Effectiveness of Telemedicine Compared with Standard Care for Patients with Rheumatic Diseases: A Systematic Review.

Objective: The aim of this study was to systematically review the evidence on the effectiveness of telemedicine compared to standard care for patients with rheumatic diseases. Methods: A search was performed in MEDLINE, EMBASE, and the Cochrane Database of Systematic Reviews; for the gray literature, GREYNET databases and a snowball search were used. MeSH or Emtree terms. Three authors independently selected systematic reviews, randomized controlled trail (RCTs), or non-RCTs with patients with autoimmune or inflammatory rheumatic diseases, where telemedicine was compared with standard care. Effectiveness was measured in terms of disease activity, quality of life, and functional activity. The patients' satisfaction was also measured. The risk of bias was assessed by the Cochrane collaboration tool for RCTs and AMSTAR II for systematic reviews. Results: Four RCTs, one cross-out study, and five systematic reviews were included. The studies were conducted with rheumatoid arthritis patients, and one study involved patients with systematic lupus erythematosus. The interventions mainly involved teleconsultation and telemonitoring, with patient-reported outcomes (PROs) being compared with standard care. Four studies measured the effectiveness of telemedicine using PROs, in which three of the RCTs did not find differences in the clinical outcomes, and one found that telemedicine improved the remission of diseases, functional impairment, and radiographic joint damage progression. Two studies measured patient satisfaction with telemedicine and standard care without a significant difference between the groups. Conclusions: Despite heterogeneity between studies, the findings were remarkably consistent in demonstrating that there was no significant difference between the telemedicine group and the control group in terms of PROs and patient satisfaction. Patients should be offered the option of telemedicine to manage their diseases as part of health-care support. Further research is needed on the effectiveness of telemedicine in the long term for patients with rheumatic diseases.

Restrictive cardiomyopathy in a patient with systemic sclerosis and Fabry disease: a case-based review.

Systemic sclerosis (SSc) is a rare immune-mediated vasculopathy characterized by fibrosis of the skin and internal organs. Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene producing α-galactosidase-A enzyme (α-Gal A) deficiency. Being a systemic disease, cardiac involvement in FD has a high mortality rate due to heart failure and arrhythmia. The coexistence of these two entities has not been reported previously. We describe the case of a female patient with limited SSc (lcSSc), a diagnosis based on the presence of sclerodactyly, Raynaud phenomenon, microvascular involvement, and positive anti-centromere antibodies. On follow-up, she developed chest pain, a second-degree A-V block, and restrictive cardiomyopathy (without cardiovascular risk factors). Although heart involvement is common in these two entities, the abnormal thickening of lateral and inferior wall, the infiltration pattern and the conduction system disorders presented herein are more characteristic in a heterozygous female with a cardiac variant of FD. The diagnosis of FD was confirmed with high globotriaosylsphingosine (Lyso-Gb3) levels and identification of GLA gene mutation. The patient was treated with enzymatic replacement (agalsidase alpha) following mild improvement in ventricular mass at 6th month, without clinical deterioration. The related literature on SSc associated with FD is also reviewed.

Clinical Profile of Levamisole-Adulterated Cocaine-Induced Vasculitis/Vasculopathy: A 30-Case Series.

