Executive summary: Guidelines for the diagnosis and treatment of septic arthritis in adults and children, developed by the GEIO (SEIMC), SEIP and SECOT / Resumen ejecutivo: Guía de diagnóstico y tratamiento de la artritis séptica en adultos y niños de GEIO (SEIMC), SEIP y SECOT

Infection of a native joint, commonly referred to as septic arthritis, is a medical emergency because of the risk of joint destruction and subsequent sequelae. Its diagnosis requires a high level of suspicion. These guidelines for the diagnosis and treatment of septic arthritis in children and adults are intended for use by any physician caring for patients with suspected or confirmed septic arthritis. They have been developed by a multidisciplinary panel with representatives from the Bone and Joint Infections Study Group (GEIO) belonging to the Spanish Society of Infectious Diseases and Clinical Microbiology (SEIMC), the Spanish Society of Paediatric Infections (SEIP) and the Spanish Society of Orthopaedic Surgery and Traumatology (SECOT), and two rheumatologists. The recommendations are based on evidence derived from a systematic literature review and, failing that, on the opinion of the experts who prepared these guidelines. A detailed description of the background, methods, summary of evidence, the rationale supporting each recommendation, and gaps in knowledge can be found online in the complete document.(AU)

La infección de una articulación nativa, generalmente denominada artritis séptica, constituye una urgencia médica por el riesgo de destrucción articular y las consecuentes secuelas. Su diagnóstico requiere un alto nivel de sospecha. Esta guía de diagnóstico y tratamiento de la artritis séptica en niños y adultos está destinada a cualquier médico que atienda pacientes con sospecha de artritis séptica o artritis séptica confirmada. La guía ha sido elaborada por un panel multidisciplinar en el que están representados el Grupo de Estudio de Infecciones Osteoarticulares (GEIO) de la Sociedad Española de Enfermedades Infecciosas y Microbiología Clínica (SEIMC), la Sociedad Española de Infectología Pediátrica (SEIP) y la Sociedad Española de Cirugía Ortopédica y Traumatología (SECOT); además han participado dos reumatólogos. Las recomendaciones se basan en la evidencia proporcionada por una revisión sistemática de la literatura y, en su defecto, en la opinión de los expertos que han elaborado la presente guía. En el texto completo online se hace una descripción detallada de los antecedentes, métodos, resumen de la evidencia, fundamentos que apoyan cada recomendación y las lagunas de conocimiento existentes.(AU)

Real-world apremilast use in biologic-naïve psoriatic arthritis patients. Data from Spanish clinical practice.

INTRODUCTION: Apremilast is approved for treatment of psoriasis and psoriatic arthritis (PsA). Real-world evidence on apremilast effectiveness in clinical practice is limited. METHODS: Observational study enrolling adult patients, across 21 Spanish centres, who had initiated apremilast in the prior 6 (±1) months and were biologic naive. Data were collected at routine follow-up visits 6 and 12 months after apremilast initiation. Primary outcome was 6 and 12-month persistence to apremilast. Secondary outcomes included Disease Activity for PsA (DAPSA), joint erosions, enthesitis, dactylitis, and patient-reported quality of life (QoL, measured using the PsA impact of disease [PsAID] questionnaire). RESULTS: We included 59 patients. Most had oligoarticular PsA, moderate disease activity, and high comorbidity burden. Three-quarters were continuing apremilast at 6 months and two-thirds at 12 months; mean (SD) apremilast treatment duration was 9.43 (1.75) months. DAPSA scores showed improved disease activity: one-third of patients in remission or low activity at apremilast initiation versus 62% and 78% at 6 and 12 months, respectively. Eleven of 46 patients with radiographic assessments had joint erosions at apremilast initiation and none at month 12. Median (Q1, Q3) number of swollen joints was 4.0 (2.0, 6.0) at apremilast initiation versus 0.0 (0.0, 2.0) at 12 months. Incidence of dactylitis and enthesitis decreased between apremilast initiation (35.6% and 28.8%, respectively) and month 12 (11.6% and 2.4%, respectively). Over two-thirds of patients had a PSAID-9 score <4 (cut-off for patient-acceptable symptom state) at month 12. CONCLUSIONS: In Spanish clinical practice, two-thirds of PsA patients continued apremilast at 12 months, with clinical benefits at the joint level, no radiographic progression of erosions, and a positive impact on patient-reported QoL. Trial registration number Clinicaltrials.gov: NCT03828045.

