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1.
Rev. colomb. anestesiol ; 49(3): e500, July-Sept. 2021. graf
Article in English | LILACS, COLNAL | ID: biblio-1280182

ABSTRACT

Systemic sclerosis is an immunological disorder characterized by tissue fibrosis and multi-organ dysfunction.1 The accompanying images exhibit electrocardiographic changes in severe systemic sclerosis. Advanced 3:1 atrioventricular block, best observed in Lead Vi, suggests extensive fibrosis of the conduction system (Image A). While one P wave is buried in the T wave (black arrows), two are evident (red arrows) along the isoelectric line. Bradyarrhythmia related prolonged QT interval, best measured in Lead II represents increased risk for torsades-de-pointes, a polymorphic ventricular tachyarrhythmia. Additionally, right bundle branch block with giant T wave inversions (T wave depth > 10 mm) in precordial leads V2- 4 suggests pulmonary hypertension. Post-induction the rhythm abruptly changes to torsades-de-pointes (Image B) necessitating defibrillation.


La esclerosis sistémica es un trastorno inmunológico caracterizado por fibrosis tisular y disfunción multiorgánica. 1 Las imágenes adjuntas muestran cambios electrocardiográficos en la esclerosis sistémica grave. El bloqueo auriculoventricular avanzado 3: 1, que se observa mejor en la derivación VI, sugiere una fibrosis extensa del sistema de conducción ( Imagen A ).Mientras que una onda P está enterrada en la onda T (flechas negras), dos son evidentes (flechas rojas) a lo largo de la línea isoeléctrica. El intervalo QT prolongado relacionado con bradiarritmia, mejor medido en la derivación II, representa un mayor riesgo de torsades-de-pointes, una taquiarritmia ventricular polimórfica. Además, el bloqueo de la rama derecha del haz con inversiones de la onda T gigante (profundidad de la onda T> 10 mm) en las derivaciones precordiales V2- 4 sugiere hipertensión pulmonar. Después de la inducción, el ritmo cambia abruptamente a torsades-de-pointes ( Imagen B ), lo que requiere desfibrilación.


Subject(s)
Humans , Arrhythmias, Cardiac , Scleroderma, Systemic , Electrocardiography , Tachycardia , Bradycardia , Bundle-Branch Block , Risk , Tachycardia, Ventricular , Atrioventricular Block , Hypertension, Pulmonary
2.
Arq. bras. oftalmol ; 84(3): 271-274, May-June 2021. graf
Article in English | LILACS | ID: biblio-1248964

ABSTRACT

ABSTRACT This study aimed to analyze the anterior lens capsule specimens from both eyes of a patient with systemic sclerosis and compare them to the eyes of a control patient. No significant differences between systemic sclerosis and control eyes were observed in the results from the hematoxylin-eosin and picrosirius staining. In the samples obtained from both systemic sclerosis and control eyes, there were expressions of caspase, a molecule expressed in cell death by apoptosis. Heparanase was overexpressed in the systemic sclerosis sample compared to the control sample. Therefore, the anterior lens capsule of the patient with systemic sclerosis is probably affected by the disease since it showed marked expression of heparanase 1.(AU)


RESUMO Analisamos as amostras das cápsulas anteriores do cristalino de uma paciente com esclerose sistêmica e comparamos com as de um paciente controle. Não foram observadas diferenças significativas entre esclerose sistêmica e controle nos resultados da coloração com hematoxilina-eosina e picrosirius. Nas amostras obtidas da esclerose sistêmica e do controle, obtivemos expressão de caspase, uma molécula expressa na morte celular por apoptose. A heparinase foi expressa de forma mais marcante na amostra de esclerose sistêmica quando comparada ao controle. Portanto, a cápsula anterior do cristalino da paciente com esclerose sistêmica provavelmente foi afetada pela doença, uma vez que mostrou expressão aumentada de heparinase 1.(AU)


Subject(s)
Humans , Scleroderma, Systemic/physiopathology , Heparin Lyase/administration & dosage , Hematoxylin , Lens Capsule, Crystalline/anatomy & histology
3.
Odontol. Clín.-Cient ; 20(3): 54-60, jul.-set. 2021. ilus, tab
Article in Portuguese | LILACS, BBO | ID: biblio-1370425

ABSTRACT

Este estudo objetivou verificar as principais manifestações bucofaciais que as doenças reumáticas podem causar. Foram selecionadas, assim, 05 doenças reumáticas: Artrite Reumatóide (AR), Síndrome de Sjogren (SS), Lúpus Eritematoso Sistêmico (LES), Esclerose Sistêmica (ES) e a Síndrome de Behçet (SB). Estas, por sua vez, foram escolhidas por apresentarem como sinais e sintomas clínicos problemas orofaciais. Foi elaborado, dessa maneira, uma revisão bibliográfica de trabalhos com vintênio de 2000 a 2020 nas seguintes bases: LILACS, MEDLINE e SCIELO. Propõe-se, portanto, a introdução de um Cirurgião-dentista na equipe multidisciplinar de Reumatologia para diagnosticar e tratar as especificidades bucofaciais que acometem os portadores de problemas reumáticos... (AU)


This study aimed to verify as main bucofacial manifestations that rheumatic diseases can cause. Thus, 05 rheumatic diseases were selected: Rheumatoid Arthritis (RA), Sjogren's Syndrome (SS), Systemic Lupus Erythematosus (SLE), Systemic Sclerosis (ES) and Behçet's Syndrome (SB). These, in turn, were chosenbecause they present as orofacial clinical signs and symptoms. In this way, a bibliographic review of works with twenty years from 2000 to 2020 was prepared on the following bases: LILACS, MEDLINE and SCIELO. Therefore, it is proposed to introduce a dental surgeon in the multidisciplinary team of Rheumatology to diagnose and treat as orofacial specificities that affect patients with rheumatic problems... (AU)


Subject(s)
Humans , Male , Female , Arthritis, Rheumatoid , Saliva , Scleroderma, Systemic , Facial Pain , Sjogren's Syndrome , Temporomandibular Joint Disorders , Behcet Syndrome , Rheumatic Diseases , Oral Health , Oral Medicine , Lupus Erythematosus, Systemic , Rheumatology
4.
Rev. cuba. reumatol ; 23(1): e839, tab, graf
Article in Spanish | LILACS, CUMED | ID: biblio-1280401

