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1.
Lupus ; 32(14): 1610-1618, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37921574

RESUMEN

INTRODUCTION: The utilisation of telemedicine has been rapidly growing among patients with rheumatic diseases, especially following the corona virus disease 2019 pandemic. Ease and convenience appear to dominate the reasons for this growth. However, the effects of this approach in patients with systemic lupus erythematosus (SLE) are yet to be revealed. In this study, we examined the effect of telemedicine on disease activity assessment and damage scores in patients with SLE. METHODS: This case-crossover study was nested within a national prospective cohort of patients with SLE in Saudi Arabia. Patients with SLE were included if they fulfilled the Systemic Lupus International Collaborating Clinics classification criteria between March 2020 and March 2021 and were assessed at three time points with 3 months between assessments, according to the standardised protocol of this cohort. Telemedicine was conducted for the first evaluation, while in-person assessments were used at the second and third visits. The primary outcome was the difference in the SLE disease activity index 2000 (SLEDAI-2K) score. The primary analysis was conducted using the repeated measure model and adjusted for potential confounders, including demographics, medications, and changes in steroid doses. Several sensitivity analyses were conducted to mitigate selection and time-varying confounders. RESULTS: A total of 92 participants were included in this study. Most patients were females (88%), with a mean (±standard deviation [SD]) age of 36 (±13) years. The mean (±SD) disease activity scores at baseline were as follows: SLEDAI-2K, 5 (±5); SLE responder index, 3.8 (±3.5); Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index, 1 (±1). The mean difference in SLEDAI-2K score was -1.641 (95% confidence interval -2.773 to -0.510, p = 0.005*) between telemedicine and follow-up visits. The results were consistent in all sensitivity analyses. CONCLUSION: We found that telemedicine assessment was associated with a much higher disease activity score than subsequent assessments, which may suggest an overestimation of disease activity and later assessment accuracy. Cautious adoption has been suggested for SLE patients with active disease.


Asunto(s)
Lupus Eritematoso Sistémico , Femenino , Humanos , Adulto Joven , Adulto , Persona de Mediana Edad , Masculino , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Estudios Cruzados , Estudios Prospectivos , Arabia Saudita/epidemiología , Índice de Severidad de la Enfermedad
2.
J Clin Apher ; 38(5): 573-581, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37317696

RESUMEN

BACKGROUND: The improvement in the clinical care for patients with thrombotic thrombocytopenic purpura (TTP) is evolving, and many efforts are being put to standardize it. Here, we aimed to assess the provided care at a national level and identify deficiencies. METHODS: A national Saudi retrospective descriptive study was carried out at six tertiary referral centers and included all patients who underwent therapeutic plasma exchange (TPE) for the diagnosis of TTP between May 2005, and July 2022. Collected information included demographic data, clinical features on presentation, and the results of laboratory investigations at admission and discharge. In addition, the number of TPE sessions, days till the first session of TPE, usage of immunological agents, and clinical outcomes were all collected. RESULTS: One hundred patients were enrolled, predominantly female (56%). The mean age was 36.8 years. At diagnosis, 53% of patients showed neurological involvement. The mean platelet count at presentation was 21 × 109 /L. All patients had anemia (mean hematocrit 24.2%). Schistocytes were present in the peripheral blood film of all patients. The mean number of TPE rounds was 13 ± 9.3, and the mean days to start TPE since admission for the first episode was 2.5 days. ADAMTS13 level was measured in 48% of patients and was significantly low in 77% of them. Assessing for clinical TTP scores, 83%, 1000%, 64% of eligible patients had an intermediate/high PLASMIC, FRENCH, and Bentley scores, respectively. Caplacizumab was used on only one patient, and rituximab was administered to 37% of patients. A complete response for the first episode was achieved in 78% of patients. The overall mortality rate was 25%. Neither time to TPE, the use of rituximab or steroid affected survival. CONCLUSIONS: Our study shows an excellent response to TPE with a survival rate approximate to the reported international literature. We observed a deficiency in using validated scoring systems in addition to confirming the disease by ADAMTS13 testing. This emphasizes the need for a national registry to facilitate proper diagnosis and management of this rare disorder.


