International Journal of Rheumatic Diseases 2015; 18: 568–569

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Recent advances in pediatric rheumatology Surjit SINGH and Sagar BHATTAD Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India

1. Clinical features, treatment and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. A multinational, multicenter study of 362 patients Minoia F, Davı S, Horne A, Demirkaya E, Bovis F, Li C, Lehmberg K, Weitzman S, Insalaco A, Wouters C, Shenoi S, Espada G, Ozen S, Anton J, Khubchandani R, Russo R, Pal P, Kasapcopur O, Miettunen P, Maritsi D, Merino R, Shakoory B, Alessio M, Chasnyk V, Sanner H, Gao Y, Huasong Z, Kitoh T, Avcin T,Fischbach M, Frosch M, Grom A, Huber A, Jelusic M, Sawhney S, Uziel Y, Ruperto N, Martini A, Cron RQ, Ravelli A Arthritis Rheum 2014 July 30 [Epub ahead of print] Macrophage activation syndrome (MAS) remains a dreaded complication in children with systemic juvenile idiopathic arthritis (SJIA). It is a medical emergency and, if not recognized and treated early, can result in significant mortality. This study has data collated from 95 investigators across 33 countries. The diagnosis of MAS was based on the typical clinical and laboratory features – evidence of macrophage hemophagocytosis in the bone marrow was not an inclusion criterion. While median interval between onset of SJIA and development of MAS was 3.5 months, as many as 22% of SJIA subjects had MAS at onset of SJIA itself. This latter subset of SJIA represents a difficult diagnostic and therapeutic challenge for the pediatrician. Glucocorticoids were the

Correspondence: Dr Surjit Singh, Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India. Emails: [email protected]; surjitsinghapc@gmail. com

most commonly used group of drugs in management of MAS. Other commonly used drugs included cyclosporine and intravenous immunoglobulin. This study reports a mortality of 8%. MAS continues to be a diagnostic and therapeutic challenge for the pediatric rheumatologist. 2. Current evidence of anti- tumor necrosis factor a treatment efficacy in childhood chronic uveitis: a systematic review and meta-analysis approach of individual drugs Simonini G, Druce K, Cimaz R, Macfarlane GJ, Jones GT Arthritis Care Res 2014; 66(7), 1073–84 Autoimmune chronic uveitis (ACU) is a common accompaniment of pauciarticular juvenile idiopathic arthritis but can be associated with several other rheumatological disorders. Its pathogenesis is poorly understood and there is no consensus on management. Topical (and occasionally systemic) glucocorticoids and methotrexate (MTX) are the most common drugs for ACU. However, a proportion of children have refractory disease and can be very difficult to manage. As TNF-a has been implicated in the pathogenesis of some forms of ACU, anti-TNF-a therapies (e.g. infliximab [INF], adalimumab [ADA], etanercept [ETA]) have been tried on this condition. Simonini et al. have presented a meta-analysis of 23 studies (2000–2012) involving usage of anti-TNF-a agents in 229 children with ACU. It was found that INF and ADA were similar in efficacy and were superior to ETA. Almost 2/3 of children responded to anti-TNF-a therapy. Biologics represent a significant advance in management of refractory ACU in children but need to be used with caution.

© 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd

Pediatric rheumatology

3. Recent advances in juvenile idiopathic inflammatory myopathies Ernste FC, Reed AM Curr Opin Rheum 2014 August 23 [Epub ahead of print] Juvenile idiopathic inflammatory myopathies (JIIM) are among the more uncommon pediatric rheumatological disorders. The term includes juvenile dermatomyositis (JDM), polymyositis (PM) and juvenile connective tissue myositis (JCTM). This is an important review on the subject and cautions against development of clinical guidelines on JIIM based merely on extrapolation of data from adult studies. JDM is the most common JIIM and accounts for 85% of patients. While pathogenesis of JDM predominantly involves humoral immunity, PM results from alterations in cell-mediated immunity. It is also evident that the autoantibody profile in different types of JIIM is quite distinctive. The review reiterates the role of magnetic resonance imaging (MRI) as the preferred investigation in diagnosis and follow-up of children with JIIM. In fact, this investigation has virtually replaced electromyography and muscle biopsy as the principal means of diagnosing JIIM. Although significant progress has been made in our understanding of the pathogenesis of JIIM, many children may continue to have poorly controlled disease despite years of therapy. This needs redressing through multicentric studies. 4. Macrophage activation syndrome in Kawasaki disease: More common than we thought? Wang W, Gong F, Zhu W, Fu S, Zhang Q Semin Arthritis Rheum 2014 August 7 [Epub ahead of print] Kawasaki disease (KD) is the most common vasculitic disorder of childhood. It has been known for several years now that a proportion of children with KD may have thrombocytopenia either at onset or during the course of the disease. This subset of patients has been identified to have a more severe phenotype of KD and is associated with poorer outcomes. What has hitherto not been clearly recognized is the fact that this thrombocytopenia may, in fact, represent the evolution of macrophage activation syndrome (MAS) in KD. Wang et al. have shown in this report from China that MAS can indeed be associated with KD. All pediatricians need to be aware of this complication while managing children with KD.

International Journal of Rheumatic Diseases 2015; 18: 568–569

5. Distinct effects of methotrexate and etanercept on the B cell compartment in patients with juvenile idiopathic arthritis Glaesener S, Quach TD, Onken N, Weller-Heinemann F, Dressler F, Huppertz HI, Thon A, Meyer-Bahlburg A Arthritis Rheum 2014; 66(9), 2590–600 Methotrexate (MTX) is the principal disease-modifying anti-rheumatic drug and the cornerstone of management of juvenile idiopathic arthritis (JIA), especially in the polyarticular and arthritic predominant systemic types. As TNF-a plays a key role in pathogenesis of polyarticular JIA, TNF-a blockade (especially with etanercept) is now the standard of care for children with polyaricular JIA who show less than appropriate response to MTX alone. Further, it is known that a combination of MTX and etanercept works better than monotherapy with either of the two agents. Glaesner et al. have evaluated the effect of MTX and etanercept on B cells, follicular helper T cells, immunoglobulin and B cell activating factor (BAFF) levels in the peripheral blood of 110 children with JIA. While MTX reduces the transitional B cells and immunoglobulins, etanercept reduces the BAFF levels. This is an important finding and may explain why combination therapy is better than monotherapy in these children. However, the results need to be replicated on a larger sample, preferably in a multicentric setting. 6. Can procalcitonin be used to distinguish between disease flare and infection in patients with systemic lupus erythematosus: a systematic literature review Serio I, Arnaud L, Mathian A, Hausfater P, Amoura Z Clin Rheum 2014; 33(9), 1209–15. Systemic lupus erythematosus remains a major cause of morbidity and mortality, especially in children. Differentiating a disease flare from infection represents the eternal clinical challenge for the treating physician. Clinical assessment is often unreliable and laboratory parameters (e.g. C-reactive protein) can be difficult to interpret in a given patient. Serio et al. have evaluated the role of procalcitonin (PCT) in differentiating SLE flare from infection through a systematic review. The authors concluded that while a PCT level above 0.5 lg/L would be suggestive of a bacterial infection in SLE patients with fever, PCT levels do not seem to be elevated in patients with lupus activity. Although PCT assay may be useful when used in this context, prudence demands caution in extrapolating these results onto the bedside in a disease as complex as lupus. The last word remains to be said on this biochemical parameter.

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Recent advances in pediatric rheumatology.

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