ABSTRACT

Introducción: La esclerosis sistémica es una enfermedad autoinmune del tejido conectivo donde ocurre inicialmente la vasculopatía y persiste durante toda la enfermedad. El índice de actividad revela un periodo crítico de la enfermedad. Objetivo: Evaluar la evolución clínica del índice de actividad de pacientes con esclerosis sistémica para determinar si el esquema terapéutico aplicado disminuye los síntomas de actividad sistémica. Métodos: Estudio cuasi experimental terapéutico de 31 pacientes atendidos en el Hospital Lucía Íñiguez Landín de Holguín que se dividieron en dos grupos según las etapas clínicas obtenidas del índice de desarrollo integral desde marzo del 2013 hasta marzo del 2016: el grupo A (etapas clínicas I y II) con 16 pacientes y el grupo B (etapas clínicas III y IV) con 15 pacientes. La evolución se evaluó según variables del instrumento al inicio, a los 6 y 12 meses de aplicado el esquema terapéutico. Se utilizó la prueba T o la prueba exacta de Fisher cuando los valores eran igual a 3 o menores. El cálculo de la media, análisis porcentual y la prueba de Wilcoxon se usaron para conocer la relación de variables en el tiempo. Resultados: El esquema terapéutico aplicado, previa validación, mejoró el índice de actividad de los pacientes de ambos grupos A y B (en etapas clínicas tempranas y tardías). Al evaluar el índice de actividad, en esta serie predominó la actividad moderada, tanto a los 6 como a los 12 meses durante el tratamiento médico. En ambos grupos la mejoría del índice de actividad fue significativa, tanto para la actividad moderada como para la intensa, más notable a partir de los 12 meses con p≤0,05 para el grupo A. Hubo baja susceptibilidad para la mejoría de los sistemas gastrointestinal y respiratorio, en el trascurso de la evaluación de este índice. Conclusiones: Se alcanzó mejoría en el índice de actividad de pacientes con esclerosis sistémica, con el esquema terapéutico aplicado, con estabilidad clínica y humoral desde las etapas iniciales de la enfermedad(AU)


Introduction: The systemic sclerosis is an autoimmune disease of the connective tissue where the vasculopathy happens initially and persist during all the disease. The immune component starts since the inflammatory process triggers off but he diminishes until you dwell on the evolutionary course and it is substituted for fibrosis, this ends pathogenic acquires great significance in the process. The index of activity reveals a critical period of the disease. Objective: Evaluating patients' clinical evolution of the index of activity with systemic sclerosis with the applied therapeutics. Methods: The study was quasi-experiences (or secondary prevention). In order to determine if the therapeutic applied scheme decreases symptomatology of its systemic activity. You started in March of the 2013 to March of the 2016, with duration of 24 months. They were 31 patients that split into two groups according to the clinical stages obtained of Comprehensive Development Index. In the group to (clinical stages I and II) 16 patients and in the group B (clinical stages III and IV) 15 patients. The evolution evaluated according to variables of the instrument of evaluation the start, to the six and 12 months itself of once the therapeutic scheme was applied. The T utilized the proof itself, or exact Fisher's proof when moral values were all the same or minor to three, the statistical significance determined in p≥ 0.05 itself. The calculation of the stocking, percentage analysis, and Wilcoxon's proof to know the relation of variables through the time. Results: The therapeutic applied scheme, previous validation, you improved the index of activity of the patients of both groups A and B that is in clinical premature and overdue stages. In the activity moderated for the group A statistical significance for system microvascular (0.023) and respiratory (0.025) to the six months, and to the 12 months' skin (0.023) and microvascular (0.006). For the intense activity significant improvement to the six months for muscleskelettic (0.005) and rheumatoid positive factor (0.008), to the 12 months' significant improvement for muscleskelettic (0.004); and examine of laboratory like erythrocyte sedimentation rate (0.008) circulating immune complexes (0.005), and rheumatoid factor (0.003). For the group B in the moderate activity significant improvement for respiratory system existed (0.014), and cardiovascular (0.020) that kept to the 12 months, added up its digestive system (0.008). Evident level improvement of skin (0.004), circulating immune complexes (0.008) and rheumatoid factor were caught up within the intense activity to the 12 months (0.014). Conclusions: Improvement in the index of activity of patients with systemic sclerosis, with the therapeutic scheme applied, with clinical stability and humoral from initial stages of the disease was caught up with(AU)


Subject(s)
Humans , Rheumatoid Factor , Scleroderma, Systemic/drug therapy , Prednisone/therapeutic use , Clinical Evolution , Cyclophosphamide/therapeutic use , Disease Susceptibility , Antigen-Antibody Complex , Secondary Prevention
5.
Hematol., Transfus. Cell Ther. (Impr.) ; 43(1): 65-86, Jan.-Mar. 2021. tab
Article in English | LILACS | ID: biblio-1154293

ABSTRACT

ABSTRACT Autoimmune diseases are an important field for the development of bone marrow transplantation, or hematopoietic stem cell transplantation. In Europe alone, almost 3000 procedures have been registered so far. The Brazilian Society for Bone Marrow Transplantation (Sociedade Brasileira de Transplantes de Medula Óssea) organized consensus meetings for the Autoimmune Diseases Group, to review the available literature on hematopoietic stem cell transplantation for autoimmune diseases, aiming to gather data that support the procedure for these patients. Three autoimmune diseases for which there are evidence-based indications for hematopoietic stem cell transplantation are multiple sclerosis, systemic sclerosis and Crohn's disease. The professional stem cell transplant societies in America, Europe and Brazil (Sociedade Brasileira de Transplantes de Medula Óssea) currently consider hematopoietic stem cell transplantation as a therapeutic modality for these three autoimmune diseases. This article reviews the evidence available.


