine and provide an approach for solution emphasizing collaboration and co-creation for innovative change in translational medicine. We cite the science in transition literature suggesting why personal reflection and a collaborative approach is important. We identify the problem with publication pressures and the bibliometric mindset. We focus on motivation to seek and find results that really matter for patients and individuals to maintain health in the real world. We review how the international EUREKA Institute for Translational Medicine (established in 2007) works with students, to harness their core values and develop personal growth skills to improve their leadership effectiveness, to work toward collaborative gain and potentially more meaningful results for patients and medical needs. We describe how the EUREKA Institute's unique setting, curriculum and hidden curriculum aspects effectively train program participants. The article highlights creating an immersive safe space, personal reflection, connection, structured brainstorming, group problem solving, collaboration and co-creation to facilitate innovation in translational medicine. The article relates program features to their theoretical underpinnings such as Theory U, Mediation Theory and Strategic Innovation Theory. The six authors from different global regions, ages, career stages, translational medicine contexts and years of attendance at the EUREKA Institute provide their reflections on training impact. Lessons learned and recommendations for research and application are discussed.
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PURPOSE: To describe the optical coherence tomography angiography findings in nonocular Behçet disease. METHODS: The superficial capillary plexus (SCP) and deep capillary plexus (DCP) and outer retinal and choroidal flow were evaluated using optical coherence tomography angiography. Perimetry was performed to correlate any microvascular and functional changes. RESULTS: Capillary nonperfusion areas were found in the superficial capillary plexus in 16/20 eyes (80%) and in the DCP in 17/20 eyes (85%). Perifoveal capillary arcade disruption and vessel rarefaction were present in both plexuses in all cases. Capillary telangiectasia was present in the superficial capillary plexus in five eyes (25%) and in the DCP in all eyes. Telangiectasia of the parafoveal capillaries was present in the DCP in all eyes. The mean area of the foveal avascular zone was not significantly different from that in 20 normal eyes (P = 0.68). However, mean and central subfield capillary density values were significantly lower (P < 0.001 and P < 0.005, respectively) in the Behçet disease group. Perimetry revealed central scotomata on the pattern deviation plot in 12 eyes (60%). CONCLUSION: Telangiectasia of the parafoveal capillaries was detected in the DCP in all cases. Microvascular changes in the superficial capillary plexus and DCP in nonocular Behçet disease can be detected by optical coherence tomography angiography.
Assuntos
Síndrome de Behçet/diagnóstico , Corioide/irrigação sanguínea , Artérias Ciliares/patologia , Vasos Retinianos/patologia , Adulto , Síndrome de Behçet/fisiopatologia , Capilares/patologia , Estudos Transversais , Feminino , Angiofluoresceinografia , Humanos , Masculino , Pessoa de Meia-Idade , Tomografia de Coerência Óptica , Testes de Campo Visual , Adulto JovemRESUMO
INTRODUCTION/OBJECTIVES: Despite its importance, adherence to treatment has not been sufficiently studied in Behçet's disease (BD). The aim of this study was to evaluate medication adherence in BD using the Compliance Questionnaire of Rheumatology (CQR) and factors potentially affecting it. METHOD: This cross-sectional study included 67 consecutive BD patients including 57 (85%) males with a mean age of 35.1 ± 9.27 years and mean disease duration of 129 ± 91 months. The cumulative clinical manifestations, the Behçet's Disease Current Activity Form (BDCAF) score, and the Vasculitis Damage Index (VDI) were recorded. The CQR, Socioeconomic Status Questionnaire for Health Research in Egypt (SES), the Beliefs about Medication Questionnaire (BMQ), and the Short Form 36 (SF-36) quality of life assessment questionnaire were administered to the patients. Linear regression analysis was done to determine independent predictors of CQR. RESULTS: The mean BDCAF score was 3.27 ± 3.54 and the VDI was 3.36 ± 2.21. The mean CQR score was 69.2 ± 11.79. The CQR score varied significantly among different health sources (p = 0.02), with no relationship detected with other sociodemographic characteristics, nor with clinical characteristics or the SF-36. Among the investigated medications' complexities, severity of side effects showed significantly different CQR scores (p = 0.004), and a weak positive correlation between medications' numbers and the CQR was detected. Predictors for higher CQR scores included the necessity beliefs score of the BMQ (ß = 1.1, p < 0.001); whereas, predictors for lower CQR scores were the harm and concern BMQ subscales ((ß = - 1.5, p = 0.004) and (ß = - 0.72, p = 0.032), respectively). CONCLUSIONS: Beliefs about medications were the only predictor for adherence in our cohort.