Patrón gammagráfico “superscan†en la osteomalacia hipofosfatémica asociada a Tenofovir en un paciente con infección por VIH.

El tratamiento antirretroviral de gran actividad (TARGA) constituye uno de los avances más importantes en el manejo de los pacientes con infección por el virus de la inmunodeficiencia humana (VIH). Sin embargo, su uso a largo plazo conlleva el riesgo de aparición de enfermedades óseas como la osteporosis y la osteonecrosis. Uno de los fármacos más utilizados dentro de los esquemas de TARGA es el tenofovir disoproxil fumarato (TDF), un análogo de nucleótido inhibidor de la transcriptasa inversa que tiene buen perfil de seguridad y tolerancia, pero que ha sido asociado con el desarrollo de osteomalacia hipofosfatémica (OMH). Presentamos el caso de un varón de 56 años con infección crónica por VIH que desarrolló dolor óseo generalizado y debilidad durante el tratamiento con TDF, detectándose en el estudio analítico hipofosfatemia y aumento de su nivel basal de fosfatasa alcalina, con fosfaturia y calciuria normales. La radiografía mostraba aplastamientos vertebrales dorsales y la gammagrafía ósea reveló una hipercaptación difusa compatible con patrón “superscan†metabólico. Este patrón ha sido descrito de forma infrecuente en la OMH asociada a TDF, por lo que realizamos una revisión de los casos previamente publicados. Â

Application and performance of disease activity indices proposed for patients with systemic sclerosis in an international cohort of patients with juvenile systemic sclerosis

Funding Information: The author(s) disclosed receipt of the following financial support for the research, authorship and/or publication of this article: the inception cohort project is supported by an unrestricted grant from the Joachim Herz Stiftung, Hamburg, Germany. Publisher Copyright: © The Author(s) 2023.Objectives: Juvenile systemic sclerosis is a rare childhood disease. Three disease activity indices have been published for adult patients with systemic sclerosis: the European Scleroderma Study Group Index, a modified version of the European Scleroderma Study Group Index and the revised European Scleroderma Trials and Research index. The objective of this study was to determine the feasibility and performance of the three disease activity indices in a prospectively followed cohort of patients with juvenile systemic sclerosis. Methods: The analysis cohort was selected from the prospective international inception cohort enrolling juvenile systemic sclerosis patients. The correlation of the disease activity indices with the physicians’ and the patients’ global assessment of disease activity was determined. The disease activity indices were compared between patients with active and inactive disease. Sensitivity to change between 6- and 12-month follow-up was investigated by mixed models. Results: Eighty percent of the 70 patients had a diffuse cutaneous subtype. The revised European Scleroderma Trials and Research index was highly correlated with the physician-reported global disease activity/parents-reported global disease activity (r = 0.74/0.64), followed by the European Scleroderma Study Group activity index (r = 0.61/0.55) and the modified version of the European Scleroderma Study Group activity index (r = 0.51/0.43). The disease activity indices significantly differed between active and inactive patients. The disease activity indices showed sensitivity to change between 6- and 12-month follow-up among patients who improved or worsened according to the physician-reported global disease activity and the parents-reported global disease activity. Conclusion: Overall, no disease activity score is superior to the other, and all three scores have limitations in the application in juvenile systemic sclerosis patients. Furthermore, research on the concept of disease activity and suitable scores to measure disease activity in patients with juvenile systemic sclerosis is necessary in future.Peer reviewe

Efficacy of Anakinra in Refractory Adult-Onset Still's Disease: Multicenter Study of 41 Patients and Literature Review