Subject(s)
Humans , Scleroderma, Systemic , Crohn Disease , Bone Marrow Transplantation , Hematopoietic Stem Cell Transplantation , Scleroderma, Diffuse , Multiple Sclerosis
6.
Adv Rheumatol ; 61: 9, 2021. tab, graf
Article in English | LILACS | ID: biblio-1152744

ABSTRACT

Abstract Background: In the past 20 years, hematopoietic stem cell transplantation (HSCT) has been investigated as treatment for systemic sclerosis (SSc). The goal of HSCT is to eradicate the autoreactive immune system, which is replaced by a new immune repertoire with long-lasting regulation and tolerance to autoantigens. Here, we describe the clinical outcomes of severe and refractory SSc patients that underwent HSCT at a single Brazilian center. Patients and methods: This is a longitudinal and retrospective study, including 70 adult SSc patients, with an established diagnosis of SSc, and who underwent autologous HSCT from 2009 to 2016. The procedure included harvesting and cryopreservation of autologous hematopoietic progenitor cells, followed by administration of an immunoablative regimen and subsequent infusion of the previously collected cells. Patients were evaluated immediately before transplantation, at 6 months and then yearly until at least 5-years of post-transplantation follow-up. At each evaluation time point, patients underwent clinical examination, including modified Rodnan's skin score (mRSS) assessment, echocardiography, high-resolution computed tomography of the lungs and pulmonary function. Results: Median (range) age was 35.9 (19-59), with 57 (81.4%) female and median (range) non-Raynaud's disease duration of 2 (1-7) years. Before transplantation, 96% of the patients had diffuse skin involvement, 84.2%, interstitial lung disease and 67%, positive anti-topoisomerase I antibodies. Skin involvement significantly improved, with a decline in mRSS at all post-transplantation time points until at least 5-years of follow-up. When patients with pre-HSCT interstitial lung disease were analyzed, there was an improvement in pulmonary function (forced vital capacity and diffusing capacity of lung for carbon monoxide) over the 5-year follow-up. Overall survival was 81% and progression-free survival was 70.5% at 8-years after HSCT. Three patients died due to transplant-related toxicity, 9 patients died over follow-up due to disease reactivation and one patient died due to thrombotic thrombocytopenic purpura. Conclusions: Autologous hematopoietic progenitor cell transplantation improves skin and interstitial lung involvement. These results are in line with the international experience and support HSCT as a viable therapeutic alternative for patients with severe and progressive systemic sclerosis.(AU)


Subject(s)
Humans , Adult , Scleroderma, Systemic/surgery , Hematopoietic Stem Cells , Cryopreservation/instrumentation , Hematopoietic Stem Cell Transplantation/instrumentation , Disease Progression , Retrospective Studies , Longitudinal Studies
7.
Adv Rheumatol ; 61: 15, 2021. tab, graf
Article in English | LILACS | ID: biblio-1152741

ABSTRACT

Abstract Background: Systemic sclerosis (SSc) Is a clinically complex and challenging disease, that leads to skin fibrosis. Its most frequent complication is interstitial lung disease (ILD), which leads to a worse prognosis. In this situation, cyclophosphamide is considered the gold standard for its treatment, despite the controversies regarding its efficacy and toxicity. However, studies using rituximab (RTX) have shown that this drug may be a promising therapeutic option. Objectives: This paper objective was to analyze the scientific evidence on the RTX effects on SSc. Methods: A systematic review (SR) was performed including clinical trials (CTs) on the use of RTX in SSc, published up to May 2020. The studies were identified through systematic searches in bibliographic databases using a predefined search strategy. The following databases were used: PUBMED, SCOPUS, SCIELO, LILACS, SCIENCE DIRECT, WEB OF SCIENCE, COCHRANE, WHOLIS, PAHO and EMBASE. Also, a manual search was performed. The methodological quality of the studies was determined using Jadad scale, Risk of Bias Tool (RoB 2.0) and Risk of Bias in Non-Randomized Studies - of Interventions tool (ROBINS-I). A meta-analysis of the randomized CTs was performed, using Review Manager. Results: Ten CTs were included in this SR. Of these, three were randomized and seven were non-randomized. Five showed a statistically significant improvement in forced vital capacity (FVC) at some time during follow-up. Regarding the skin, eight studies showed statistically significant improvements according toa the modified Rodnan skin score. The meta-analysis found positive effects of RTX in SSc, with a statistical significance for lung disease. Conclusion: Rituximab is a promising strategy for the SSc-associated ILD and cutaneous fibrosis treatment. PROSPERO registration number: CRD42019132018.(AU)


Subject(s)
Humans , Scleroderma, Systemic/drug therapy , Rituximab/therapeutic use , Prognosis , Cyclophosphamide/adverse effects , Cyclophosphamide/therapeutic use
8.
Autops. Case Rep ; 11: e2020220, 2021. graf
Article in English | LILACS | ID: biblio-1142413

ABSTRACT

Scleroderma is a rare autoimmune disease characterized by excessive collagen production. The oral manifestations of the patient with scleroderma can include microstomia, xerostomia, and changes in the resorption teeth. We report the case of a 7-year-old female patient diagnosed with systemic scleroderma where photobiomodulation therapy was used to treat xerostomia associated with hyposalivation. She attended a pediatric clinic and presented with dry and rigid facial skin, trismus, xerostomia, malocclusion, and difficulty swallowing. Stimulated salivary flow was assessed before, during, and after treatment. Photobiomodulation therapy was conducted at four points at the sublingual glands with 660 nm, 100 mW, and 0.8 J/cm2 to each point; eight points at the parotid glands; and six points at the submandibular glands with 808 nm, 100 mW, and 0.8 J/cm2 for 8 seconds at each point. After this therapy, an increase in salivary flow, remission of the xerostomia, and an improvement in mastication and swallowing were observed. Photobiomodulation therapy was effective in controlling xerostomia in this pediatric patient, resulting in increased salivary flow and an improvement in her quality of life.