Real-world apremilast use in biologic-naïve psoriatic arthritis patients. Data from Spanish clinical practice / Uso real de apremilast en pacientes con artritis psoriásica sin tratamiento biológico. Datos de la práctica clínica española

Introduction: Apremilast is approved for treatment of psoriasis and psoriatic arthritis (PsA). Real-world evidence on apremilast effectiveness in clinical practice is limited. Methods: Observational study enrolling adult patients, across 21 Spanish centres, who had initiated apremilast in the prior 6 (±1) months and were biologic naive. Data were collected at routine follow-up visits 6 and 12 months after apremilast initiation. Primary outcome was 6 and 12-month persistence to apremilast. Secondary outcomes included Disease Activity for PsA (DAPSA), joint erosions, enthesitis, dactylitis, and patient-reported quality of life (QoL, measured using the PsA impact of disease [PsAID] questionnaire). Results: We included 59 patients. Most had oligoarticular PsA, moderate disease activity, and high comorbidity burden. Three-quarters were continuing apremilast at 6 months and two-thirds at 12 months; mean (SD) apremilast treatment duration was 9.43 (1.75) months. DAPSA scores showed improved disease activity: one-third of patients in remission or low activity at apremilast initiation versus 62% and 78% at 6 and 12 months, respectively. Eleven of 46 patients with radiographic assessments had joint erosions at apremilast initiation and none at month 12. Median (Q1, Q3) number of swollen joints was 4.0 (2.0, 6.0) at apremilast initiation versus 0.0 (0.0, 2.0) at 12 months. Incidence of dactylitis and enthesitis decreased between apremilast initiation (35.6% and 28.8%, respectively) and month 12 (11.6% and 2.4%, respectively). Over two-thirds of patients had a PSAID-9 score <4 (cut-off for patient-acceptable symptom state) at month 12. Conclusions: In Spanish clinical practice, two-thirds of PsA patients continued apremilast at 12 months, with clinical benefits at the joint level, no radiographic progression of erosions, and a positive impact on patient-reported QoL.(AU)

Introducción: Apremilast está aprobado para el tratamiento de la psoriasis y la artritis psoriásica (APs). La evidencia sobre la efectividad de apremilast en la práctica clínica es limitada. Métodos: Estudio observacional en el que se incluyó a pacientes adultos, de 21 centros españoles, que habían iniciado apremilast en los 6 (± 1) meses previos y no habían recibido biológicos. Los datos se recogieron en visitas rutinarias de seguimiento a los 6 y 12 meses del inicio de apremilast. El objetivo primario fue la persistencia de apremilast a los 6 y 12 meses. Los objetivos secundarios incluyeron la actividad de la enfermedad para APs (DAPSA), erosiones articulares, entesitis, dactilitis y la calidad de vida informada por el paciente (CdV, medida mediante el cuestionario “PsA Impact of disease [PsAID]”). Resultados: Se incluyó a 59 pacientes. La mayoría presentaba APs oligoarticular, actividad moderada de la enfermedad y alta comorbilidad. Tres cuartas partes continuaban con apremilast a los 6 meses y 2 tercios a los 12 meses; la duración media (DE) del tratamiento con apremilast fue de 9,43 (1,75) meses. Las puntuaciones DAPSA mostraron una mejora de la actividad de la enfermedad: un tercio de los pacientes en remisión o baja actividad al inicio de apremilast frente al 62 y el 78% a los 6 y 12 meses, respectivamente. Once de 46 pacientes con evaluaciones radiográficas presentaban erosiones articulares al inicio de apremilast y ninguno en el mes 12. La mediana (Q1, Q3) del número de articulaciones inflamadas fue de 4,0 (2,0, 6,0) al inicio de apremilast frente a 0,0 (0,0, 2,0) a los 12 meses. La incidencia de dactilitis y la entesitis disminuyeron entre el inicio de apremilast (el 35,6 y el 28,8%, respectivamente) y el mes 12 (el 11,6 y el 2,4%, respectivamente). Más de 2 tercios de los pacientes tenían una puntuación PSAID-9 < 4 (punto de corte del estado sintomático aceptable para el paciente) en el mes 12.(AU)

