Position statement on infection screening, prophylaxis, and vaccination in pediatric patients with rheumatic diseases and immunosuppressive therapies, part 2: infection prophylaxis.

This study aims to provide practical recommendations on prophylaxis for infection in pediatric patients with immune-mediated rheumatic diseases receiving/scheduled to receive immunosuppressive therapy. A qualitative approach was applied. A narrative literature review was performed via Medline. Primary searches were conducted using MeSH terms and free text to identify articles that analyzed data on infections and vaccinations in pediatric patients with immune-mediated rheumatic diseases receiving immunosuppressive therapy. The results were presented and discussed in a nominal group meeting comprising a committee of 12 pediatric rheumatologists from the Prevention and Treatment of Infections Working Group of the Spanish Society of Pediatric Rheumatology. Several recommendations were generated. A consensus procedure was implemented via a Delphi process that was extended to members of the Spanish Society of Pediatric Rheumatology and the Vaccine Advisory Committee of the Spanish Association of Pediatrics. Participants produced a score ranging from 0 (completely disagree) to 10 (completely agree). Agreement was considered to have been reached if at least 70% of participants voted ≥ 7. The literature review included more than 400 articles. Overall, 63 recommendations were generated (23 on infection prophylaxis) and voted by 59 pediatric rheumatologists and other pediatric specialists, all of whom achieved the pre-established level of agreement. The recommendations on prophylaxis of infection cover vaccination and prophylaxis against varicella zoster virus, tuberculosis, Pneumocystis jiroveccii, and invasive fungal infections in pediatric patients with immune-mediated rheumatic diseases receiving/scheduled to receive immunosuppressive therapy.  Conclusion: Based on current evidence and a Delphi process, we provided consensus and updated recommendations on prophylaxis and treatment of infections to guide those caring for pediatric rheumatology patients. What is Known: •Data largely derived from adults find that infectious diseases and related complications are a major cause of morbidity and mortality in patients with immune-mediated rheumatic diseases. •It is crucial to be aware of the preventive measures that should be implemented to prevent these infections in children, although most guidelines are often extrapolated from adult cases. What is New: •In the absence of evidence, a literature review and a Delphi survey were conducted to establish a series of expert recommendations that could prove useful in clinical practice, providing a practical and simple day-to-day approach to be used by pediatric rheumatologists. •The recommendations focus on tuberculosis, herpes zoster virus, fungal infections, and Pneumocystis jirovecii.

Fatal Pulmonary Veno-Occlusive Disease and Systemic Juvenile Idiopathic Arthritis: Case Report and Literature Review.

Systemic juvenile idiopathic arthritis (sJIA) is a chronic childhood inflammatory disease. SJIA accounts for approximately 5-15 per cent of all cases of JIA and has a high morbidity and mortality rate. In this disease, pulmonary complications (PC) other than pleuritis are much less frequent and not easily recognised by clinicians. Pulmonary hypertension, the most severe PC, is associated with uncontrolled disease and use of biologic therapies. We present a case of a school-age female with sJIA who died of acute cardiopulmonary instability secondary to pulmonary venous-occlusive disease demonstrated by necropsy. We describe her clinical evolution. We also undertook a narrative review of the literature about PC in sJIA to discuss the current state of the art regarding this complication. High disease activity and the use of multiple therapies include disease-modifying anti-rheumatic drugs should be a red flag for clinicians when discounting PC and pulmonary hypertension. The combination of chest X-ray, electrocardiogram and echocardiogram appear to be the best tests to achieve an early diagnosis.

Lupus nephritis in children. / Nefropatía lúpica en pacientes pediátricos.

INTRODUCTION: Lupus nephritis is an early manifestation in the development of systemic lupus erythematosus that worsens the morbidity and mortality of these patients. OBJECTIVE: To study the form of presentation in patients with lupus nephritis, the clinical and immunological characteristics, and their relationship with renal histology. PATIENTS AND METHOD: Retrospective study in children under 18 years of age, with lupus nephritis, in follow-up in a third level children's hospital in Madrid, between January 2012 and May 2020. We recorded demographic, clinical, and laboratory data (blood count, renal function, liver function, protein, ionogram, blood glucose, uric acid, lactate dehydrogenase, coagulation, and urine analysis), as well as immunological data (immunoglobulins, antinuclear antibodies, comple ment, and lupus anticoagulant), and histological classification data. Descriptive analysis and analysis of associations between variables was performed, with a significant p < 0.05. RESULTS: 16 patients (11 women) were included, the median age at presentation was 10.6 ± 2.3 years (5.7-15.3). The median time between symptoms onset and renal involvement was 6.3 months ± 10.5 (range 0 - 33.6). Renal involvement was the initial manifestation in 37.5% of patients. 50% had arthralgias or arthritis prior to diagnosis, and 25% had fever and constitutional symptoms (asthenia, anorexia, and/or weight loss). The most frequent form of renal involvement was microhematuria associated with proteinuria in non-nephrotic range. In the renal anatomo-pathological study, according to the ISN/RPS 2003 classification, grades III (46.6%) and IV (33.3%) predominated. CONCLUSIONS: Six patients presented renal involvement at baseline with musculoskeletal involvement being more frequent. Most patients (86.6%) presented advanced lupus nephritis in the histological study at diagnosis. Immunologic in volvement was the only marker that correlated with systemic involvement.