Subject(s)
Humans , Female , Child , Scleroderma, Systemic , Xerostomia , Low-Level Light Therapy
9.
Med. interna (Caracas) ; 37(1): 26-30, 2021. ilus, tab
Article in Spanish | LILACS, LIVECS | ID: biblio-1253885

ABSTRACT

La enfermedad indiferenciada del tejido conectivo es una condición de etiología desconocida que comparte características clínicas, patológicas y de laboratorio de varias colagenosis, sin cumplir los criterios del Colegio Americano de Reumatología para el diagnóstico de una enfermedad reumática específica y muchos pacientes evolucionan a condiciones definidas a lo largo del tiempo tales como Lupus, Esclerosis sistémica progresiva, Enfermedad de Sjögren entre otros. Antecedentes: Linfoma Hodgkin diagnosticado desde 2012 para lo cual recibió múltiples esquemas de quimioterapia. Las muestras de ganglio y médula ósea se habían enviado al laboratorio de Inmunopatologia de la Universidad de Stanford y allí no se apreciaron hallazgos compatibles con enfermedad linfoproliferativa. Enfermedad actual: Mujer de 27 años de edad con cuadro clínico de 1 mes de evolución, caracterizado por edema blando en miembros inferiores acompañado de edema palpebral matutino; concomitantemente presenta aumento de temperatura intermitente sin patrón especifico y dolor osteomuscular generalizado con limitación para la deambulación. Se ingresa. Al examen físico, regulares condiciones clínicas. En la piel se aprecia engrosamiento cutáneo importante. Se realizó biopsia cutánea y los hallazgos fueron compatibles con Esclerosis Sistémica(AU)


Undifferentiated connective tissue disease is a condition of unknown etiology that shares clinical, pathological and laboratory characteristics of several collagenopathies that do not meet the criteria of the American College of Rheumatology for the diagnosis of a specific disease; a large number of patients evolve to conditions defined over time such as Lupus, Systemic Sclerosis, Sjogren's Disease, among others. Past history: Hodgkin lymphoma was diagnosed since 2012 for which she received multiple chemotherapy schemes. A gland biopsy was sent to the Stanford University, as well as a bone marrow sample, and lymphoma was discarded. Present history: this 27-year-old female consulted for edema in lower limbs present during one month, accompanied by eyelid edema in the mornings; also fever without a specific pattern, myalgias and arthralgias. On physical examination, the skin was thickened and limb edema was present. A skin biopsy was performed, and the findings were consistent with Systemic Sclerosis. The patient is receiving cyclophosphamide and Azathioprine and leading her normal life(AU)


Subject(s)
Rheumatology , Scleroderma, Systemic/diagnosis , Undifferentiated Connective Tissue Diseases/physiopathology , Hematologic Diseases , Biopsy , Diagnostic Imaging
10.
Rev. Méd. Paraná ; 79(1): 97-101, 2021.
Article in Portuguese | LILACS | ID: biblio-1282503

ABSTRACT

INTRODUÇÃO: A calcinose é uma complicação frequente da esclerose sistêmica e da dermatomiosite, resultando em dor local, contraturas, ulcerações, infecções secundárias e limitação funcional. Não há tratamento eficaz para calcinose mas o tiossulfato de sódio tem se mostrado promissor. OBJETIVOS: Analisar a resposta terapêutica ao tiossulfato de sódio 10% intralesional nas calcinoses de pacientes com dermatomiosite e esclerose sistêmica. MÉTODOS: Estudo prospectivo, não controlado, incluindo 7 pacientes, totalizando 10 calcinoses, sendo um paciente com dermatomiosite e 6 com esclerose sistêmica. Desfechos primários: melhora da dor pela escala visual analógica e diminuição do maior e menor diâmetros das calcinoses ao raio-x. Desfechos secundários: melhora da qualidade de vida pelo SF12 (Medical Outcomes Study Short Form 12) e da função pelo HAQ (Health Assesment Questionnaire). Resultados foram expressos em médias e as variáveis contínuas comparadas pelo teste t-Student ou pelo teste de Wilcoxon. RESULTADOS: Foram realizadas 2 a 8 aplicações intralesionais (média de 3,7), com média de 8,9mg de tiossulfato por injeção a intervalos de 13 a 56 dias, com intervalo médio de 19 dias. Todos os pacientes relataram melhora da dor, porém esse resultado não foi significativamente estatístico. Não houve redução do diâmetro das calcinoses, nem melhora da qualidade de vida ou funcional. CONCLUSÃO: Baixas doses de tiossulfato de sódio administradas por injeções intralesionais, em intervalos médios de 19 dias, em número limitado de aplicações, não foram efetivas para o tratamento da calcinose


BACKGROUND: Calcinosis is a frequent complication of systemic sclerosis and dermatomyositis, resulting in local pain, contractures, ulcerations, secondary infections and functional limitations. There is no effective treatment for calcinosis, but sodium thiosulphate has shown to be promising. OBJECTIVE: To analyze the therapeutic response to 10% intralesional sodium thiosulphate on calcinosis of patients with dermatomyositis and systemic sclerosis. METHODS: Non-controlled prospective study, with 7 patients and 10 calcinoses, of which one had dermatomyositis and six had scleroderma. Primary outcomes: improvement in the pain by the visual analogue scale, and a decrease in both diameters (biggest and smallest) of calcinoses on X-ray. Secondary outcomes: improvement in quality of life by SF12 (Medical Outcomes Study Short Form 12), and of function by HAQ (Health Assessment Questionnaire). Results were expressed as means; continuous variables were compared by the t-Sudent test or by the Wilcoxon test. RESULTS: Two to 8 intralesional applications were performed (mean of 3.7), with mean of 8.9mg of thiosulphate per injection, at 13-56 days intervals (mean of 19 days). All patients reported improvement in pain, albeit such result was not statistically significant. There was no reduction in the diameters of calcinoses, nor there were quality of life or functional improvements. CONCLUSION: Low doses of sodium thiosulphate, administered through intralesional injections, in mean intervals of 19 days, in a limited number of applications, were not effective for calcinosis treatment


Subject(s)
Humans , Scleroderma, Systemic , Therapeutics , Calcinosis , Dermatomyositis , Thiosulfates
11.
Rev. argent. reumatolg. (En línea) ; 31(4): 25-29, dic. 2020. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1288207