Executive summary: Guidelines for the diagnosis and treatment of septic arthritis in adults and children, developed by the GEIO (SEIMC), SEIP and SECOT.

Infection of a native joint, commonly referred to as septic arthritis, is a medical emergency because of the risk of joint destruction and subsequent sequelae. Its diagnosis requires a high level of suspicion. These guidelines for the diagnosis and treatment of septic arthritis in children and adults are intended for use by any physician caring for patients with suspected or confirmed septic arthritis. They have been developed by a multidisciplinary panel with representatives from the Bone and Joint Infections Study Group (GEIO) belonging to the Spanish Society of Infectious Diseases and Clinical Microbiology (SEIMC), the Spanish Society of Paediatric Infections (SEIP) and the Spanish Society of Orthopaedic Surgery and Traumatology (SECOT), and two rheumatologists. The recommendations are based on evidence derived from a systematic literature review and, failing that, on the opinion of the experts who prepared these guidelines. A detailed description of the background, methods, summary of evidence, the rationale supporting each recommendation, and gaps in knowledge can be found online in the complete document.

Assessment of liver fibrosis in patients with rheumatoid arthritis treated with methotrexate: Utility of fibroscan and biochemical markers in routine clinical practice.

OBJECTIVES: To study the prevalence of liver fibrosis (LF) measured by FibroScan and APRI index in patients with rheumatoid arthritis (AR) undergoing treatment with methotrexate (MTX). METHODS: We included 59 patients with RA on MTX. Medical records, FibroScan measures and serological markers of liver damage were compared on the basis of cumulative methotrexate dose. RESULTS: Mean treatment duration was 82.4±65.1 months and mean cumulative dose was 5214.5±4031.9mg. Five patients met LF criteria by fibroscan, while only one patient had a suggestive APRI score. No statistically significant differences were found in terms of LF measured by both APRI and fibroScan between patients with cumulative doses above and below 4000mg. There was also no relationship between LF and treatment duration. CONCLUSIONS: The occurrence of LF in patients with RA on MTX is a multifactorial process that does not seem directly related to its cumulative dose. FibroScan may be a useful technique in clinical practice to screen for this complication.

Coronavirus Disease 2019 in Rheumatic Patients with Inflammatory Disorders: A Descriptive Study from a High Infection Incidence Region of Northern Spain.