OBJECTIVES: The aims of this study were to describe clinical and laboratory manifestations of patients with levamisole-adulterated cocaine-induced vasculitis/vasculopathy and to propose a skin classification according to the distribution and severity of lesions. METHODS: We report the characteristics of 30 patients admitted with levamisole-adulterated cocaine-induced vasculitis/vasculopathy in 4 high-complexity institutions in Colombia, from December 2010 to May 2017. We compare our findings with the main published series. RESULTS: Median age was 31 years (interquartile range, 27-38 years) with a male-to-female ratio of 5:1. Eighty-three percent of the patients had retiform purpura affecting the limbs, buttocks, face, or abdomen; 73% had ear necrosis, 50% cutaneous ulcers, 17% genital necrosis, 13% oral ulcers, and 10% digital necrosis. Cutaneous involvement was classified according to the frequency of the compromised corporal area, and purpuric lesions were stratified in 4 grades of severity. Anti-neutrophil cytoplasmic autoantibodies were positive in 85% of the cases, lupus anticoagulant in 73%, and antinuclear autoantibodies in 57%; rheumatoid factor was negative in all cases. We found nephritis in 17 cases (57%). Prednisolone was used in most of the patients (70%), with other immunosuppressive agents being used in a lower percentage. Improvement was observed in 93% of the patients, but symptoms recurred in 40%, attributed to relapses in consumption. End-stage chronic renal disease developed in 10% of the cases, and 1 patient died. CONCLUSIONS: Because of rising cocaine consumption and levamisole adulteration frequency, levamisole-adulterated cocaine-induced vasculitis/vasculopathy is becoming more common. Detailed characterization of skin involvement coupled with multiple antibody positivity is essential for a diagnosis. Renal involvement is frequent, clinically and histologically heterogeneous, and potentially serious.

Polyarthritis and pancreatic panniculitis associated with pancreatic carcinoma: review of the literature.

Pancreatic disorders, such as chronic or acute pancreatitis, and carcinoma may be infrequently accompanied or preceded by panniculitis or polyarthritis. This triad is known in the literature as the pancreatitis, panniculitis, and polyarthritis syndrome. Although the pancreatic disease of pancreatitis, panniculitis, and polyarthritis syndrome usually includes pancreatitis, here we review the literature with report of 1 additional case of polyarthritis and panniculitis occurring in the presence of pancreatic carcinoma. Given that the diagnosis is often difficult when abdominal symptoms are absent, knowledge of the association between panniculitis and polyarthritis with pancreatic disease may lead to a prompt diagnosis and management. The histopathology of the skin lesions can be a valuable clue for focusing attention to a pancreatic disease.

Identification of Levels of Serum Amyloid A and Apolipoprotein A1 in Serum Proteomic Analysis of Neuropsychiatric Systemic Lupus Erythematosus Patients.

Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) has multiple pathogenic mechanisms that cause diverse manifestations and whose diagnosis is challenging because of the absence of appropriate diagnostic tests. In the present study the application of proteomics using two-dimensional electrophoresis (2D) and mass spectrometry (MS) allowed the comparison of the protein profile of the serum low and high abundance protein fractions of NPSLE patients (NPSLE group) and SLE without neuropsychiatric syndromes (SLE group), Neuropsychiatric syndromes not associated with SLE (NPnoSLE groups), and healthy controls (CTRL group). The gels obtained were digitalized and analyzed with the PDQuest software. The statistical analysis of the spots was performed using the nonparametric Kruskal Wallis and Dunn's multiple comparison tests. Two spots showed significant differences and were identified by MS. Spot 4009 was significantly lower in NPSLE with regard to NPnoSLE (p= 0,004) and was identified as apolipoprotein A1 (APOA1) (score 809-1132). Spot 8001 was significantly higher in NPSLE regarding CTRL and NPnoSLE (p= 0,01 y 0,03, respectively) and was identified as serum amyloid A (SAA) (score 725-2488). The proinflammatory high density lipoproteins (HDL) have been described in SLE. In this HDL the decrease of APOA1 is followed by an increase in SAA. This altered level of both proteins may be related to the inflammatory state that is characteristic of an autoimmune disease like SLE, but this is not specific for NPSLE.