ABSTRACT

La marihuana medicinal ha sido propuesta en los últimos años como una solución a algunos problemas médicos refractarios a otros tratamientos. El dolor crónico es un síntoma habitual entre los pacientes que consultan al reumatólogo. Distintas enfermedades pueden verse involucradas en este pan-sindrome que engloba desde enfermedades autoinmunes, sindromes dolorosos locorregionales, enfermedades degenerativas hasta desórdenes con componentes psicológicos. En muchos de estos casos, sobre todo los dos últimos, las intervenciones farmacológicas no siempre son eficaces o seguras en el corto, mediano o largo plazo. A pesar de la puesta en primer plano del tema desde hace unos años y la reciente reglamentación de la Ley del cannabis medicinal para su autocultivo y venta en farmacias en Argentina, está la sensación entre los médicos que siguen faltando suficientes datos para el uso de los cannabinoides en las enfermedades reumáticas. Desde la puesta al día realizada en esta revista hace tres años, han aparecido algunos estudios fisiológicos acerca del potencial efecto inmunomodulatorio de los cannabinoides en artritis reumatoidea (AR) y esclerosis sistémica (ES) que resultan de interés, y algunos estudios clínicos que se mencionarán. En esta revisión se analizará la literatura actual de los cannabinoides en el tratamiento de las enfermedades reumáticas y se discutirá dicha evidencia en relación al contexto regulatorio local.


Medical marijuana has been proposed in recent years as a solution to some medical problems refractory to other treatments. Chronic pain is a common symptom among patients who consult a rheumatologist. Different diseases can be involved in this pan-syndrome that ranges from autoimmune diseases, locoregional pain syndromes, degenerative diseases to disorders with psychological components. In many of these cases, especially the last two, pharmacological interventions are not always effective or safe in the short, medium or long term. In this review, the most current literature on the subject regarding cannabinoids and the treatment of rheumatic diseases will be analyzed. Despite the focus of the issue in recent years and the recent regulation of the Medical Cannabis Law for its self-cultivation and sale in pharmacies in Argentina, there is still insufficient data for the use of cannabinoids in the short, medium and long-term in rheumatic diseases. In recent years, some physiological studies have appeared about the potential immunomodulatory effect of cannabinoids in rheumatoid arthritis and systemic sclerosis (SS), and some clinical studies in SS, but there is still a long way to go to travel in this direction. In this review, the current literature on cannabinoids in the treatment of rheumatic diseases will be analyzed and this evidence will be discussed in relation to the local regulatory context.


Subject(s)
Cannabis , Arthritis , Scleroderma, Systemic , Cannabinoids , Fibromyalgia , Medical Marijuana
13.
Rev. colomb. reumatol ; 27(supl.1): 2-9, Oct.-Dec. 2020. tab, graf
Article in English | LILACS | ID: biblio-1341319

ABSTRACT

ABSTRACT Background: Systemic sclerosis is an autoimmune and multisystemic disease characterized by vascular involvement and fibrosis that mainly affects the skin and internal organs; its morbidity and mortality are the highest of rheumatic diseases. Objective: To determine the sociodemographic, clinical, and paraclinical characteristics of adult patients with systemic sclerosis in a reference center in rheumatology in northwestern Colombia, between 2006 and 2016. Methods: A retrospective descriptive study was conducted. Information on sociodemographic, clinical, and paraclinical variables was collected from the review of medical records. The data were processed with the IBM SPSS 22 statistical package. The qualitative variables were expressed in absolute and relative frequencies and the quantitative variables in mean and standard deviation or median and interquartile range, according to their distribution. Results: 44 patients were included, predominantly women (90.9%), with an average age of 59 years; the most common variety was the limited (61.3%). One hundred percent of the subjects presented Raynaud's phenomenon and the most common compromised systems were: cutaneous (100%), musculoskeletal (59.1%), and gastrointestinal (50%). 95.5% of the patients fulfilled the diagnostic criteria ACR / EULAR 2013. Regarding the immunological profile, 72.7% of the individuals had positive antinuclear antibodies, of which 53.1% had a centromeric pattern. Conclusions: Sociodemographic, clinical and similar characteristics were found in this cohort, according to what was published in the literature. The most frequent extracutaneous systems involved were: musculoskeletal, gastrointestinal, and pulmonary, which cause a large part of the associated morbidity, as well as a decrease in quality of life.


RESUMEN Introducción: La esclerosis sistémica es una enfermedad autoinmune y multisistémica, caracterizada por vasculopatía y fibrosis que afecta principalmente la piel y órganos internos; su morbilidad y mortalidad son las más altas de las enfermedades reumatológicas. Objetivo: Determinar las características sociodemográficas, clínicas y paraclínicas de pacientes adultos con esclerosis sistémica en un centro de referencia en reumatología del noroccidente de Colombia, entre el 2006 y el 2016. Métodos: Estudio descriptivo retrospectivo. La información sobre las variables sociodemográficas, clínicas y paraclínicas se recogió de la revisión de registros médicos. Los datos se procesaron con el paquete estadístico IBM SPSS 22. Las variables cualitativas se expresaron en frecuencias absolutas y relativas y las cuantitativas en media y desviación estándar o mediana y rango intercuartílico, según su distribución. Resultados: Se incluyeron 44 pacientes, predominantemente mujeres (90,9%), con una edad promedio de 59 arios; la variedad más común fue la limitada (61,3%). El 100% de los sujetos presentaron el fenómeno de Raynaud y los sistemas comprometidos con mayor frecuencia fueron: cutáneo (100%), musculoesquelético (59,1%) y gastrointestinal (50%). El 95,5% de los pacientes cumplieron con los criterios diagnósticos ACR/EULAR 2013. Con respecto al perfil inmunológico, el 72,7% de los individuos tenía anticuerpos antinucleares positivos, de los cuales, un 53,1% tenía un patrón centromérico. Conclusiones: Se encontraron características sociodemográficas y clínicas similares en esta cohorte, de acuerdo a lo publicado en la literatura. Los sistemas extracutáneos más frecuentemente involucrados fueron: musculoesquelético, gastrointestinal y pulmonar, que causan una gran parte de la morbilidad asociada, así como una disminución en la calidad de vida.