BACKGROUND: Since the first confirmed case of severe acute respiratory syndrome coronavirus 2 in Spain in January 2020, the susceptibility of patients with rheumatic disease has remained unclear. In this report, we will describe the main features of coronavirus disease 2019 (COVID-19) that occurred in rheumatic patients with inflammatory disorders and try to identify features associated with severe disease. METHODS: We included all rheumatic patients with immune-mediated diseases followed at 6 centers belonging to the public healthcare system in the Basque Country (Spain) and diagnosed with COVID-19 from March 1, 2020, to May 31, 2020. RESULTS: In total, 131 patients were included in this study. The most frequent rheumatic disease was rheumatoid arthritis (46.6%), and the main comorbidities were arterial hypertension (45%). Fortyseven percent were taking glucocorticoids (GC) (62 patients), 61.8% were under treatment with conventional synthetic disease-modifying antirheumatic drugs (csDMARD), and 25 patients (19.1%) were receiving targeted therapies (TT). Thirty-eight percent of patients required hospital admission, 2.3% required transfer to intensive care uni, and the rate of mortality was 9.2%. Associated factors in univariate analysis for a bad outcome were older age, use of GC, obesity, previous cardiovascular disease, and lymphopenia. Use of GC and lymphopenia remained within the multivariate model. CONCLUSION: The frequency of COVID-19 seems to be similar in rheumatic patients as in the general population. Advanced age, obesity, heart disease, glucocorticoids, and low levels of lymphocytes were more common among the patients with a bad outcome. Neither exposure to csDMARD nor TT was associated with severe cases.

Assessment of liver fibrosis in patients with rheumatoid arthritis treated with methotrexate: Utility of fibroscan and biochemical markers in routine clinical practice / Evaluación de la fibrosis hepática en pacientes con artritis reumatoide en tratamiento con metotrexato: utilidad en la práctica clínica habitual del FibroScan y marcadores bioquímicos

Objectives: To study the prevalence of liver fibrosis (LF) measured by FibroScan and APRI index in patients with rheumatoid arthritis (AR) undergoing treatment with methotrexate (MTX). Methods: We included 59 patients with RA on MTX. Medical records, FibroScan measures and serological markers of liver damage were compared on the basis of cumulative methotrexate dose. Results: Mean treatment duration was 82.4±65.1 months and mean cumulative dose was 5214.5±4031.9mg. Five patients met LF criteria by fibroscan, while only one patient had a suggestive APRI score. No statistically significant differences were found in terms of LF measured by both APRI and fibroScan between patients with cumulative doses above and below 4000mg. There was also no relationship between LF and treatment duration. Conclusions: The occurrence of LF in patients with RA on MTX is a multifactorial process that does not seem directly related to its cumulative dose. FibroScan may be a useful technique in clinical practice to screen for this complication. (AU)

Objetivos: Estudiar la prevalencia de la fibrosis hepática (FH) medida por FibroScan e índice APRI en pacientes con artritis reumatoide (AR) en tratamiento con metotrexato (MTX). Métodos: Se incluyeron 59 pacientes con AR en tratamiento con MTX. Se compararon las historias clínicas, las mediciones de FibroScan y los marcadores serológicos de daño hepático en función de la dosis acumulada de MTX. Resultados: La duración media del tratamiento fue de 82,4±65,1 meses y la dosis media acumulada de 5214,5±4031,9mg. Cinco pacientes cumplían criterios de FH por FibroScan y un solo paciente por APRI. No se encontraron diferencias estadísticamente significativas en cuanto a FH tanto por APRI como por FibroScan en base a dosis acumuladas superiores o inferiores a 4000mg. Tampoco hubo relación entre FH y duración del tratamiento. Conclusiones: La FH en pacientes con AR tratados con MTX es un proceso multifactorial sin aparente relación directa con la dosis acumulada. El FibroScan puede ser una técnica útil en la práctica clínica para detectar esta complicación. (AU)

Síndrome de activación macrofágica secundario a enfermedades autoinmunes, hematológicas, infecciosas y oncológicas. Serie de 13 casos clínicos y una revisión bibliográfica / Secondary macrophage activation syndrome due to autoimmune, hematologic, infectious and oncologic diseases. Thirteen case series and review of the literature