Asunto(s)
Intercambio Plasmático , Púrpura Trombocitopénica Trombótica , Humanos , Femenino , Adulto , Masculino , Intercambio Plasmático/métodos , Púrpura Trombocitopénica Trombótica/diagnóstico , Rituximab/uso terapéutico , Estudios Retrospectivos , Arabia Saudita , Proteína ADAMTS13 , Sistema de Registros
3.
Lupus Sci Med ; 11(1)2024 May 24.
Artículo en Inglés | MEDLINE | ID: mdl-38789277

RESUMEN

OBJECTIVE: This study examined the prevalence of major adverse cardiovascular events (MACE) among Saudi patients with SLE and the general population and considered factors associated with such outcomes were taken into consideration. METHODS: This is a cohort study evaluating the period prevalence of MACE from 2020 to 2023. The study used two datasets, namely the Saudi national prospective cohort for SLE patients and the Prospective Urban-Rural Epidemiology Study Saudi subcohort (PURE-Saudi) for the general population. Participants in both studies were monitored using a standardised protocol. MACE was defined as myocardial infarction (MI), stroke or angina. The analysis was adjusted for demographics, traditional cardiovascular risk factors and SLE diagnosis through logistic regression models. RESULTS: The PURE and national SLE cohorts comprised 488 and 746 patients, respectively. Patients with SLE from the SLE cohort were younger (40.7±12.5 vs 49.5±8.6 years) and predominantly female (90.6% vs 41.6%). The prevalence of traditional risk factors was greater in the PURE cohort compared with the SLE cohort. These factors included dyslipidaemia (28.9% vs 49.4%), obesity (63% vs 85%) and diabetes (7.8% vs 27.2%), but not hypertension (19.3% vs 18.8%). MACE (defined as MI or stroke or venous thromboembolism or heart failure) occurred more frequently in patients with SLE (4.3% vs 1.6%, p=0.004). Older age and lupus diagnosis were independently associated with MACE after adjusting for conventional risk factors. The odds of MACE were significantly related to age and lupus diagnosis (p=0.00 and p=0.00, respectively), but not cardiovascular disease (CVD) risk factors (p=0.83). CONCLUSION: Patients with SLE have a significantly higher risk of developing MACE than the general population. This risk is not well explained by traditional risk factors, which may explain the failure of CVD risk scores to stratify patients with SLE adequately. Further studies are needed to understand CVD risk's pathogenesis in SLE and mitigate it.


Asunto(s)
Enfermedades Cardiovasculares , Lupus Eritematoso Sistémico , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/epidemiología , Femenino , Masculino , Arabia Saudita/epidemiología , Persona de Mediana Edad , Adulto , Prevalencia , Estudios Prospectivos , Enfermedades Cardiovasculares/epidemiología , Factores de Riesgo , Infarto del Miocardio/epidemiología , Accidente Cerebrovascular/epidemiología , Accidente Cerebrovascular/etiología , Dislipidemias/epidemiología , Obesidad/epidemiología , Obesidad/complicaciones , Estudios de Cohortes
4.
Arthritis Rheumatol ; 76(7): 1141-1152, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38499989

