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

Clinical Spectrum Time Course in Anti Jo-1 Positive Antisynthetase Syndrome

Anti Jo-1 antibodies are the main markers of the antisynthetase syndrome (ASSD), an autoimmune disease clinically characterized by the occurrence of arthritis, myositis, and interstitial lung disease (ILD). These manifestations usually co-occur (for practical purpose complete forms) in the same patient, but cases with only 1 or 2 of these findings (for practical purpose incomplete forms) have been described. In incomplete forms, the ex novo occurrence of further manifestations is possible, although with frequencies and timing not still defined. The aim of this international, multicenter, retrospective study was to characterize the clinical time course of anti Jo-1 positive ASSD in a large cohort of patients. Included patients should be anti Jo-1 positive and with at least 1 feature between arthritis, myositis, and ILD. We evaluated the differences between complete and incomplete forms, timing of clinical picture appearance and analyzed factors predicting the appearance of further manifestations in incomplete ASSD. Finally, we collected 225 patients (58 males and 167 females) with a median follow-up of 80 months. At the onset, complete ASSD were 44 and incomplete 181. Patients with incomplete ASSD had frequently only 1 of the classic triad findings (110 cases), in particular, isolated arthritis in 54 cases, isolated myositis in 28 cases, and isolated ILD in 28 cases. At the end of follow-up, complete ASSD were 113, incomplete 112. Only 5 patients had an isolated arthritis, only 5 an isolated myositis, and 15 an isolated ILD. During the follow-up, 108 patients with incomplete forms developed further manifestations. Single main feature onset was the main risk factor for the ex novo appearance of further manifestation. ILD was the prevalent ex novo manifestation (74 cases). In conclusion, ASSD is a condition that should be carefully considered in all patients presenting with arthritis, myositis, and ILD, even when isolated. The ex novo appearance of further manifestations in patients with incomplete forms is common, thus indicating the need for an adequate clinical and instrumental follow-up. Furthermore, the study clearly suggested that in ASSD multidisciplinary approach involving Rheumatology, Neurology, Pneumology, and Internal Medicine specialists is mandatory

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