Objetivo: Describir las características demográficas y trastornos de pacientes con diagnóstico de síndrome de activación macrofágica (SAM) en el periodo comprendido entre diciembre de 2008-enero de 2014. Métodos: Se revisaron las historias clínicas desde el diagnóstico de SAM y tras su alta hospitalaria hasta enero de 2014. Los pacientes se agruparon en 4 grupos: autoinmunes (AI), hemato-oncólogicas (HO), infecciosas (Inf) y oncológicas (Onc). Las variables fueron analizadas entre los 4 grupos y entre AI y HO. Resultados: Trece pacientes (7 hombres, con una mediana de 54 años [32-63]) se estudiaron. Las etiologías encontradas fueron: 5 AI, 5 HO, 2 Inf y una Onc. Se encontraron células hemofagocíticas en el líquido ascítico en uno de los pacientes. Se encontró un paciente con SAM secundario a enfermedad relacionada con la IgG4. Conclusiones: La mortalidad, el pronóstico y la evolución de la enfermedad puede verse influida por el retraso en el diagnóstico, el inicio del tratamiento y la etiología del SAM. Los pacientes con enfermedades HO presentaron peor pronóstico (AU)

Objective: Describe the demographic characteristics and disorders of patients with diagnosis of Macrophage Activation Syndrome (MAS) in the December 2008 - January 2014 period. Methods: Medical records were reviewed from diagnosis of MAS and after discharge until January 2014. Patients were divided into 4 groups according to the primary disease: Autoimmune (AI), Hemato - oncologic (HO), Infectious (Inf) and Oncologic (Onc). The variables were analyzed among the 4 groups and between AI and HO. Results: Thirteen patients [7 men, with a median of 54 years (32-63)] were studied. The etiologies were: 5 AI, 5 HO, 2 Inf. and 1 Onc. disease. Hemophagocitc cells were found in the ascitic fluid of one patient. A patient with MAS secondary to IgG4-related disease was found. Conclusions: Mortality, prognosis and disease progression may be influenced by the delay in diagnosis, treatment initiation and etiology of MAS. HO ill patients had a worse prognosis (AU)

Secondary macrophage activation syndrome due to autoimmune, hematologic, infectious and oncologic diseases. Thirteen case series and review of the literature.

OBJECTIVE: Describe the demographic characteristics and disorders of patients with diagnosis of Macrophage Activation Syndrome (MAS) in the December 2008 - January 2014 period. METHODS: Medical records were reviewed from diagnosis of MAS and after discharge until January 2014. Patients were divided into 4 groups according to the primary disease: Autoimmune (AI), Hemato - oncologic (HO), Infectious (Inf) and Oncologic (Onc). The variables were analyzed among the 4 groups and between AI and HO. RESULTS: Thirteen patients [7 men, with a median of 54 years (32-63)] were studied. The etiologies were: 5 AI, 5 HO, 2 Inf. and 1 Onc. disease. Hemophagocitc cells were found in the ascitic fluid of one patient. A patient with MAS secondary to IgG4-related disease was found. CONCLUSIONS: Mortality, prognosis and disease progression may be influenced by the delay in diagnosis, treatment initiation and etiology of MAS. HO ill patients had a worse prognosis.

Síndrome de activación macrofágica como complicación severa de la enfermedad de Still del adulto. Células hemofagocíticas en líquido ascítico / Macrophage activation syndrome as a severe manifestation of adult's still's disease. Hemophagocytic cells in ascites

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Síndrome hemofagocítico como manifestación clínica inicial del lupus eritematoso sistémico / Hemophagocytic syndrome as the initial manifestation of systemic lupus erythematosus

Introducción. El síndrome hemofagocítico (SH) se produce en enfermedades autoinmunes y pertenece al grupo de enfermedades linfohistiocitosis hemofagocítica. El presente trabajo describe las características de 2 pacientes con lupus eritematoso sistemico (LES) que comenzaron con SH. Observaciones clínicas. Ambos pacientes presentaron fiebre prolongada no asociada a proceso infeccioso y que no respondía a antibióticos. Discusión. El diagnóstico de SH secundario a LES es complicado, ya que presentan características comunes; sin embargo, el SH presenta hiperferritinemia, hipofibrinogenemia, hipertrigliceridemia y descenso de la velocidad de sedimentación globular a diferencia del LES. El tratamiento no está bien establecido, pero los corticoides y/o inmunoglobulinas son efectivos en el tratamiento inicial, y en casos refractarios la ciclosporina o la ciclofosfamida se pueden asociar. Conclusiones. El SH puede ser la manifestación inicial del LES y debe sospecharse en pacientes con organomegalias, citopenias, trastornos en la coagulación, alteraciones hepáticas y fiebre prolongada que no responde a antibióticos. Anakinra puede ser una opción de tratamiento en el SH secundario al LES adulto (AU)