RESUMEN

OBJECTIVE: We aimed to evaluate the clinical usefulness of the systemic score in the prediction of life-threatening evolution in Still disease. We also aimed to assess the clinical relevance of each component of the systemic score in predicting life-threatening evolution and to derive patient subsets accordingly. METHODS: A multicenter, observational, prospective study was designed including patients included in the Gruppo Italiano Di Ricerca in Reumatologia Clinica e Sperimentale Adult-Onset Still Disease Study Group and the Autoinflammatory Disease Alliance Network Still Disease Registry. Patients were assessed to see if the variables to derive the systemic score were available. The life-threatening evolution was defined as mortality, whatever the clinical course, and/or macrophage activation syndrome, a secondary hemophagocytic lymphohistiocytosis associated with a poor prognosis. RESULTS: A total of 597 patients with Still disease were assessed (mean ± SD age 36.6 ± 17.3 years; male 44.4%). The systemic score, assessed as a continuous variable, significantly predicted the life-threatening evolution (odds ratio [OR] 1.24; 95% confidence interval [CI] 1.07-1.42; P = 0.004). A systemic score ≥7 also significantly predicted the likelihood of a patient experiencing life-threatening evolution (OR 3.36; 95% CI 1.81-6.25; P < 0.001). Assessing the clinical relevance of each component of the systemic score, liver involvement (OR 1.68; 95% CI 1.48-2.67; P = 0.031) and lung disease (OR 2.12; 95% CI 1.14-4.49; P = 0.042) both significantly predicted life-threatening evolution. The clinical characteristics of patients with liver involvement and lung disease were derived, highlighting their relevance in multiorgan disease manifestations. CONCLUSION: The clinical utility of the systemic score was shown in identifying Still disease at a higher risk of life-threatening evolution in a large cohort. Furthermore, the clinical relevance of liver involvement and lung disease was highlighted.


Asunto(s)
Sistema de Registros , Enfermedad de Still del Adulto , Humanos , Masculino , Femenino , Adulto , Estudios Prospectivos , Persona de Mediana Edad , Enfermedad de Still del Adulto/diagnóstico , Enfermedad de Still del Adulto/complicaciones , Adulto Joven , Índice de Severidad de la Enfermedad , Pronóstico , Progresión de la Enfermedad , Síndrome de Activación Macrofágica/diagnóstico
5.
Ocul Immunol Inflamm ; : 1-8, 2024 May 17.
Artículo en Inglés | MEDLINE | ID: mdl-38759215

RESUMEN

PURPOSE: The clinical relevance of human leukocyte antigen (HLA) subtypes such as HLA-B51 on Behçet's disease (BD)-related uveitis and non-infectious uveitis (NIU) unrelated to BD remains largely unknown. METHODS: Data were prospectively collected from the International AIDA Network Registry for BD and for NIU. We assessed differences between groups (NIU unrelated to BD and positive for HLA-B51, BD-related uveitis positive for HLA-B51 and BD-related uveitis negative for HLA-B51) in terms of long-term ocular complications, visual acuity (VA) measured by best corrected visual acuity (BCVA), anatomical pattern, occurrence of retinal vasculitis (RV) and macular edema over time. RESULTS: Records of 213 patients (341 eyes) were analyzed. No differences in complications were observed (p = 0.465). With regard to VA, a significant difference was detected in median BCVA (p = 0.046), which was not maintained after Bonferroni correction (p = 0.060). RV was significantly more prevalent in NIU-affected patients who tested positive for HLA-B51, irrespective of the systemic diagnosis of BD (p = 0.025). No differences emerged in the occurrence of macular edema (p = 0.99). CONCLUSIONS: Patients with NIU testing positive for HLA-B51 exhibit an increased likelihood of RV throughout disease course, irrespective of a systemic diagnosis of BD. The rate of complications as well as VA are comparable between NIU cases unrelated to BD testing positive for HLA-B51 and uveitis associated with BD. Therefore, it is advisable to perform the HLA-B typing in patients with NIU or retinal vasculitis, even in the absence of typical BD features.

