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Esplugues de Llobregat, Spain

Iglesias E.,Unit of Pediatric Rheumatology | Eleftheriou D.,University College London | Mankad K.,Great Ormond Street Hospital for Children National Health Service Foundation Trust | Prabhakar P.,Great Ormond Street Hospital for Children National Health Service Foundation Trust | Brogan P.A.,University College London
Journal of Child Neurology | Year: 2014

Microscopic polyangiitis associated with antineutrophil cytoplasmic antibodies directed against myeloperoxidase rarely affects the central nervous system, and this is common in the presence of other organ involvement. The authors report the case of a 12-year-old girl who presented with multiple acute parieto-occipital hematomas as the only manifestation of presumed microscopic polyangiitis. Early treatment with immunosuppression resulted in complete recovery and a favorable outcome. © The Author(s) 2013.

Garcia-De-Vicuna C.,University of Barcelona | Diaz-Llopis M.,University of Valencia | Salom D.,University of Valencia | Bou R.,Unit of Pediatric Rheumatology | And 11 more authors.
Mediators of Inflammation | Year: 2013

Purpose. To assess the efficacy and safety of adalimumab in patients with juvenile idiopathic arthritis (JIA) and associated refractory uveitis. Design. Multicenter, prospective case series. Methods. Thirty-nine patients (mean [SD] age of 11.5 [7.9] years) with JIA-associated uveitis who were either not responsive to standard immunosuppressive therapy or intolerant to it were enrolled. Patients aged 13-17 years were treated with 40 mg of adalimumab every other week for 6 months and those aged 4-12 years received 24 mg/m2 body surface. Results. Inflammation of the anterior chamber (2.02 [1.16] versus 0.42 [0.62]) and of the posterior segment (2.38 [2.97] versus 0.35 [0.71] decreased significantly between baseline and the final visit (P < 0.001). The mean (SD) macular thickness at baseline was 304.54 (125.03) and at the end of follow-up was 230.87 (31.12) (P < 0.014). Baseline immunosuppression load was 8.10 (3.99) as compared with 5.08 (3.76) at the final visit (P < 0.001). The mean dose of corticosteroids also decreased from 0.25 (0.43) to 0 (0.02) mg (P < 0.001). No significant side effects requiring discontinuation of therapy were observed. Conclusion. Adalimumab seems to be an effective and safe treatment for JIA-associated refractory uveitis and may reduce steroid requirement. © 2013 Carmen García-De-Vicuña et al.

Palmblad K.,Unit of Pediatric Rheumatology | Schierbeck H.,Unit of Pediatric Rheumatology | Sundberg E.,Unit of Pediatric Rheumatology | Horne A.-C.,Unit of Pediatric Rheumatology | And 4 more authors.
Molecular Medicine | Year: 2014

Macrophage activation syndrome (MAS) is a potentially fatal complication of systemic inflammation. High mobility group box 1 (HMGB1) is a nuclear protein extensively leaked extracellularly during necrotic cell death or actively secreted by natural killer (NK) cells, macrophages and additional cells during infection or sterile injury. Extracellular HMGB1 orchestrates key events in inflammation as a prototypic alarmin. The redox states of its three cysteines render the molecule mutually exclusive functions: fully reduced “all-thiol HMGB1”exerts chemotactic activity; “disulfide HMGB1” has cytokine-inducing, toll-like receptor 4 (TLR4)- mediated effects—while terminally oxidized “sulfonyl HMGB1” lacks inflammatory activity. This study examines the kinetic pattern of systemic HMGB1 isoform expression during therapy in four children with severe MAS. Three of the four patients with underlying systemic rheumatic diseases were treated with biologics and two suffered from triggering herpes virus infections at the onset of MAS. All patients required intensive care unit therapy due to life-threatening illness. Tandem mass-spectrometric analysis revealed dramatically increased systemic levels of the cytokine-inducing HMGB1 isoform during early MAS. Disease control coincided with supplementary etoposide therapy initiated to boost apoptotic cell death, when systemic HMGB1 levels drastically declined and the molecule emerged mainly in its oxidized, noninflammatory isoform. Systemic interferon (IFN)-©and ferritin peaked concomitantly with HMGB1, whereas interleukin (IL)-18 and monocyte chemotactic protein (MCP)-1 levels developed differently. In conclusion, this work provides new insights in HMGB1 biology, suggesting that the molecule is not merely a biomarker of inflammation, but most likely also contributes to the pathogenesis of MAS. These observations encourage further studies of disulfide HMGB1 antagonists to improve outcome of MAS. © 2014, Uninversity of Michigan. All rights reserved.

Gimenez-Roca C.,Unit of Pediatric Rheumatology | Iglesias E.,Unit of Pediatric Rheumatology | Torrente-Segarra V.,Unit of Pediatric Rheumatology | Bou R.,Unit of Pediatric Rheumatology | And 5 more authors.
Rheumatology International | Year: 2014

The aims of the study were to assess efficacy and safety of TNF-alpha antagonists (anti-TNF) in a cohort of patients with juvenile idiopathic arthritis (JIA) who began treatment under 4 years old and to assess relapse rate after methotrexate and/or anti-TNF withdrawal. We made a retrospective charts review of our non-systemic JIA patients treated with anti-TNF under 4 years of age between January 2006 and April 2013. Demographics, epidemiologic, clinical, laboratory data and rate of relapse after treatment withdrawal due to clinical remission were collected. Efficacy and safety end points included side effects (SE) and time to achieve clinical remission. We included 27 patients, 23 received etanercept and 4 adalimumab with a median age of 3.01 (range 0.88–3.97) years at anti-TNF beginning and 1.94 (range 0.18–5.44) and 2.39 (range 0.18–7.24) years of treatment and follow-up, respectively. All patients had previously received disease-modifying antirheumatic drugs at optimal dose. Nineteen patients reached clinical remission on treatment in a median time of 9.1 (range 6.23–21.17) months. Four of those relapsed during treatment. Six developed mild SE, mostly mild infections. No serious SE were described. Eleven patients who reached clinical remission relapsed after treatment withdrawal. None achieved clinical remission off treatment. Most patients reached clinical remission on anti-TNF. In our cohort of patients, etanercept and adalimumab were safe, with mostly mild infections and no serious SE. We observed a high relapse rate during treatment withdrawal. © 2014, Springer-Verlag Berlin Heidelberg.

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