Adult-onset Still's disease (AOSD) is often refractory to standard therapy. Anakinra (ANK), an interleukin-1 receptor antagonist, has demonstrated efficacy in single cases and small series of AOSD. We assessed the efficacy of ANK in a series of AOSD patients. Multicenter retrospective open-label study. ANK was used due to lack of efficacy to standard synthetic immunosuppressive drugs and in some cases also to at least 1 biologic agent. Forty-one patients (26 women/15 men) were recruited. They had a mean age of 34.4 ± 14 years and a median [interquartile range (IQR)] AOSD duration of 3.5 [2-6] years before ANK onset. At that time the most common clinical features were joint manifestations 87.8%, fever 78%, and cutaneous rash 58.5%. ANK yielded rapid and maintained clinical and laboratory improvement. After 1 year of therapy, the frequency of joint and cutaneous manifestations had decreased to 41.5% and to 7.3% respectively, fever from 78% to 14.6%, anemia from 56.1% to 9.8%, and lymphadenopathy from 26.8% to 4.9%. A dramatic improvement of laboratory parameters was also achieved. The median [IQR] prednisone dose was also reduced from 20 [11.3-47.5] mg/day at ANK onset to 5 [0-10] at 12 months. After a median [IQR] follow-up of 16 [5-50] months, the most important side effects were cutaneous manifestations (n = 8), mild leukopenia (n = 3), myopathy (n = 1), and infections (n = 5). ANK is associated with rapid and maintained clinical and laboratory improvement, even in nonresponders to other biologic agents. However, joint manifestations are more refractory than the systemic manifestations

DIFFUSE JUVENILE SYSTEMIC SCLEROSIS PATIENTS SHOW DISTINCT ORGAN INVOLVEMENT, ANTIBODY PATTERN AND HAVE SIGNIFICANTLY MORE SEVERE DISEASE IN THE LARGEST JSSC COHORT OF THE WORLD. RESULTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT

Peer reviewe

Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum TimeCourse

Introduction: Increased cardiovascular (CV) morbidity and mortality is observed in inflammatory joint diseases (IJDs) such as rheumatoid arthritis, ankylosing spondylitis, and psoriatic arthritis. However, the management of CV disease in these conditions is far from being well established.Areas covered: This review summarizes the main epidemiologic, pathophysiological, and clinical risk factors of CV disease associated with IJDs. Less common aspects on early diagnosis and risk stratification of the CV disease in these conditions are also discussed. In Europe, the most commonly used risk algorithm in patients with IJDs is the modified SCORE index based on the revised recommendations proposed by the EULAR task force in 2017.Expert opinion: Early identification of IJD patients at high risk of CV disease is essential. It should include the use of complementary noninvasive imaging techniques. A multidisciplinary approach aimed to improve heart-healthy habits, including strict control of classic CV risk factors is crucial. Adequate management of the underlying IJD is also of main importance since the reduction of disease activity decreases the risk of CV events. Non-steroidal anti-inflammatory drugs may have a lesser harmful effect in IJD than in the general population, due to their anti-inflammatory effects along with other potential beneficial effects.This research was partially funded by FOREUM—Foundation for Research in Rheumatolog

Atenciones debidas a patología reumatológica en el Servicio de Emergencias del Instituto Nacional de Salud del Niño (Lima, Perú) durante el periodo Enero 2012-Junio 2014

Objetivos: Evaluar las causas más frecuentes de atenciones por patología reumatológica en el servicio de emergencias de un hospital pediátrico peruano de tercer nivel.Pacientes y Métodos: Se llevó a cabo un estudio descriptivo, observacional y retrospectivo mediante la revisión de la base de datos y registros del servicio de emergencias del hospital, correspondientes al periodo comprendido entre enero 2012 y junio 2014, seleccionándose las atenciones debidas a patología reumatológica. Los datos fueron procesados mediante el programa estadístico SPSS 16.0.Resultados: Durante el periodo evaluado el número total de atenciones en el servicio de emergencias fue de 133484, correspondiendo 835 casos (0,63%) a diagnósticos reumatológicos segúnla Clasificación Internacionalde Enfermedades 10° (CIE-10). La mayoría de los pacientes fueron hombres (450, 53,08%) y la distribución por grupos etáreos fue: 1-4 años 327 (39,16%), 5-9 años 251 (30,02%), 10-14 años 158 (18,98%), mayores de 15 años 68 (8,11%) y menores de un año 32 (3,77%). Las 5 primeras causas de atenciones reumatológicas, de acuerdo al CIE-10, durante este período fueron: artritis reactiva 173 casos (20,72%), dolor en articulación 168 (20,12%), púrpura de Schönlein-Henoch (púrpura alérgica) 107 (12,81%), artritis séptica (artritis piógena) 89 (10,67%) y mialgias 77 (9,22%). El número de atenciones por problemas reumatológicos se mantuvo estable a través del tiempo, siendo de325 a345 casos por año.Conclusiones: Las atenciones debidas a patología reumatológica en el servicio de emergencias de un hospital pediátrico terciario como el nuestro son frecuentes y estables durante el transcurso del tiempo. Una adecuada evaluación inicial y un apropiado periodo de seguimiento aseguran un correcto diagnóstico y un tratamiento eficaz. Los pediatras que trabajan en el área de emergencias deberían estar capacitados en reumatología pediátrica