6.
Intern Emerg Med ; 18(3): 743-754, 2023 04.
Artículo en Inglés | MEDLINE | ID: mdl-36881285

RESUMEN

This study aims to describe musculoskeletal manifestations (MSM) in children with Behçet's syndrome (BS), their association with other disease manifestations, response to therapy, and long-term prognosis. Data were retrieved from the AIDA Network Behçet's Syndrome Registry. Out of a total of 141 patients with juvenile BS, 37 had MSM at disease onset (26.2%). The median age at onset was 10.0 years (IQR 7.7). The median follow-up duration was 21.8 years (IQR 23.3). Recurrent oral (100%) and genital ulcers (67.6%) and pseudofolliculitis (56.8%) were the most common symptoms associated with MSM. At disease onset, 31 subjects had arthritis (83.8%), 33 arthralgia (89.2%), and 14 myalgia (37.8%). Arthritis was monoarticular in 9/31 cases (29%), oligoarticular in 10 (32.3%), polyarticular in 5 (16.1%), axial in 7 (22.6%). Over time, arthritis became chronic-recurrent in 67.7% of cases and 7/31 patients had joint erosions (22.6%). The median Behçet's Syndrome Overall Damage Index was 0 (range 0-4). Colchicine was inefficacious for MSM in 4/14 cases (28.6%), independently from the type of MSM (p = 0.46) or the concomitant therapy (p = 0.30 for cDMARDs, p = 1.00 for glucocorticoids); cDMARDs and bDMARDs were inefficacious for MSM in 6/19 (31.4%) and 5/12 (41.7%) cases. The presence of myalgia was associated with bDMARDs inefficacy (p = 0.014). To conclude, MSM in children with BS are frequently associated with recurrent ulcers and pseudofolliculitis. Arthritis is mostly mono- or oligoarticular, but sacroiliitis is not unusual. Prognosis of this subset of BS is overall favorable, though the presence of myalgia negatively affects response to biologic therapies. ClinicalTrials.gov Identifier: NCT05200715 (registered on December 18, 2021).


Asunto(s)
Artritis , Síndrome de Behçet , Niño , Humanos , Artritis/complicaciones , Síndrome de Behçet/complicaciones , Síndrome de Behçet/epidemiología , Síndrome de Behçet/diagnóstico , Mialgia , Sistema de Registros , Úlcera/complicaciones
7.
Semin Arthritis Rheum ; 62: 152244, 2023 10.
Artículo en Inglés | MEDLINE | ID: mdl-37517110

RESUMEN

OBJECTIVES: To describe clinical characteristics of patients with Still's disease treated with methotrexate (MTX) and to assess drug effectiveness evaluating change in disease activity, reduction of inflammatory markers, and glucocorticoid (GC)-sparing effect. METHODS: Patients with Still's disease treated with MTX were assessed among those included in AIDA Network Still Disease Registry. RESULTS: In this registry, 171 patients with Still's disease were treated with MTX (males 43.3%, age 37.1 ± 16.0 years). They were mainly characterised by joint features and fever without a prominent multiorgan involvement. MTX was administered with GCs in 68.4% of patients, with other conventional synthetic DMARDs in 6.4%, and with biologic DMARDs in 25.1%. A significant reduction of the modified systemic score was observed, and 38.6% patients were codified as being in clinical remission at the end of follow-up. The concomitant administration of a biologic DMARD resulted a predictor of the clinical remission. Furthermore, a reduction of inflammatory markers and ferritin levels was observed following the administration of MTX. Additionally, a marked reduction of the dosage of concomitant GCs was identified, while 36.7% discontinued such drugs. Male gender appeared as a predictor of GC discontinuation. MTX was discontinued in 12.3% of patients because of adverse effects, and in 12.3% for lack of efficacy. CONCLUSIONS: Clinical characteristics of patients with Still's disease treated with MTX were described, mainly joint features and fever without a prominent multiorgan involvement. The clinical usefulness of MTX was reported in reducing the disease activity, decreasing the inflammatory markers, and as GC-sparing agent.


Asunto(s)
Antirreumáticos , Artritis Juvenil , Productos Biológicos , Enfermedad de Still del Adulto , Humanos , Masculino , Adulto Joven , Adulto , Persona de Mediana Edad , Metotrexato/uso terapéutico , Artritis Juvenil/tratamiento farmacológico , Antirreumáticos/uso terapéutico , Glucocorticoides/uso terapéutico , Sistema de Registros , Fiebre , Productos Biológicos/uso terapéutico , Enfermedad de Still del Adulto/tratamiento farmacológico
8.
Intern Emerg Med ; 18(8): 2231-2243, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37828268