Hospital outcomes in patients evaluated by rheumatology in a Colombian historical cohort

Introduction: Hospitalized patients with systemic autoimmune rheumatic diseases (SARDs) generate high impact in clinical terms. Objectives: To characterize the study population and estimate risk factors associated with the presence of adverse outcomes in hospitalized patients consulting rheumatology at Clínica Imbanaco between January 2013 and December 2019. Methods: We analyzed a historical cohort of hospitalized patients who were evaluated by rheumatology. The population was classified as follows: group 1, patients with new onset diagnosed SARDs; group 2, patients with known diagnosed SARDs; group 3, patients without diagnosed SARDs; and group 4, patients with unconfirmed suspicion of SARDs. A composite adverse outcome was defined if at least one of the following occurred: (1) hospital mortality, (2) admission to the intensive care unit, (3) hospital infection, or (4) readmission. Results: Information was collected from 327 hospitalization events (307 patients). The median age was 48 (34-63) years and 222 (72.3%) were women. The composite adverse outcome occurred in 136 (41.5%) hospitalization events. Group 2 had the highest number of adverse outcomes (61/128; 47.6%). The variables associated with the worst outcomes were cardiovascular diagnosis at admission (OR = 4.63; CI: 1.60-13.43; p = 0.005), longer hospital stay (OR = 1.04; CI: 1.01-1.07; p = 0.005), and a treating specialty other than internal medicine (OR = 2.79; CI: 1.26-6.17; p = 0.011). Male sex (OR = 0.29; CI: 0.12-0.66; p = 0.004), having special health coverage (OR = 0.39; CI: 0.15-.099; p = 0.047), and hemoglobin > 11.4 g/dL (OR = 0.82; CI: 0.69-0.99; p = 0.039) were the factors associated with lower odds of developing the composite outcome. Conclusions: In this historical cohort, the group of patients with known diagnosed SARDs presented a higher number in percentage terms of adverse outcomes. The most frequent adverse outcomes were admission to the ICU and hospital readmission.

Introducción: Los pacientes hospitalizados con enfermedades reumáticas o autoinmunes sistémicas (ERAS) generan gran impacto en términos clínicos. Objetivos: Caracterizar a la población y estimar factores de riesgo asociados con la presencia de desenlaces adversos en pacientes evaluados hospitalariamente por reumatología en la Clínica Imbanaco durante los arios 2013-2019. Metodología: Se analizó una cohorte histórica de pacientes hospitalizados que fueron evaluados por reumatología. La población se clasificó así: grupo 1, pacientes con ERAS diagnosticada de novo; grupo 2, pacientes con ERAS diagnosticada conocida; grupo 3, pacientes sin ERAS diagnosticada; y grupo 4, pacientes con sospecha no confirmada de ERAS. Se definió un desenlace adverso compuesto si se presentó al menos uno de los siguientes casos: 1) mortalidad hospitalaria; 2) ingreso a la unidad de cuidado intensivo; 3) infección intrahospitalaria; 4) reingreso. Resultados: En un total de 327 eventos de hospitalización (307 pacientes), la mediana de edad fue 48 (34-63) años y 222 (72,3%) fueron mujeres. El desenlace adverso compuesto se presentó en 136 (41,5%) eventos. El grupo 2 tuvo mayor número de desenlaces adversos (61/128; 47,6%). Las variables asociadas con peores resultados fueron: diagnóstico inicial cardiovascular (OR = 4,63; IC: 1,60-13,43; p = 0,005), mayor estancia hospitalaria (OR = 1,04; IC: 1,01-1,07; p = 0,005) y tener una especialidad tratante diferente a medicina interna (OR = 2,79; IC: 1,266,17; p = 0,011). El sexo masculino (OR = 0,29; IC: 0,12-0,66; p = 0,004), pertenecer a un régimen especial de salud (OR = 0,39; IC: 0,15-0,99; p = 0,047) y tener hemoglobina > 11,4 g/dL (OR = 0,82; IC: 0,69-0,99; p = 0,039) fueron factores asociados con menor oportunidad de desarrollar el desenlace compuesto. Conclusiones: En esta cohorte histórica se encontró que porcentualmente el grupo de pacientes con ERAS diagnosticadas conocidas presentó mayor número de desenlaces adversos, entre los que se destacan para el mismo grupo, el ingreso a UCI y el reingreso hospitalario.