Subject(s)
Humans , Female , Adult , Middle Aged , Aged , Patients , Scleroderma, Systemic , Adult , Autoimmune Diseases , Epidemiology, Descriptive
14.
Rev. colomb. reumatol ; 27(supl.1): 10-25, Oct.-Dec. 2020. tab, graf
Article in English | LILACS | ID: biblio-1341320

ABSTRACT

ABSTRACT Background: Capillaroscopy is an essential tool for the diagnosis of systemic sclerosis. Using this exam as a prognostic factor will allow earlier intervention and probably, delay on disease progression. We aimed to evaluate the prognostic value of capillaroscopy for the prediction of systemic compromise and subtype differentiation in systemic sclerosis. Methods: A systematic literature search was applied in the following electronic databases: Medline, PubMed, Embase, Cochrane, and Lilacs. The research question was designed based on the PICOT model, and the search strategy was built using the MeSH terms "Microscopic Angioscopy," "Scleroderma systemic," "Scleroderma diffuse," Scleroderma Limited," "Early Diagnosis" and Boolean operators. The language was restricted to papers published in Spanish or English, from 1990 to 2019. The search terms were explored for each database, and new terms were added, as appropriate. The searches were made again before the final analyses and further studies were retrieved for inclusion at that time. Reference lists of included studies and recent aligned systematic reviews were also screened. Gray literature was not considered in this review. Results: A total of 183 articles were found in the selected databases: Medline (n: 115), Embase (n: 66), Cochrane (n: 2), Lilacs (n: 0). After excluding articles due to duplication, a total of 66 studies were selected. Within these articles, a screening process was applied based on the title and abstract, taking into account the eligibility criteria, finally obtaining 21 references. Two researchers assessed the selected articles, and all disagreements were solved by consensus. Finally, a total of 14 articles were included. Conclusions: The different abnormalities found in capillaroscopy, especially loss of capillaries, have been consistently associated not only with organ involvement but also with severity of the disease, especially with vascular manifestations (digital ulcers and pulmonary hypertension). The importance of capillaroscopy is not only its diagnostic value but also its predictive value with its consequent implications in the follow-up and management of systemic sclerosis.


RESUMEN Introducción: La capilaroscopia es una herramienta esencial para el diagnóstico de la esclerosis sistémica. Usar este examen como factor pronóstico permitirá realizar una intervención temprana y probablemente retardará la progresión de la enfermedad. Se realizó una revisión de la literatura evaluando el valor pronóstico de la capilaroscopia para predecir el compromiso sistémico de la esclerosis sistémica y su diferenciación por subtipos. Métodos: Se realizó una revisión sistemática de la literatura en las siguientes bases de datos: Medline, PubMed, Embase, Cochrane y Lilacs. La búsqueda se hizo basada en el modelo PICOT y la estrategia de búsqueda fue construida mediante los términos MeSH «Microscopic angioscopy¼, «Scleroderma systemic¼, «Scleroderma diffuse¼, «Scleroderma limited¼, «Early diagnosis¼ y operadores booleanos. El lenguaje fue restringido a artículos publicados en español e inglés desde 1990 hasta 2019. Se realizó la búsqueda en cada base de datos y se adicionaron nuevos términos según fuera apropiado. La búsqueda se realizó de nuevo al final del análisis y se incluyeron los estudios más recientes. La lista de referencias de los estudios incluidos y las revisiones sistemáticas recientemente adicionadas también fueron registradas. No se consideró literatura gris en esta revisión. Resultados: Un total de 183 artículos fueron encontrados en las siguientes bases de datos: Medline (n = 115), Embase (n = 66), Cochrane (n = 2), Lilacs (n = 0). Después de excluir los que estaban duplicados, un total de 66 estudios fueron seleccionados. Dentro de estos artículos, se realizó un proceso de selección basado en título y resumen tomando en cuenta los criterios de elegibilidad, obteniendo finalmente 21 referencias. Dos investigadores revisaron los artículos seleccionados y todas las discrepancias fueron resueltas en consenso. Finalmente, un total de 14 artículos fueron incluidos. Conclusiones: Las diferentes anormalidades encontradas en la capilaroscopia, especialmente la pérdida de capilares, han sido constantemente asociadas no solo con compromiso de órganos sino también a la severidad de la enfermedad, especialmente con manifestaciones vasculares (úlceras digitales e hipertensión pulmonar). La importancia de la capilaroscopia no solo es por su valor diagnóstico sino también por su valor predictivo en relación al seguimiento y manejo de la esclerosis sistémica.


Subject(s)
Humans , Adult , Middle Aged , Aged , Aged, 80 and over , Prognosis , Scleroderma, Systemic , Microscopic Angioscopy , Capillaries , Early Diagnosis , Early Medical Intervention
15.
Rev. colomb. reumatol ; 27(supl.1): 26-35, Oct.-Dec. 2020. graf
Article in English | LILACS | ID: biblio-1341321

ABSTRACT

ABSTRACT Vascular compromise in systemic sclerosis is a pivotal feature of the disease and plays a fundamental role in its morbidity and mortality. Raynaud's syndrome is present in almost every patient and is often reported as the first clinical manifestation. Digital ulcers may present several etiologies, although an ischemic cause is the most frequent origin and occurs in up to 50% of patients. A profound impact on daily life is often observed due to pain and functional impairment. Their primary pathophysiological mechanism is microvascular compromise, although larger vessels may be affected as well. When recurrent lesions are observed, large vessel compromise should be assessed, which may be due to the disease itself or due to atherosclerosis, whenever risk factors are present. Further, these ulcers present an increased risk of infection and progression to gangrene. The presence of digital lesions may be a marker of severity of the disease, as some reports have suggested an association with pulmonary hypertension and cardiac involvement. Treatment strategies have progressed significantly over the last years. Vasodilatation using calcium channel inhibitors is universally offered. When ischemic signs are observed, treatment should be started readily. Prostacyclin infusions should be considered in severe cases, as they have shown the capacity to foster ulceration healing. Whenever recurring lesions are observed, bosentan may be offered. Management with phosphodiesterase inhibitors may be proposed, although their positioning is unclear. Local treatment is equally important over the course of the disease. Surgical interventions are seldom needed.