RESUMEN

To characterize clinical and laboratory signs of patients with Still's disease experiencing macrophage activation syndrome (MAS) and identify factors associated with MAS development. Patients with Still's disease classified according to internationally accepted criteria were enrolled in the AutoInflammatory Disease Alliance (AIDA) Still's Disease Registry. Clinical and laboratory features observed during the inflammatory attack complicated by MAS were included in univariate and multivariate logistic regression analysis to identify factors associated to MAS development. A total of 414 patients with Still's disease were included; 39 (9.4%) of them developed MAS during clinical history. At univariate analyses, the following variables were significantly associated with MAS: classification of arthritis based on the number of joints involved (p = 0.003), liver involvement (p = 0.04), hepatomegaly (p = 0.02), hepatic failure (p = 0.01), axillary lymphadenopathy (p = 0.04), pneumonia (p = 0.03), acute respiratory distress syndrome (p < 0.001), platelet abnormalities (p < 0.001), high serum ferritin levels (p = 0.009), abnormal liver function tests (p = 0.009), hypoalbuminemia (p = 0.002), increased LDH (p = 0.001), and LDH serum levels (p < 0.001). At multivariate analysis, hepatomegaly (OR 8.7, 95% CI 1.9-52.6, p = 0.007) and monoarthritis (OR 15.8, 95% CI 2.9-97.1, p = 0.001), were directly associated with MAS, while the decade of life at Still's disease onset (OR 0.6, 95% CI 0.4-0.9, p = 0.045), a normal platelet count (OR 0.1, 95% CI 0.01-0.8, p = 0.034) or thrombocytosis (OR 0.01, 95% CI 0.0-0.2, p = 0.008) resulted to be protective. Clinical and laboratory factors associated with MAS development have been identified in a large cohort of patients based on real-life data.


Asunto(s)
Hepatopatías , Síndrome de Activación Macrofágica , Enfermedad de Still del Adulto , Humanos , Síndrome de Activación Macrofágica/diagnóstico , Síndrome de Activación Macrofágica/complicaciones , Hepatomegalia/complicaciones , Enfermedad de Still del Adulto/complicaciones , Enfermedad de Still del Adulto/diagnóstico , Hepatopatías/complicaciones
9.
RMD Open ; 9(4)2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37989322

RESUMEN

BACKGROUND: Different patient clusters were preliminarily suggested to dissect the clinical heterogeneity in Still's disease. Thus, we aimed at deriving and validating disease clusters in a multicentre, observational, prospective study to stratify these patients. METHODS: Patients included in GIRRCS AOSD-study group and AIDA Network Still Disease Registry were assessed if variables for cluster analysis were available (age, systemic score, erythrocyte sedimentation rate (ESR), C reactive protein (CRP) and ferritin). K-means algorithm with Euclidean metric and Elbow plot were used to derive an adequate number of clusters. RESULTS: K-means clustering assessment provided four clusters based on means standardised according to z-scores on 349 patients. All clusters mainly presented fever, skin rash and joint involvement. Cluster 1 was composed by 115 patients distinguished by lower values of age and characterised by skin rash myalgia, sore throat and splenomegaly. Cluster 2 included 128 patients identified by lower levels of ESR, ferritin and systemic score; multiorgan manifestations were less frequently observed. Cluster 3 comprised 31 patients categorised by higher levels of CRP and ferritin, they were characterised by fever and joint involvement. Cluster 4 contained 75 patients derived by higher values of age and systemic score. Myalgia, sore throat, liver involvement and life-threatening complications, leading to a high mortality rate, were observed in these patients. CONCLUSIONS: Four patient clusters in Still's disease may be recognised by a multidimensional characterisation ('Juvenile/Transitional', 'Uncomplicated', 'Hyperferritinemic' and 'Catastrophic'). Of interest, cluster 4 was burdened by an increased rate of life-threatening complications and mortality, suggesting a more severe patient group.