Características clínico-radiológicas de la hiperostosis esquelética idiopática difusa en 2 centros médicos de Cali, Colombia: reporte de 24 casos / Clinical and radiological characteristics of diffuse idiopathic skeletal hyperostosis in two medical centers in Cali, Colombia: Report of 24 cases

RESUMEN Introducción: La hiperostosis esquelética idiopática difusa (DISH, por sus siglas en inglés) es una afección caracterizada por la calcificación y la osificación progresiva de los ligamentos y las entesis. La mayoría de los pacientes permanecen asintomáticos hasta etapas avanzadas de la enfermedad, donde la limitación y el dolor son característicos. Objetivo: Describir las características demográficas, clínicas y radiológicas de los pacientes con DISH evaluados en el Centro Médico Imbanaco de Cali y en la Clínica de Artritis Temprana, en Cali, Colombia. Materiales y métodos: Es un estudio descriptivo, de corte transversal. Se revisaron los registros de pacientes diagnosticados con DISH, seguidos entre enero de 2000 y octubre de 2018. El diagnóstico se confirmó según los criterios de Resnick-Niwayama. Se encontraron 24 pacientes, todos se incluyeron para el análisis final. Resultados y discusión: En esta serie se encontraron 20 varones y 4 mujeres. La mediana de edad al diagnóstico fue de 70,5 arios (RIQ: 61,3-73,8 arios), siendo menor en las mujeres (71,5 versus 60 años; p = 0,04). La mediana de tiempo de evolución de los síntomas fue de 5 años (RIQ: 3-10 años), la duración fue menor en el grupo de las mujeres (5 versus 4 años; p = 0,20). El 54,2% tenían sobrepeso y el 20,8% eran diabéticos. El síntoma principal fue la limitación cervical. Los segmentos vertebrales (C: cervical; T: torácico; L: lumbar) más afectados por la osificación del ligamento longitudinal anterior (LLA) fueron C5-C6, T8-T10 y L1-L3. La afección periférica predominó en las crestas ilíacas. Todos los pacientes realizaron terapia física y 3 fueron sometidos a cirugía cervical. El grado de limitación funcional fue valorado en 19 pacientes a través de los cuestionarios modified Health Assessment Questionnaire (mHAQ) y Bath Ankylosing Spondylitis Functional Index (BASFI). La mediana del puntaje de ambos cuestionarios fue 2 veces más alta en las mujeres. Conclusión: La DISH fue más frecuente en varones mayores de 65 años y se asoció con enfermedades metabólicas como la obesidad y la diabetes. Aunque las diferencias no fueron significativas, los resultados sugieren que las mujeres presentan un fenotipo grave de la enfermedad explicado por el inicio temprano y curso progresivo de los síntomas, así como mayor limitación funcional medida por mHAQ y BASFI.

