RESUMO
INTRODUCTION: Timely diagnosis and proper recognition of Systemic Lupus Erythematosus (SLE) is essential to establish early management in inpatients and outpatients. There are different classification scales to identify SLE, which include various clinical and serological aspects. In 2021, the SLE Risk Probability Index (SLERPI) was published, which focuses predominantly on the clinical characteristics of patients with suspected SLE and uses a simple algorithm for early recognition of the disease. The aim of this study is to compare the European League Against Rheumatism/American College of Rheumatology (ACR/EULAR) classification criteria, the Systemic Lupus International Collaborating Clinics (SLICC) criteria, and the SLERPI criteria in a cohort of Colombian patients with SLE and to analyze the correlations observed between their absolute scores. METHODS: A registry of SLE patients from two referral hospitals in Bogotá, Colombia, was used. 2021 SLERPI, 2019 ACR/EULAR, and 2012 SLICC scores were calculated for each patient and the correlations found between the scales were analyzed. The sensitivities of each were compared, and frequency analyses were conducted among different clinical and laboratory variables. RESULTS: Between 2016 and 2019, 146 patients diagnosed with SLE were registered, including inpatients and outpatients. The median age was 36 years (interquartile range 26-51), and 82.2% were women. According to the SLERPI criteria, a high prevalence of antinuclear antibodies (92%), immunological disorders (71%), and arthritis (64%) were observed. The most used treatments were corticosteroids (87.6%) and chloroquine (67.8%). A Spearman evaluation analysis was performed, with a moderately strong correlation of 0.76 (p = .000) between the SLERPI and ACR/EULAR scales and very strong correlation of 0.80 (p = .000) between the SLERPI and SLICC. Patients classified with SLE according to the SLERPI scale exhibited a higher incidence of hematological compromise, along with elevated levels of serological markers such as anti-DNA antibodies. Additionally, this group more commonly received treatments involving corticosteroids and azathioprine, and displayed a higher prevalence of hypertension. CONCLUSION: The SLERPI scale could be useful in the diagnosis of SLE, especially in early stages, given its good correlation with other classification scales and its good sensitivity.
Assuntos
Lúpus Eritematoso Sistêmico , Doenças Reumáticas , Reumatologia , Humanos , Feminino , Estados Unidos , Adulto , Masculino , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/epidemiologia , Anticorpos Antinucleares , CorticosteroidesRESUMO
Bone marrow fibrosis is a rare disorder that can be associated with autoimmune diseases such as systemic lupus erythematosus (SLE), and can be confused with the manifestations of that disease. The case is presented on a patient with autoimmune myelofibrosis in the context of SLE.
La fibrosis de la médula ósea es una enfermedad rara que se puede ver asociada con enfermedades autoinmunes como el lupus eritematoso sistémico (LES) y que puede llegar a ser confundida con las manifestaciones propias de la enfermedad. Se presenta el caso de una paciente con mielofibrosis autoinmune en el contexto de LES.
Assuntos
Humanos , Feminino , Adulto , Condições Patológicas, Sinais e Sintomas , Processos Patológicos , Fibrose , Doenças da Pele e do Tecido Conjuntivo , Doenças do Tecido Conjuntivo , Lúpus Eritematoso SistêmicoRESUMO
RESUMEN Introducción: El síndrome de Sjögren (SS) es una enfermedad autoinmune, con compromiso multisistémico, especialmente de la función de las glándulas exocrinas. Los pacientes cursan con una alta carga de la enfermedad que compromete su calidad de vida, asociado sobre todo a fatiga crónica, ansiedad, depresión y disfunción sexual, entre otras dificultades. Objetivo: Describir las generalidades de las principales escalas (específicas y no específicas) para la evaluación de la calidad de vida en pacientes con SS. Métodos: Se realizó una revisión narrativa y ampliada de la literatura. Se hizo una búsqueda en las diferentes bases de datos (PubMed, Embase, Cochrane, BVS) y de literatura gris, seleccionando los artículos relacionados con el tema y realizando una extracción de datos de estos. Resultados: Se encontraron 3 escalas realizadas específicamente para pacientes con SS: Sjögren's Syndrome Patient Reported Index, Psychometric Testing of the Primary Sjögren's Syndrome Quality of Life Questionnaire y Profile of Fatigue and Discomfort, y 14 escalas realizadas en el contexto de otras enfermedades (no específicas), utilizadas en pacientes con SS. Conclusiones: Como en otras afecciones, en pacientes con SS es importante la evaluación de la calidad de vida. A tal efecto se cuenta con múltiples escalas, a parte de las 3 citadas, elaboradas específicamente para este tipo de pacientes, sin dejar de lado las escalas no específicas, como el Beck Depression Inventory y el Ocular Surface Disease Index, que tienen su mayor utilidad en la evaluación específica de síntomas asociados a esta enfermedad. Resultados: Se encontraron 3 escalas realizadas específicamente para pacientes con SS: Sjögren's Syndrome Patient Reported Index, Psychometric Testing of the Primary Sjögren's Syndrome Quality of Life Questionnaire y Profile of Fatigue and Discomfort, y 14 escalas realizadas en el contexto de otras enfermedades (no específicas), utilizadas en pacientes con SS.