Asunto(s)
Artritis Juvenil , Exantema , Faringitis , Enfermedad de Still del Adulto , Humanos , Artritis Juvenil/complicaciones , Proteína C-Reactiva/metabolismo , Exantema/complicaciones , Ferritinas , Fiebre , Mialgia/complicaciones , Faringitis/complicaciones , Estudios Prospectivos , Enfermedad de Still del Adulto/complicaciones , Enfermedad de Still del Adulto/diagnóstico , Enfermedad de Still del Adulto/epidemiología
10.
RMD Open ; 9(4)2023 12 01.
Artículo en Inglés | MEDLINE | ID: mdl-38053457

RESUMEN

OBJECTIVE: Still's disease is more frequently observed in the paediatric context, but a delayed onset is not exceptional both in the adulthood and in the elderly. However, whether paediatric-onset, adult-onset and elderly-onset Still's disease represent expressions of the same disease continuum or different clinical entities is still a matter of controversy. The aim of this study is to search for any differences in demographic, clinical features and response to treatment between pediatric-onset, adult-onset and elderly-onset Still's disease. METHODS: Subjects included in this study were drawn from the International AutoInflammatory Disease Alliance Network registry for patients with Still's disease. RESULTS: A total of 411 patients suffering from Still's disease were enrolled; the disease occurred in the childhood in 65 (15.8%) patients, in the adult 314 (76.4%) patients and in the elderly in 32 (7.8%) patients. No statistically significant differences at post-hoc analysis were observed in demographic features of the disease between pediatric-onset, adult-onset and elderly-onset Still's disease. The salmon-coloured skin rash (p=0.004), arthritis (p=0.009) and abdominal pain (p=0.007) resulted significantly more frequent among paediatric patients than in adult cases, while pleuritis (p=0.015) and arthralgia (p<0.0001) were significantly more frequent among elderly-onset patients compared with paediatric-onset subjects. Regarding laboratory data, thrombocytosis was significantly more frequent among paediatric patients onset compared with adult-onset subjects (p<0.0001), while thrombocytopenia was more frequent among elderly-onset patients although statistical significance was only bordered. No substantial differences were observed in the response to treatments. CONCLUSIONS: Despite some minor difference between groups, overall, demographic, clinical, laboratory and treatments aspects of Still's disease were similarly observed in patients at all ages. This supports that pediatric-onset, adult-onset and elderly-onset Still's disease is the same clinical condition arising in different ages.


Asunto(s)
Artritis Juvenil , Enfermedad de Still del Adulto , Adulto , Humanos , Niño , Anciano , Enfermedad de Still del Adulto/diagnóstico , Enfermedad de Still del Adulto/epidemiología , Enfermedad de Still del Adulto/tratamiento farmacológico , Artralgia
11.
Intern Emerg Med ; 17(7): 1977-1986, 2022 10.
Artículo en Inglés | MEDLINE | ID: mdl-35831701

RESUMEN

Purpose of the present paper is to point out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients with Behçet's disease (BD). The Registry is a clinical physician-driven non-population- and electronic-based instrument implemented for the retrospective and prospective collection of real-life data about demographics, clinical, therapeutic, laboratory, instrumental and socioeconomic information from BD patients; the Registry is based on the Research Electronic Data Capture (REDCap) tool, which is thought to collect standardised information for clinical real-life research, and has been realised to change over time according to future scientific acquisitions and potentially communicate with other existing and future Registries dedicated to BD. Starting from January 31st, 2021, to February 7th, 2022, 110 centres from 23 countries in 4 continents have been involved. Fifty-four of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 175 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 5993 fields organised into 16 instruments, including patient's demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. The development of the AIDA International Registry for BD patients will facilitate the collection of standardised data leading to real-world evidence, enabling international multicentre collaborative research through data sharing, international consultation, dissemination of knowledge, inclusion of patients and families, and ultimately optimisation of scientific efforts and implementation of standardised care.Trial registration NCT05200715 in 21/01/2022.