ABSTRACT Introduction: Diffuse idiopathic skeletal hyperostosis (DISH) is a condition characterised by calcification and progressive ossification of ligaments and entheses. Most patients remain asymptomatic until advanced stages of the disease, where limitation and pain are characteristic. Objective: To describe the demographic, clinical, and radiological characteristics of patients with DISH evaluated in the Centro Médico Imbanaco and Clínica de Artritis Temprana, in Cali, Colombia. Materials and methods: A descriptive, cross-sectional study was conducted by reviewing the records of patients diagnosed with DISH between January 2000 and October 2018. The diagnosis was confirmed according to the Resnick-Niwayama criteria. A total of 24 patients were found, and all were included for the final analysis. Results and discussion: The series included 20 men and 4 women, with a median age at diagnosis of 70.5 years (IQR 61.3-73.8 years), beinglower in women (71.5 versus 60 years; P=.04). The median time of onset of the symptoms was 5 years (IQR 3-10 years), and the duration was shorter in women (5 versus 4 years; P=.20). It was observed that 54% were overweight and 20% were diabetic. The main symptom was cervical limitation. The most affected vertebral segments due to the ossification of the anterior longitudinal ligament (ALL) were C5-C6, T8-T10 and L1-L3 (cervical C; thoracic T; lumbar L). The peripheral involvement was mainly in the iliac crests. All patients received physiotherapy, and three of them underwent cervical surgery. The degree of functional limitation was assessed in 19 patients using the mHAQ (Modified Health Assessment Questionnaire) and BASFI (Functional Ankylosing Spondylitis Functional (BASFI) questionnaires. The median score of both questionnaires was 2 times higher in women Conclusion: Diffuse idiopathic skeletal hyperostosis was more frequent in men over 65 years of age, and was associated with metabolic conditions such as obesity and diabetes. Although the differences were not significant, the results suggest that women have a more severe phenotype of the disease, explained by the early onset and progressive course of symptoms, as well as greater functional limitation measured by mHAQ and BASFI.

Comportamiento de los embarazos de mujeres con enfermedades reumáticas en la Clínica de Artritis Temprana de Cali: 2011-2017 / Behavior of pregnancies in women with rheumatic diseases in Cali early arthritis Clinic: 2011-2017

Introducción y objetivo: La respuesta inmune en el embarazo varía de acuerdo a la enfermedad reumática presente. Describir los embarazos de mujeres con enfermedades reumáticas en una Clínica de Artritis Temprana. Métodos: Estudio descriptivo. Se revisaron registros de pacientes embarazadas atendidas desde agosto/2011 hasta noviembre/2017. Se encontraron 42 pacientes, 14 sin una entidad reumática definida (solo ANA positivos: se excluyeron). Veintiocho presentaban una enfermedad reumática autoinmune y tuvieron 29 embarazos (2 gemelares). Se escogieron para el análisis final. Solo tres se perdieron. Resultados: El promedio de edad fue 28.2 años. Los diagnósticos fueron: lupus eritematoso sistémico (13), artritis reumatoide (6), síndrome antifosfolipídico (2), anemia hemolítica autoinmune (2), artritis idiopática juvenil (2),

Historia del tratamiento de las vasculitis primarias / History of treatment of primary vasculitis

(AU)Las vasculitis primarias constituyen un grupo de enfermedades reumáticas con expresión clínicavariable y pronóstico reservado cuando no se tratan adecuadamente. En esta revisión haremosun análisis pormenorizado del tratamiento en las diferentes formas de vasculitis primaria, iniciandocon el uso de los corticoides, desde casi su descubrimiento en 1949, pasando por otrosinmunosupresores como: ciclofosfamida, metotrexate, azatioprina, mofetil, micofenolato, aligual que medicamentos biológicos como rituximab y anti-TNF. Una mención especial se hacesobre las guías de tratamientos para las vasculitis, tanto de grandes como de pequeños vasos,implementadas desde 2009 por el Grupo Europeo de Estudio de las Vasculitis

Poliangiítis microscópica: reporte de un caso con enfermedad renal, pulmonar y neurológica, y revisión de la literatura / Microscopic Polyangiitis: a case report with renal, pulmonary and neurological involvement and review of the literature

Informamos el caso de un hombre de 79 años con hemorragia alveolar, glomerulonefritis necrosante y polineuropatía por poliangiítis microscópica, una enfermedad autoinmune, caracterizada por vasculitis sistémica, predominantemente de vasos de pequeño calibre. Se revisan los aspectos clínicos y terapéuticos de la enfermedad.

We report the case of a 79 years-old male, with alveolar hemorrhage, necrotizing glomerulonephritis and polyneuropathy due to microscopic polyangiitis, an autoimmune disease, characterized by systemic vasculitis, predominantly of small vessels. We review the clinical and therapeutic aspects of the disease.