ABSTRACT Introduction: Sjögren's syndrome (SS) is an autoimmune disease, with multisystemic invol- vement, particularly compromising the function of exocrine glands. The patients have a high burden of the disease associated with chronic fatigue, anxiety, depression, sexual dysfunction, among others. Objective: To describe the generalities of the main scales (specific and non-specific) for the evaluation of quality of life in patients with SS. Methods: A narrative review and expanded review was performed, including a search conducted in the different databases (PubMed, Embase, Cochrane, VHL) and grey literature. Articles related to the topic were selected and data extracted from them. Results: Three scales were developed specifically for patients with SS, and included Sjögren's Syndrome Patient Reported Index, Psychometric Testing of the Primary Sjögren's Syndrome Quality of Life Questionnaire, and Profile of Fatigue and Discomfort, and 14 scales performed in context of other diseases (non-specific) used in patients with SS. Conclusions: As is the case in other diseases, it is important to evaluate the quality of life in patients with SS. There are multiple scales for this evaluation, including the 3 scales mentioned before, specifically developed for these types of patients. The non-specific scales worth noting are, the Beck Depression Inventory and the Ocular Surface Disease Index, having their greatest use in the specific evaluation of symptoms associated with this disease.
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Humanos , Qualidade de Vida , Síndrome de Sjogren , Sinais e Sintomas , Pesos e Medidas , DiagnósticoRESUMO
Presentamos el caso de una mujer de 32 años, con dos años de evolución de lesiones enpiel tipo placas, induradas, nodulares, dolorosas, inicialmente en miembros inferiores conposterior compromiso generalizado, con evidencia de lesiones cutáneas sugestivas dedermatomiositis y biopsia de piel que reporta calcinosis; inicialmente sin evidencia clínicani paraclínica que sugiriera compromiso muscular inflamatorio asociado. La resonanciamagnética nuclear de músculos y la biopsia muscular confirmaron la presencia de estecompromiso, definiendo los diagnósticos de dermatomiositis hipomiopática y calcinosisuniversal, dos espectros de una patología con baja frecuencia de presentación en lapoblación adulta.
The case is presented of a 32 year-old woman with indurated, raised and painful plaques in the skin of 2 years onset. It began in the legs, but later with a generalized extension to other regions, with evidence of cutaneous lesions very suggestive of dermatomyositis. The skin biopsy reported as calcinosis; initially without clinical evidence of inflammatory muscle compromise. A Nuclear Magnetic Resonance study and muscle biopsy showed this compromise, leading to the diagnosis of hypomyopathic dermatomyositis and universal calcinosis, two pathologies with rare presentation in an adult patient.
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Humanos , Calcinose , DermatomiositeRESUMO
Introducción: La identificación radiológica de sacroiliítis es de vital importancia para el diagnóstico de las SpA, el uso de la resonancia magnética nuclear (RNM) ha ayudado en la detección temprana de este hallazgo, mejorando el tratamiento y pronóstico de los pacientes, con el inconveniente de ser un estudio costoso y de limitado acceso, lo que restringe su uso en la práctica clínica, situación que facilita la solicitud de estudios más económicos y de fácil acceso, como la radiografía de pelvis y la gammagrafía ósea (GO).Objetivos: Establecer la utilidad de la GO para el diagnóstico de sacroiliítis, en pacientes con SpA.Materiales y métodos: Se determinó sensibilidad, especificidad, valor predictivo positivo y valor predictivo negativo de la GO para el diagnóstico de sacroiliítis, usando como patrón de oro diagnóstico la RNM, en una cohorte colombiana de pacientes con diagnóstico de SpA.Resultados: Se analizaron 55 pacientes, 43.6% mujeres y 53.3% hombres, promedio de duración de la enfermedad 9.1±8.4 años, edad promedio de 31±10.5 años al inicio de los síntomas. Predominaron los pacientes con espondiloartritis no diferenciada (uSpA) (60.5%), y compromiso mixto (axial y periférico) (58,2%). El comportamiento de la GO fue sensibilidad 15% (IC 95% 0.04-0,25), especificidad 81% (IC 95% 0.72-0,9), valor predictivo positivo 32% (IC 95% 0.11-0.52), valor predictivo negativo 62% (IC 95% 0.52-0.72). Conclusiones: Se evidencia una sensibilidad variable de la GO con baja especificidad, valor predictivo positivo y valor predictivo negativo, para la detección de sacroiliítis, lo que permite establecer que no es útil como ayuda diagnóstica en espondiloartritis (SpA).
Introduction: Radiological identification of sacroiliitis is essential in the diagnosis of Sacroiliitis spondyloarthritis. Nuclear Magnetic Resonance has helped to optimize its early detection, Scintigraphy leading to improved treatment and a better prognosis for patients. The problem with NMR Spondyloarthritis is that it is expensive and there is limited access to it. For this reason cheaper studies such Magnetic resonance imaging pelvis x-rays and scintigraphy used more often than nuclear magnetic resonance in clinical practice. Objective: To determine the usefulness of scintigraphy in diagnosing sacroiliitis. Methods: The sensitivity, specificity, positive predictive value, negative predictive value of Bone Scintigraphy were analyzed for the diagnosis of sacroiliitis in a Colombian patient cohort with Spondyloarthritis using nuclear magnetic resonance as the gold standard. Results: A total of 55 patients were analyzed, 43.6% females and 53.3% males, with a mean disease duration of 9.1± years. The mean age of onset of symptoms was 31± years. Undifferentiated spondyloarthritis was the most frequent pathology (60.5%), and mixed pattern was the most common (58.2%). The scintigraphy performance compared to NMR was, sensitivity: 15% (95% CI; 0.04- 0.25), specificity: 81% (95% CI; 0.72- 0.9), positive predictive value: 32% (95% CI; 0.11-0.52), and negative predictive value: 62% (95% CI; 0.52-0.72). Conclusions: Scintigraphy has variable sensitivity but a low specificity, positive predictive value, and negative predictive value, for sacroiliitis detection, thus it can be said that it is not useful in the diagnosis of spondyloarthritis.