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There is an increasing body of literature suggesting a relationship between environmental factors and the development of systemic sclerosis (SSc). These include occupational exposures, chemical materials, medications, alterations in the microbiome, and dysbiosis. Environmental exposures may impact epigenetic regulation thereby triggering an aberrant immune response resulting in the clinical and serologic phenotype that we diagnose as SSc. Screening and studying putative triggers will not only improve our understanding of the pathogenesis of SSc but also inform the institution for protective measures.
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Disbiosis , Exposición a Riesgos Ambientales , Epigénesis Genética , Esclerodermia Sistémica , Humanos , Exposición a Riesgos Ambientales/efectos adversos , Epigénesis Genética/genética , Epigénesis Genética/inmunología , Exposición Profesional/efectos adversos , Factores de Riesgo , Esclerodermia Sistémica/inducido químicamente , Esclerodermia Sistémica/etiología , Esclerodermia Sistémica/genética , Esclerodermia Sistémica/inmunología , Disbiosis/complicacionesRESUMEN
ABSTRACT: Systemic Lupus erythematosus (SLE) is a chronic multisystem, multifactorial inflammatory autoimmune disease. The SLE patients have 3 times increased risk of mortality based on international data with ethnicity playing an important impact on patients' morbidity and mortality. Descriptive studies from Saudi Arabia showed variation in clinical features from one region to another. Moreover, reliable inference from these studies is limited by study methodology and lack of translational data using biological samples to understand clinical phenotypes of Saudi SLE patients.The aim of this report is to describe the prospective study protocol of the National Systemic Lupus Erythematosus Cohort in Saudi Arabia. The purpose of this cohort study is multifold: first, to examine clinical characteristics and molecular phenotypes of Saudi SLE patients in relation to local environment and practices/lifestyles; second, to assess long-term outcomes of SLE in Saudi population and factors that influence favorable outcomes; third, to compare the effectiveness of various treatment regimens in Saudi SLE population.This study is a longitudinal prospective cohort study of adult, Saudi SLE patients using open cohort study design. Primary outcomes include disease-related outcomes (activity, improvement, and organ damage) and patient-reported outcomes (quality of life). Secondary outcomes include physiological and molecular modifications associated with changes in disease activity states.Results and analysis are in on-going study.This study provides a source of reliable data for clinical and translational research. This will allow us to have a holistic approach to SLE pathogenesis especially in Saudi population and may take us a step further toward much more personalized medicine.This protocol has been registered in NIH ClinicalTrial.gov (ClinicalTrial.gov identifier: NCT04604990) on October 27, 2020.
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Protocolos Clínicos , Lupus Eritematoso Sistémico/psicología , Estudios de Cohortes , Humanos , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/epidemiología , Estudios Prospectivos , Arabia Saudita/epidemiología , Cumplimiento y Adherencia al Tratamiento/psicologíaRESUMEN
BACKGROUND: Anti-neutrophil cytoplasm antibodies-associated vasculitis (AAV) is a rare autoimmune condition with high-relapsing rate and incidence of complications, resulting in increased morbidity and mortality. Characters of patients with anti-neutrophil cytoplasm antibodies-associated vasculitis in Saudi Arabia require further exploration. OBJECTIVE: To evaluate the clinical profile, relapse rate and disease-related complications among patients with AAV at a tertiary hospital in Saudi Arabia. To estimate the role of BVAS score at the time of presentation in predicting relapse during the disease course. DESIGN AND SETTING: This retrospective cohort study was performed through data collection from patients' records who had AAV, who visited the rheumatology clinic. The collected data involved the demographics of patients and their investigations, medications, and outcomes of treatment. Statistical analysis was executed through SPSS version 26. RESULTS: Fifty-two patients were eligible for inclusion, while 48 patients were analyzed because of missing data. Females represented 60.4%. Half of the patients were more than 50 years old, and 68.8% had comorbidities. As for diagnosis, 62.5% had granulomatosis with polyangiitis, 25% had eosinophilic granulomatosis with polyangiitis, and 12.5% had microscopic polyangiitis. The rate of relapse was 31.3%, while the remission rate was 68.8%. Additionally, 66.7% had lower respiratory involvement, and 43.8% had renal involvement. More than half of the patients had BVAS score below 14.5 points. The study did not explore a positive correlation between the disease relapse and high BVAS at the first presentation. CONCLUSION: Early prediction of relapse and such intervention is of paramount importance in order to avoid accrual of organ damage with treatments that prevent further relapses. BVAS score was not found to be a potential predictor in our study. Future studies are highly endorsed, with prospective design and large sample size to achieve statistical significance for the incidence of relapses and complications.
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Filler injection or implantation is a progressing revolutionary subject. Although the widely available kinds in many implications are considered safe, post filler adverse events are not uncommon. These reactions range from mild reactions such as edema or erythema to detrimental reactions such as recurrent infected granuloma or vascular occlusion, which are predominantly related to non-FDA approved materials. Here, we presented a patient with a significant history of gluteal augmentation using unlicensed silicone who developed extensive retroperitoneal fibrosis complicated by deep venous occlusions and obstructive uropathy.
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Rituximab (RTX) is a chimeric B-cell-depleting monoclonal antibody against CD-20 positive cells that has been approved for the induction and maintenance of granulomatosis with polyangiitis (GPA). Reports have identified RTX to cause drug-related psoriasis. Many theories of underlying pathways have been proposed. However, further workup around the mechanism and treatment is required. Here, we present a 38-year-old woman known to have GPA that developed drug-related psoriasis that was successfully treated with treatment discontinuation and starting adalimumab along with a literature review.
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AIM: To study the clinical presentations of Behçet's disease in patients visiting a tertiary hospital in south-western Saudi Arabia. PATIENTS AND METHODS: Forty-seven patients with Behçet's disease attending the Rheumatology Department at Asser Central Hospital, Saudi Arabia were recruited into the study. The study was conducted over a period of 5 years from January 2012 to July 2017. Medical records of the patients were reviewed to analyze the frequency of different clinical manifestations. RESULTS: The study had 26 men and 21 women. The mean age of the patients was 37.11 ± 11.9 years (range <30-60 years). Frequency of main clinical manifestations in these patients included 89.4% (42/47) oral ulcers, 80.9% (38) genital ulcers, 55.3% (26/47) ocular complications, 55.3% arthralgia (26/47), 31.9% arthritis (15/47), 36% neurological complications (17/47), 34% gastrointestinal involvement (16), 17% pulmonary complication (8/47), cutaneous lesions in the form of skin pustules were found in 31.9% of cases and erythema only in 4.3% of patients. Deep venous thrombosis was observed in 66.6% of patients. About 96% of patients showed improvement with drugs. Only 2 patients died during the study period. CONCLUSION: Behçet's disease showed higher male predominance in south-western Saudi Arabia, similar to other Middle-Eastern countries. The clinical characteristics are comparable to different studies reported from other countries. Regarding the care outcome, the majority of patients were cured using oral corticosteroids and other immunosuppressive drugs.