No abstract

TCZ appears to be a useful therapy for severe refractory JIA-associated uveitis.

Background: Osteoporosis incidence in children is increasing due to the increased survival rate of patients suffering from chronic diseases and the increased use of drugs that can damage bones. Recent changes made to the definition of childhood osteoporosis, along with the lack of guidelines or national consensuses regarding its diagnosis and treatment, have resulted in a wide variability in the approaches used to treat this disease. For these reasons, the Osteogenesis Imperfecta and Childhood Osteoporosis Working Group of the Spanish Society of Pediatric Rheumatology has sounded the need for developing guidelines to standardize clinical practice with regard to this pathology. Methods: An expert panel comprised of 6 pediatricians and 5 rheumatologists carried out a qualitative literature review and provided recommendations based on evidence, when that was available, or on their own experience. The level of evidence was determined for each section using the Oxford Centre for Evidence-based Medicine (CEBM) system. A Delphi survey was conducted for those recommendations with an evidence level of IV or V. This survey was sent to all members of the SERPE. All recommendations that had a level of agreement higher or equal to 70% were included. Results: Fifty-one recommendations, categorized into eight sections, were obtained. Twenty-four of them presented an evidence level 4 or 5, and therefore a Delphi survey was conducted. This was submitted electronically and received a response rate of 40%. All recommendations submitted to the Delphi round obtained a level of agreement of 70% or higher and were therefore accepted. Conclusion: In summary, we present herein guidelines for the prevention, diagnosis and treatment of secondary childhood osteoporosis based on the available evidence and expert clinical experience. We believe it can serve as a useful tool that will contribute to the standardization of clinical practice for this pathology. Prophylactic measures, early diagnosis and a proper therapeutic approach are essential to improving bone health, not only in children and adolescents, but also in the adults they will become in the future.

Cystoid macular edema (CME) is a leading cause of blindness. In this study we assessed the efficacy and safety of Tocilizumab (TCZ) in refractory CME. DESIGN: Retrospective case series. METHODS: Patients with CME secondary to non-infectious uveitis who had inadequate response to corticosteroids and at least one conventional immunosuppressive drug, and in most cases to other biological agents were studied. CME was defined as central retinal thickness greater than 300 µm. The primary outcome measure was macular thickness. Intraocular inflammation, best corrected visual acuity (BCVA), and corticosteroid-sparing effect were also analyzed. RESULTS: A total of 25 patients (mean± SD age 33.6±18.9 years; 17 women) with CME were assessed. Underlying diseases associated with uveitis-related CME are juvenile idiopathic arthritis (n=9), Behçet's disease (n=7), birdshot retinochoroidopathy (n=4), idiopathic (n=4), and sarcoidosis (n=1). The ocular patterns were panuveitis (n=9), anterior uveitis (n=7), posterior uveitis (n=5) and intermediate uveitis (n=4). Most patients had CME in both eyes (n=24). TCZ was used in monotherapy (n=11) or combined with conventional immunosuppressive drugs. Regardless of the underlying disease, compared to baseline, a statistically significant improvement in macular thickness (415.7±177.2 vs 259.1±499.5 microns; p=0.00009) and BCVA (0.39±0.31 vs 0.54±0.33; p =0.0002) was obtained, allowing us to reduce the daily dose of prednisone (15.9±13.6 mg/day vs 3.1±2.3 p=0.002) after 12 months of therapy. Remission was achieved in 14 patients. Only minor side effects were observed after a mean follow up of 12.7±8.34 months. CONCLUSION: Macular thickness is reduced following administration of TCZ in refractory uveitis-related CME.