RESUMEN El compromiso vascular en la esclerosis sistémica es una característica principal de la enfermedad y desempeña un papel fundamental en su morbimortalidad. El síndrome de Raynaud está presente en casi todos los pacientes, y con frecuencia es reportado como la primera manifestación clínica. Las úlceras digitales pueden tener varias etiologías, aunque una causa isquémica es el origen más frecuente y ocurre hasta en 50% de los pacientes. A menudo se observa un profundo impacto en la vida diaria debido al dolor y al deterioro funcional. Su mecanismo fisiopatológico primario es el compromiso microvascular, aunque los vasos más grandes también pueden verse afectados. Cuando se observan lesiones recurrentes se debe evaluar el compromiso de los vasos grandes, que puede deberse a la enfermedad en sí o a aterosclerosis, siempre que existan factores de riesgo. Además, estas úlceras presentan un mayor riesgo de infección y progresión a gangrena. La presencia de lesiones digitales puede ser un marcador de la gravedad de la enfermedad, ya que algunos informes han sugerido una asociación con hipertensión pulmonar y compromiso cardíaco. Las estrategias de tratamiento han progresado significativamente en los últimos anos. La vasodilatación con inhibidores de los canales de calcio se ofrece universalmente. Cuando se observan signos isquémicos el tratamiento debe iniciarse de inmediato. Las infusiones de prostaciclina se deben considerar en casos graves, ya que han demostrado la capacidad de promover la curación de la ulceración. Siempre que se observen lesiones recurrentes se puede administrar bosentán. Se puede proponer el manejo con inhibidores de la fosfodiesterasa, aunque su posicionamiento no está claro. El tratamiento local es igualmente importante durante el curso de la enfermedad. Las intervenciones quirúrgicas rara vez son necesarias.


Subject(s)
Humans , Scleroderma, Systemic , Ulcer , Raynaud Disease , Therapeutics , Indicators of Morbidity and Mortality , Infections
16.
Rev. colomb. reumatol ; 27(supl.1): 36-43, Oct.-Dec. 2020.
Article in English | LILACS | ID: biblio-1341322

ABSTRACT

ABSTRACT Introduction: Systemic sclerosis can involve the lung parenchyma leading to serious complications and even death. Objectives: To describe the most relevant aspects of interstitial lung disease related to systemic sclerosis emphasizing diagnosis and treatment. Materials and methods: A literature review was performed searching in the databases Medline and EMBASE using the MeSH terms «Scleroderma, Systemic", «Lung Diseases, Interstitial¼ and «Pulmonary Fibrosis¼ Results and conclusions: Interstitial lung disease is a common clinical manifestation of systemic sclerosis and one of the main causes of death. Treatment options are limited and have a modest effect in most of the cases.


RESUMEN Introducción: La esclerosis sistémica puede potencialmente comprometer el parénquima pulmonar, llevando a serias complicaciones e incluso a la muerte. Objetivos: Describir los aspectos más relevantes en cuanto a las generalidades de la enfermedad pulmonar intersticial en esclerosis sistémica, su diagnóstico y su tratamiento. Materiales y métodos: Se realizó una búsqueda de literatura en las bases de datos Medline y EMBASE utilizando los términos MeSH «Scleroderma, Systemic¼, «Lung Diseases, Interstitial¼ y «Pulmonary Fibrosis¼. Resultados y conclusiones: La enfermedad pulmonar intersticial es una manifestación frecuente de la esclerosis sistémica y una de las principales causas de muerte en los pacientes que la padecen. Las opciones terapéuticas son limitadas y su efecto es, en muchos casos, modesto.


Subject(s)
Humans , Male , Aged , Aged, 80 and over , Scleroderma, Systemic , Lung Diseases, Interstitial , Therapeutics , Cause of Death , Diagnosis , Lung
17.
Rev. colomb. reumatol ; 27(supl.1): 44-54, Oct.-Dec. 2020. tab, graf
Article in English | LILACS | ID: biblio-1341323

ABSTRACT

ABSTRACT Systemic sclerosis (SSc) is a systemic autoimmune disease in which gastrointestinal manifestations are a frequent complication. Gastrointestinal involvement is present in up to 90 % of patients. The most affected areas are the esophagus and the anorectal tract. Reflux, heartburn and dysmotility are the leading causes of gastrointestinal discomfort. Disordered anorectal function can occur early in the course of SSc and is an important factor in the development of fecal incontinence. Current recommendations to treat gastrointestinal disorders in SSc include the use of proton pump inhibitors, prokinetics and rotating antibiotics. This review discusses the proposed pathophysiological mechanisms, the clinical presentation, the different diagnostic techniques and the current management of the involvement of each section of the gastrointestinal tract in SSc.


RESUMEN La esclerosis sistémica (ES) es una enfermedad autoinmune sistémica en la que las manifestaciones gastrointestinales son una complicación frecuente. El compromiso gastrointestinal está presente hasta en 90% de los pacientes. Las áreas más afectadas son el esófago y el tracto anorrectal. El reflujo, la pirosis y la dismotilidad son las principales causas de malestar gastrointestinal. La función anorrectal alterada puede presentarse temprano en el curso de la ES y es un factor importante en el desarrollo de incontinencia fecal. Las recomendaciones actuales para tratar los trastornos gastrointestinales en la ES incluyen el uso de inhibidores de la bomba de protones, procinéticos y de antibióticos en forma rotativa. Esta revisión discute los mecanismos fisiopatológicos propuestos, la presentación clínica, las diferentes técnicas de diagnóstico y el manejo actual del compromiso de cada sección del tracto gastrointestinal en la ES.


Subject(s)
Humans , Adult , Middle Aged , Aged , Aged, 80 and over , Scleroderma, Systemic , Gastrointestinal Diseases , Autoimmune Diseases , Causality , Diagnosis
18.
Rev. colomb. reumatol ; 27(supl.1): 55-61, Oct.-Dec. 2020. tab, graf
Article in English | LILACS | ID: biblio-1341324

ABSTRACT

ABSTRACT Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by vasculopathy, inflammation, collagen deposits and fibrosis in the skin and internal organs. Renal complications are common in SSc. The spectrum of renal complications in systemic sclerosis includes scleroderma renal crisis (SRC), abnormal renal function, proteinuria, renal chronic vascular disease, kidney injury from nephrotoxic drugs, oxalate calcium nephropathy, glomerulonephritis and vasculitis associated to anti-neutrophil cytoplasmic antibody. Renal involvement remains an important cause of morbidity and mortality in scleroderma. The objective of this review is to describe the renal complications of scleroderma.