Asunto(s)
Síndrome de Behçet , Adulto , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/epidemiología , Síndrome de Behçet/terapia , Niño , Humanos , Estudios Prospectivos , Sistema de Registros , Estudios Retrospectivos
13.
Neurosciences (Riyadh) ; 13(3): 291-5, 2008 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-21063342

RESUMEN

OBJECTIVES: To describe the pattern of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and evaluate its local diagnostic and management practices. To define factors responsible for the delay in reaching a diagnosis and initiating treatment. METHODS: Patients with the diagnosis of CIDP attending King Khalid University Hospital, Riyadh, Saudi Arabia between 1986 and 2006 were retrospectively studied, in relation to diagnosis and management. Diagnosis was reassessed, and patients included in view of American Academy of Neurology as well as Latov's criteria. Available patients were reevaluated and prospectively followed up until the end of the study. RESULTS: Twenty-two patients were included (18 males and 4 females, 4.5:1). Age at onset range was 3-70 years (mean of 33 years). Diagnosis in 80% of patients was delayed from 6 months to 10 years (mean of 2.5 years). No case was diagnosed before referral. The course was progressive in 53% and relapsing in 47%. Most patients made significant initial improvement, though less than expected. Two patients with long delay in diagnosis (7.5 and 10 years) showed no improvement. CONCLUSION: Diagnosis of CIDP is frequently delayed, with a deleterious effect on response to treatment. This is related to some degree to the lack of awareness among general physicians, which needs to be corrected. Treatment was also hindered by patients` suboptimal compliance, which could be improved by better education. Management is not standardized, and this could be improved by establishing up-to-date treatment guidelines.

14.
Int J Cardiol ; 235: 94-99, 2017 May 15.
Artículo en Inglés | MEDLINE | ID: mdl-28284505

RESUMEN

BACKGROUND: Several registries have described patients hospitalized with heart failure (HF), but only few looked at outpatients in the ambulatory setting mostly without long-term follow-up. We sought to determine the clinical characteristics, management, and 1-year outcomes of patients with chronic HF in Saudi Arabia. METHODS: Part of a prospective multicenter nationwide registry; HEart function Assessment Registry Trial in Saudi Arabia (HEARTS) and included chronic HF patients referred to four HFCs between September 2009 and December 2011. RESULTS: We enrolled 685 patients with mean age 55.66±15.97years, 70.1% were men and 96.1% were Saudis. The main etiologies of HF were CAD (38.8%), dilated cardiomyopathy (36.5%), and hypertension (10.5%). Severe left ventricular dysfunction was present in 70.6% and median NT-proBNP was 2934.37pg/ml. The prescription rates of evidence based therapies (EBTs) before admission to HFC, at discharge from 1st clinic visit, and at 1-year follow up were 90%, 91% and 94% for beta-blockers, 79%, 80%, and 86% for ACEi/ARBs and 44%, 45%, and 42% for aldosterone antagonists; respectively. ICD was inserted in 21.9% and CRT in 6.6% at enrollment and increased to 29.1% and 8.8% after one year respectively. The all-cause mortality rate at 1year was 9% and 93.7% of which was cardiac-related. The all-cause one-year hospitalization rate was 39% and the total emergency room visit rate was 50%. CONCLUSIONS: Chronic HF patients in Saudi Arabia are younger, commonly have severe LV systolic dysfunction and have relatively high annual mortality and re-hospitalization rates.


Asunto(s)
Inhibidores de la Enzima Convertidora de Angiotensina/uso terapéutico , Cardiomiopatía Dilatada/complicaciones , Insuficiencia Cardíaca , Hipertensión/complicaciones , Antagonistas de Receptores de Mineralocorticoides/uso terapéutico , Antagonistas Adrenérgicos beta/uso terapéutico , Adulto , Anciano , Enfermedad Crónica , Femenino , Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/fisiopatología , Insuficiencia Cardíaca/terapia , Hospitalización/estadística & datos numéricos , Humanos , Masculino , Persona de Mediana Edad , Mortalidad , Péptido Natriurético Encefálico/análisis , Evaluación de Procesos y Resultados en Atención de Salud , Fragmentos de Péptidos/análisis , Sistema de Registros/estadística & datos numéricos , Arabia Saudita/epidemiología , Índice de Severidad de la Enfermedad , Evaluación de Síntomas/métodos , Evaluación de Síntomas/estadística & datos numéricos , Disfunción Ventricular Izquierda/diagnóstico , Disfunción Ventricular Izquierda/etiología
15.
PLoS One ; 11(1): e0147385, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-26807577