Introduction: Hemophagocytic syndrome (HS) occurs in autoimmune diseases and belongs o the hemophagocytic lymphohistiocytosis group of diseases. This paper describes the features of 2 patients with systemic lupus erythematosus (SLE) who presented HS as the initial clinical manifestation. Clinical observations: Both patients had prolonged fever not associated to an infectious process and did not respond to broad-spectrum antibiotics. Discussion: The diagnosis of HS secondary to SLE is complicated, because it has some features in common, but HS is characterized by hyperferritinemia, hipofibrinogemia, hypertriglyceridemia and a decrease in the erythrocyte sedimentation rate, unlike SLE. HS treatment when associated to SLE is not well established, but steroids and/or immunoglobulins are effective as the initial treatment, and in refractory cases, cyclosporine or cyclophosphamide may be associated. Conclusions: HS can be the initial manifestation of SLE and should be suspected in patients with organ enlargement, cytopenias, clotting disorders, liver disorders and prolonged fever unresponsive to antibiotics. Anakinra may be a treatment option in adult HS associated to SLE (AU)

Infección protésica por Mycobacterium tuberculosis en paciente con artritis reumatoide: reporte de un caso y revisión bibliográfica / Prosthesis infection by Mycobacterium tuberculosis in a patient with rheumatoid arthritis: a case report and literature review

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Hemophagocytic syndrome as the initial manifestation of systemic lupus erythematosus.

INTRODUCTION: Hemophagocytic syndrome (HS) occurs in autoimmune diseases and belongs to the hemophagocytic lymphohistiocytosis group of diseases. This paper describes the features of 2 patients with systemic lupus erythematosus (SLE) who presented HS as the initial clinical manifestation. CLINICAL OBSERVATIONS: Both patients had prolonged fever not associated to an infectious process and did not respond to broad-spectrum antibiotics. DISCUSSION: The diagnosis of HS secondary to SLE is complicated, because it has some features in common, but HS is characterized by hyperferritinemia, hipofibrinogemia, hypertriglyceridemia and a decrease in the erythrocyte sedimentation rate, unlike SLE. HS treatment when associated to SLE is not well established, but steroids and/or immunoglobulins are effective as the initial treatment, and in refractory cases, cyclosporine or cyclophosphamide may be associated. CONCLUSIONS: HS can be the initial manifestation of SLE and should be suspected in patients with organ enlargement, cytopenias, clotting disorders, liver disorders and prolonged fever unresponsive to antibiotics. Anakinra may be a treatment option in adult HS associated to SLE.

Mielitis transversa y neuritis óptica bilateral en una paciente con lupus eritematoso sistémico / Transverse myelitis and bilateral optic neuritis in a patient with systemic lupus erythematosus

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Disfagia por anillos esofágicos en el síndrome de Sjögren / Dysphagia caused by oesophageal webs in a patient with primary Sjögren's syndrome

Se presenta un caso de disfagia en una mujer con síndrome de Sjögren primario. Los exámenes complementarios demostraron la presencia de dos anillos que constreñían la luz esofágica. Mediante técnicas intervencionistas se consiguió superar dichas constricciones. La paciente permanece asintomática después de 3 años de seguimiento. Se comentan los posibles mecanismos patogénicos de la disfagia en el síndrome de Sjögren (AU)