RESUMEN La esclerosis sistémica es una enfermedad autoinmune generalizada que se caracteriza por vasculopatía, inflamación, depósito de colágeno y fibrosis en la piel y órganos internos. Las complicaciones renales son comunes y con un amplio espectro e incluyen crisis renal esclerodérmica, función renal anormal, proteinuria, enfermedad vascular renal crónica, daño renal por fármacos nefrotóxicos, nefropatía por oxalato de calcio, glomerulonefritis y vasculitis asociadas a anticuerpos contra el citoplasma de los neutrófilos. La alteración renal permanece siendo una causa importante de morbilidad y mortalidad en la esclerodermia. El objetivo de esta revisión narrativa es describir las complicaciones renales de esta enfermedad.


Subject(s)
Humans , Scleroderma, Systemic , Kidney Diseases , Causality , Morbidity , Mortality
19.
Rev. colomb. reumatol ; 27(supl.1): 62-84, Oct.-Dec. 2020. tab, graf
Article in English | LILACS | ID: biblio-1341325

ABSTRACT

ABSTRACT Mounting evidence has shown non-systemic sclerosis (SSc) related complications as a rising cause of hospital admission and mortality, out of which infections are among the top-five causes. Patients with SSc are at an increased risk of infection due to several features of the treatment options and to the disease itself. For instance, lung involvement is associated with a higher frequency of respiratory infections, whereas the presence of digital ulcers or calcinosis may result in skin and soft tissue infections, and even osteomyelitis. On the other hand, the growing trend towards immunomodulation and immunosuppression in patients with autoimmune diseases will place SSc patients at a higher risk of infectious complications, including opportunistic infections. A low suspicion threshold and an increasing awareness among treating specialists, particularly rheumatologists, are warranted for prevention, early diagnosis and management of infectious complications. Nonetheless, data on risk management strategies in SSc, such as vaccination and antimicrobial prophylaxis, are scarce. A narrative non-systematic review was performed to provide an update of infectious complications in patients with SSc.


RESUMEN La evidencia creciente muestra que las complicaciones no asociadas a la esclerosis sistémica (ES) son una causa cada vez más frecuente de hospitalización y mortalidad, dentro de las cuales las infecciones se encuentran entre las primeras cinco causas. Los pacientes con ES presentan un riesgo elevado de infección asociado con las opciones terapéuticas y con la enfermedad misma. Por ejemplo, el compromiso pulmonar se asocia con una mayor frecuencia de infecciones respiratorias, mientras que la presencia de úlceras digitales o calcinosis pueden resultar en infecciones de piel y tejidos blandos, incluso en osteomielitis. Por otro lado, la tendencia creciente hacia la inmunomodulación y la inmunosupresión, como tratamiento de las enfermedades autoinmunes, pondrá a estos pacientes en un mayor riesgo de infecciones, incluidas las infecciones oportunistas. Son necesarios un umbral bajo de sospecha y un alto nivel de alerta entre las especialidades tratantes, particularmente los reumatólogos, para la prevención, el diagnóstico temprano y el manejo de las complicaciones infecciosas. Sin embargo, la información respecto a estrategias de gestión de riesgo en ES, como la vacunación o la profilaxis antibiótica, es escasa. Se realizó una revisión narrativa no sistemática que presenta una actualización sobre las complicaciones infecciosas en pacientes con ES.


Subject(s)
Humans , Scleroderma, Systemic , Infections , Autoimmune Diseases , Risk , Causality
20.
Rev. colomb. reumatol ; 27(supl.1): 85-110, Oct.-Dec. 2020. tab, graf
Article in English | LILACS | ID: biblio-1341326

ABSTRACT

ABSTRACT Introduction: Osteoarticular manifestations (OAM) are frequently present in patients with systemic sclerosis (SSc). These OAM are related to important functional disability and a severe impact on patient's life quality, therefore, they require special attention from clinicians. Objective: Determining the frequency of reported OAM in SSc patients, the general impact of osteoarticular manifestations in patient's condition and the tools available for diagnosis and treatment. Methodology: A systematic review of the literature was performed for information published between January 1970 and December 2018 in the medical research databases of: Medline, Embase, Lilacs, Scielo, Cochrane and clinicaltrials.gov. Results and Conclusions: A total of 116 articles were included in this review. The type and prevalence of the different OAM reported in the literature were determined. In addition to the evolution of their diagnostic methods, there are more validated methods to assess the impact of OAM in patients with SSc. Randomized clinical trials are required to establish the best treatment strategy for these patients.


RESUMEN Introducción: Las manifestaciones osteoarticulares (MOA) están presentes de forma frecuente en pacientes con esclerosis sistémica (SSc). Generan gran discapacidad funcional con un impacto importante en la calidad de vida de los pacientes, por lo que requieren especial atención por parte de los clínicos. Objetivos: Determinar la frecuencia de las MOA dentro de la SSc, su impacto en la condición general de los pacientes y las diferentes herramientas disponibles de diagnóstico y tratamiento. Metodología: Se realizó una revisión sistemática de la literatura disponible desde enero de 1970 hasta diciembre de 2018 en las bases de datos de Medline, Embase, Lilacs, Scielo, Cochrane y clinicaltrials.gov. Resultados y conclusiones: Se incluyeron un total de 116 artículos en esta revisión. Se determinaron el tipo y la prevalencia de las diferentes MOA reportadas en la literatura. Además de la evolución en los métodos diagnósticos de las mismas, cada vez existen más métodos validados para evaluar el impacto de las MOA en los pacientes con SSC. Se requieren ensayos clínicos aleatorizados que permitan establecer la mejor estrategia de tratamiento para estos pacientes.


Subject(s)
Humans , Scleroderma, Systemic , Quality of Life , Randomized Controlled Trials as Topic , Diagnosis , Joint Diseases
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