Tocilizumab and Aflibercept as a Treatment Option for Refractory Macular Edema after Acute Retinal Necrosis.

Introduction: The inflammatory milieu after acute retinal necrosis (ARN) may lead to a breakdown of the inner and outer blood-retinal barrier and consequently to a cystoid macular edema (CME) with accumulation of intra- and subretinal fluid. Up to now, there is no established therapeutic approach for CME in ARN patients.Case report: We report a case of an immunocompetent 14-year-old female with chronic ARN-related CME, which was unresponsive to valacyclovir, prednisone and intravitreal ranibizumab injections.

Current practice of transitional care for adolescents and young adults in Swiss paediatric and adult rheumatology centres.

Background: About half of all children with rheumatic diseases need continuous medical care during adolescence and adulthood. A good transition into adult rheumatology is essential. Guidelines for a structured transition process have therefore been recommended by the European League Against Rheumatism (EULAR) and the Paediatric Rheumatology European Society (PReS).

Chronic nonbacterial osteomyelitis in children: a retrospective multicenter study.

Background: To determine the clinical presentation, current treatment and outcome of children with nonbacterial inflammatory bone disease.

Methods: Retrospective multicenter study of patients entered into the Swiss Pediatric Rheumatology Working Group registry with a diagnosis of chronic nonbacterial osteomyelitis (CNO) and synovitis acne pustulosis hyperostosis osteitis (SAPHO) syndrome. The charts were reviewed for informations about disease presentation, treatment, course and outcome.

Dissecting the heterogeneity of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis.

Objective: To seek insights into the heterogeneity of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA) through the analysis of a large patient sample collected in a multinational survey.

Methods: International pediatric rheumatologists and hemato-oncologists entered their patient data, collected retrospectively, in a Web-based database. The demographic, clinical, laboratory, histopathologic, therapeutic, and outcome data were analyzed in relation to (1) geographic location of caring hospital, (2) subspecialty of attending physician, (3) demonstration of hemophagocytosis, and (4) severity of clinical course.

Immune response to influenza vaccination in children treated with methotrexate or/and tumor necrosis factor-alpha inhibitors.

In children treated with immunosuppressive medication such as methotrexate and tumor necrosis factor-alpha (TNF-α) inhibitors, additional immunizations are recommended because of increased susceptibility to infections. However, it is unclear if adequate antibody response to vaccinations can be established in children receiving methotrexate and/or TNF-α inhibitors. In a prospective open label study, we assessed seroprotection and seroconversion following influenza vaccination during 2 seasons (6 strains) in 36 children with autoimmune disease treated either with methotrexate (n=18), TNF-α inhibitors (n=10) or both (n=8) and a control group of 16 immunocompetent children.

Adrenal insufficiency after glucocorticoid withdrawal in children with rheumatic diseases.

Aim:  Glucocorticoids (GCs) are often used for the treatment of rheumatic disorders. However, doses are prescribed, which may suppress the hypothalamic-pituitary-adrenal (HPA) axis. After GC withdrawal, recovery of the HPA axis may be delayed putting the patient at risk for adrenal insufficiency.

Early diagnosis of temporomandibular joint involvement in juvenile idiopathic arthritis: a pilot study comparing clinical examination and ultrasound to magnetic resonance imaging.

Objectives: To study the validity of both rheumatological and orthodontic examinations and ultrasound (US) as screening methods for early diagnosis of TMJ arthritis against the gold standard MRI.

Methods: Thirty consecutive juvenile idiopathic arthritis (JIA) patients were included in this pilot study. Rheumatological and orthodontic examinations as well as US were performed within 1 month of the MRI in a blinded fashion.

Risk factors and longterm outcome of juvenile idiopathic arthritis-associated uveitis in Switzerland.

Objective: To determine rate, risk factors, and longterm outcome of uveitis in children with juvenile idiopathic arthritis (JIA) in Switzerland and compare the results with a study of a different center in Switzerland from 1992.

Methods: Retrospective analysis of the charts and ophthalmologists' reports of all patients with JIA in a tertiary care outpatient clinic between January 1, 1997, and December 31, 2005, for diagnosis, course, and outcome of uveitis.

Results: Uveitis occurred in 35/265 patients (13.

Factors influencing the efficacy of intra-articular steroid injections in patients with juvenile idiopathic arthritis.

A retrospective chart review was performed of all patients with juvenile idiopathic arthritis (JIA) followed at our clinic who had an intra-articular steroid injection between 1 January 1997 and 31 December 2001. The aim of the study was to evaluate the outcome of intra-articular steroid injections (iaS) and determine prognostic factors. During the study period, 202 iaS were performed in 60 patients, of whom 37 had oligoarticular JIA, 15 had polyarticular, rheumatoid factor-negative JIA and four each had systemic and enthesitis-related JIA.

Cardiac features in the presymptomatic period in a neonate with anomalous left coronary artery arising from the pulmonary trunk.

We describe a neonate presenting with a cholestatic jaundice due to Alagille syndrome. On echocardiography as part of the diagnostic work-up, we noted a slight dilation of the normally functioning left ventricle at the initial examination in the third week of life. Over the next 2 weeks, serial echocardiograms showed slowly progressive dilation and dysfunction of the left ventricle, together with a persistent mild elevation of levels of cardiac Troponin-I in the serum.