Epidemiological and clinical features of Kawasaki disease in Spain over 5 years and risk factors for aneurysm development. (2011-2016): KAWA-RACE study group.

BACKGROUND: Kawasaki disease (KD) is an acute self-limited systemic vasculitis of unknown etiology affecting mainly children less than 5 years of age. Risk factors for cardiac involvement and resistance to treatment are insufficiently studied in non-Japanese children. OBJECTIVE: This study aimed to investigate the epidemiology, clinical features and risk factors for resistance to treatment and coronary artery lesions (CAL) in KD in Spain. METHODS: Retrospective study (May 2011-June 2016) of all patients less than 16 years of age diagnosed with KD included in KAWA-RACE network (84 Spanish hospitals). RESULTS: A total of 625 cases were analyzed, 63% were males, 79% under 5 year-olds and 16.8% younger than 12 months. On echocardiographic examination CAL were the most frequent findings (23%) being ectasia the most common (12%). Coronary aneurysms were diagnosed in 9.6%, reaching 20% in infants under 12 months (p<0.001). A total of 97% of the patients received intravenous immunoglobulin (IVIG) with a median number of days from fever onset to IVIG administration of 7.2. A second dose was given to 15.7% and steroids to 14.5% patients. Only 1.4% patients received infliximab. No deaths were reported. A multivariate analysis identified anemia, hypoalbuminemia, hyponatremia, higher creatinine and procalcitonin as independent risk factors for treatment failure and length under 103 cm, hemoglobin < 10.2 mg/dL, platelets > 900,000 cells/mm3, maximum temperature < 39.5°C, total duration of fever > 10 days and fever before treatment ≥ 8 days as independent risk factors for developing coronary aneurysms. CONCLUSIONS: In our population, children under 12 months develop coronary aneurysms more frequently and children with KD with anemia and leukocytosis have high risk of cardiac involvement. Adding steroids early should be considered in those patients, especially if the treatment is not started before 8 days of fever. A score applicable to non-Japanese children able to predict the risk of aneurysm development and IVIG resistance is necessary.

Lupus eritematoso sistémico de debut atípico: a propósito de 3 casos / Systemic lupus erythematosus of atypical onset: A presentation of 3 cases

No disponible

Fatal Pulmonary Veno-Occlusive Disease and Systemic Juvenile Idiopathic Arthritis: Case Report and Literature Review / Enfermedad venooclusiva fatal y artritis idiopática juvenil sistémica: caso clínico y revisión de la literatura

Systemic juvenile idiopathic arthritis (sJIA) is a chronic childhood inflammatory disease. SJIA accounts for approximately 5–15 per cent of all cases of JIA and has a high morbidity and mortality rate. In this disease, pulmonary complications (PC) other than pleuritis are much less frequent and not easily recognised by clinicians. Pulmonary hypertension, the most severe PC, is associated with uncontrolled disease and use of biologic therapies. We present a case of a school-age female with sJIA who died of acute cardiopulmonary instability secondary to pulmonary venous-occlusive disease demonstrated by necropsy. We describe her clinical evolution. We also undertook a narrative review of the literature about PC in sJIA to discuss the current state of the art regarding this complication. High disease activity and the use of multiple therapies include disease-modifying anti-rheumatic drugs should be a red flag for clinicians when discounting PC and pulmonary hypertension. The combination of chest X-ray, electrocardiogram and echocardiogram appear to be the best tests to achieve an early diagnosis.(AU)

La artritis idiopática juvenil sistémica (AIJs) es una enfermedad juvenil crónica que representa aproximadamente del 5 al 15% de todos los casos de AIJ y tiene una elevada tasa de morbimortalidad. En esta enfermedad, las complicaciones pulmonares (CP) distintas a pleuritis son mucho menos frecuentes, y no fácilmente reconocibles por los clínicos. La hipertensión pulmonar, la CP más grave, está asociada a la enfermedad incontrolada y el uso de terapias biológicas. Presentamos el caso de una mujer en edad escolar con AIJs que falleció debido a inestabilidad cardiopulmonar aguda secundaria a enfermedad venosooclusiva confirmada en la necropsia. Describimos su evolución clínica, y también realizamos una revisión narrativa de la literatura relativa a CP en AIJs, para debatir los avances más recientes sobre esta complicación. La elevada actividad de la enfermedad y el uso de terapias múltiples que incluyen fármacos antirreumáticos modificadores de la enfermedad deberían servir de signo de alarma a los clínicos para descartar CP e hipertensión pulmonar. La combinación de placas de tórax, electrocardiograma y ecocardiograma parece ser el mejor conjunto de pruebas para lograr un diagnóstico precoz.(AU)

Enfermedad de Van Neck-Odelberg: una causa más de cojera en la infancia / Van Neck-Odelberg disease: a cause of limp in childhood

No disponible