Persistence and adverse events of biological treatment in adult patients with juvenile idiopathic arthritis: results from BIOBADASER

Abstract Background Biologic therapy has changed the prognosis of patients with juvenile idiopathic arthritis (JIA). The aim of this study was to examine the pattern of use, drug survival, and adverse events of biologics in patients with JIA during the period from diagnosis to adulthood. Methods All patients included in BIOBADASER (Spanish Registry for Adverse Events of Biological Therapy in Rheumatic Diseases), a multicenter prospective registry, diagnosed with JIA between 2000 and 2015 were analyzed. Proportions, means, and SDs were used to describe the population. Incidence rates and 95% CIs were calculated to assess adverse events. Kaplan-Meier analysis was used to compare the drug survival rates. Results A total of 469 patients (46.1% women) were included. Their mean age at diagnosis was 9.4 ± 5.3 years. Their mean age at biologic treatment initiation was 23.9 ± 13.9 years. The pattern of use of biologics during their pediatric years showed a linear increase from 24% in 2000 to 65% in 2014. Biologic withdrawal for disease remission was higher in patients who initiated use biologics prior to 16 years of age than in those who were older (25.7% vs 7.9%, p < 0.0001). Serious adverse events had a total incidence rate of 41.4 (35.2–48.7) of 1000 patient-years. Patients younger than 16 years old showed significantly increased infections (p < 0.001). Conclusions Survival and suspension by remission of biologics were higher when these compounds were initiated in patients with JIA who had not yet reached 16 years of age. The incidence rate of serious adverse events in pediatric vs adult patients with JIA treated with biologics was similar; however, a significant increase of infection was observed in patients under 16 years old

Are diffuse and limited juvenile systemic sclerosis different in clinical presentation? Clinical characteristics of a juvenile systemic sclerosis cohort

Introduction: Juvenile systemic sclerosis is an orphan disease. Currently, the majority of juvenile systemic sclerosis cohort studies are retrospective in design without standardized assessment. This study was conducted prospectively to investigate the difference in manifestations of limited cutaneous juvenile systemic sclerosis and diffuse cutaneous juvenile systemic sclerosis subtypes. An additional aim was to compare these data to other juvenile systemic sclerosis cohorts and a large adult systemic sclerosis cohort. Methods: Patients fulfilling the Paediatric Rheumatology European Society juvenile systemic sclerosis classification criteria were included. Clinical characteristics and patient-related outcomes were assessed. Results: In all, 88 patients with a mean disease duration of 3.5 years were enrolled, 72.5% with diffuse cutaneous juvenile systemic sclerosis with a mean modified Rodnan Skin score of 18 and 27.5% with limited cutaneous juvenile systemic sclerosis with mean modified Rodnan Skin score of 9. The mean age at the onset of Raynaud's and first non-Raynaud's symptoms was similar in both groups, approximately 9 and 10.5 years. Active digital tip ulcerations were present in 29% diffuse cutaneous juvenile systemic sclerosis and none in the limited cutaneous juvenile systemic sclerosis subjects (p = 0.005). Of those with cardiopulmonary testing, 3% of diffuse cutaneous juvenile systemic sclerosis and 23% of limited cutaneous juvenile systemic sclerosis group had cardiac involvement (p = 0.015), and 41% diffuse cutaneous juvenile systemic sclerosis and 22% of the limited cutaneous juvenile systemic sclerosis group had pulmonary involvement (p = 0.009). Physician global disease damage assessment was higher in the diffuse cutaneous juvenile systemic sclerosis group compared to the limited cutaneous juvenile systemic sclerosis group: 35 and 15 (p = 0.021). Discussion: The majority of this international juvenile systemic sclerosis cohort had diffuse cutaneous juvenile systemic sclerosis (72.5%) with more frequent vascular and pulmonary involvement compared to the limited cutaneous group, who had increased cardiac involvement. Our cohort reflects prior findings of published juvenile systemic sclerosis cohorts and emphasizes a difference in the presentation compared to adult-onset systemic sclerosis