RESUMEN

BACKGROUND: Little is known about Emergency Medical Services (EMS) use and pre-hospital triage of patients with acute ST-elevation myocardial infarction (STEMI) in Arabian Gulf countries. METHODS: Clinical arrival and acute care within 24 h of STEMI symptom onset were compared between patients transferred by EMS (Red Crescent and Inter-Hospital) and those transferred by non-EMS means. Data were retrieved from a prospective registry of 36 hospitals in 6 Arabian Gulf countries, from January 2014 to January 2015. RESULTS: We enrolled 2,928 patients; mean age, 52.7 (SD ±11.8) years; 90% men; and 61.7% non-Arabian Gulf citizens. Only 753 patients (25.7%) used EMS; which was mostly via Inter-Hospital EMS (22%) rather than direct transfer from the scene to the hospital by the Red Crescent (3.7%). Compared to the non-EMS group, the EMS group was more likely to arrive initially at a primary or secondary health care facility; thus, they had longer median symptom-onset-to-emergency department arrival times (218 vs. 158 min; p˂.001); they were more likely to receive primary percutaneous coronary interventions (62% vs. 40.5%, p = 0.02); they had shorter door-to-needle times (38 vs. 42 min; p = .04); and shorter door-to-balloon times (47 vs. 83 min; p˂.001). High EMS use was independently predicted mostly by primary/secondary school educational levels and low or moderate socioeconomic status. Low EMS use was predicted by a history of angina and history of percutaneous coronary intervention. The groups had similar in-hospital deaths and outcomes. CONCLUSION: Most acute STEMI patients in the Arabian Gulf region did not use EMS services. Improving Red Crescent infrastructure, establishing integrated STEMI networks, and launching educational public campaigns are top health care system priorities.


Asunto(s)
Servicios Médicos de Urgencia/estadística & datos numéricos , Infarto del Miocardio , Adulto , Anciano , Angina de Pecho/tratamiento farmacológico , Angina de Pecho/epidemiología , Angioplastia Coronaria con Balón/estadística & datos numéricos , Fármacos Cardiovasculares/uso terapéutico , Escolaridad , Electrocardiografía , Servicio de Urgencia en Hospital/estadística & datos numéricos , Emigrantes e Inmigrantes , Femenino , Mortalidad Hospitalaria , Humanos , Masculino , Persona de Mediana Edad , Medio Oriente/epidemiología , Infarto del Miocardio/epidemiología , Infarto del Miocardio/cirugía , Infarto del Miocardio/terapia , Intervención Coronaria Percutánea/estadística & datos numéricos , Estudios Prospectivos , Sistema de Registros , Factores Socioeconómicos , Factores de Tiempo , Resultado del Tratamiento , Triaje
16.
Neurosciences (Riyadh) ; 9(4): 281-6, 2004 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-23377249

RESUMEN

OBJECTIVE: To show prospectively the impact of establishing a consultation-liaison (C/L) psychiatry model on the consultation clinical indices. METHODS: A structured, organized model of C/L psychiatry was established in 1990 at King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia and all clinical indices of the psychiatric consultations were compared prospectively over the first 3 years after January 1990 with those over the previous 2 years retrospectively before January 1990. RESULTS: Three hundred and two patients were referred over 3 years after January 1990, group A and 99 patients were referred over the 2 years before January 1990, group B and the male to female ratio were similar in both groups 1:1.99 and 1:2.1. Referred cases were found more to be females, married and living in urban areas and referred from the Department of Medicine. Positive influences of establishing the C/L service were; nearly double rate of referral, more selectivity of cases, more referred cases for assessment of competence and with past psychiatric history, and more patients referred with informed consent. Depressive disorder was more common in both groups. CONCLUSION: A well structured C/L psychiatry model, with clearly defined aims, has had a definite positive impact on the clinical indices of the psychiatric consultations promoting close cooperation for the excellence of patient care. This opens room for future research to explore specific developments in proposed models of C/L psychiatry.

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