Volume 22, Number 10—October 2016
CME ACTIVITY - Synopsis
Infection-Related Death among Persons with Refractory Juvenile Idiopathic Arthritis
|Characteristic||Patient 1||Patient 2|
|Year of disease onset/year of death||1996/2004||2000/2004|
|Age at So-JIA diagnosis/at death, y
|JIA symptom or sign, ever|
|Macrophage activation syndrome
|Disease remission, ever||No||No|
|Side effects of treatment|
*JADAS, Juvenile Arthritis Disease Activity Score; JIA, juvenile idiopathic arthritis; So-JIA, systemic JIA.
†Linear sum of the scores of the 4 JADAS components (0–40): physician global assessment of disease activity (measured on a 10-cm visual analog scale; 0 = no activity and 10 = maximum activity); parent/patient global assessment of well-being (measured on a 10-cm visual analog scale; 0 = very well and 10 = very poor); count of joints with active disease (0–10); erythrocyte sedimentation rate (actual value/10; range 0–10) (13).
‡Methylprednisolone pulses (intravenous 30 mg/kg/day; maximum. 1 g) given as a 3-day course or a single dose (weekly or otherwise), when indicated based on clinical decision (for treating a disease flare). Prednisolone maintenance dose (orally 0.5–1 mg/kg/d) with adjusting and aiming for alternate day regimen whenever possible depending on the clinical course or disease activity.
§Treatment at time of death.
¶Subcutaneously 15 mg/m2/wk.
††December 2003–January 2004.
‡‡0.4 mg/kg/wk during March–May 2000 (12) and again during May 2003–January 2004.
§§5 mg/kg single dose, February 2004.
¶¶6 mg/kg/mo during December 2002–December 2003.
- Prakken B, Albani S, Martini A. Juvenile idiopathic arthritis. Lancet. 2011;377:2138–49.
- Hinze C, Gohar F, Foell D. Management of juvenile idiopathic arthritis: hitting the target. Nat Rev Rheumatol. 2015;11:290–300.
- Ringold S, Weiss PF, Beukelman T, Dewitt EM, Ilowite NT, Kimura Y, ; American College of Rheumatology. 2013 update of the 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic arthritis: recommendations for the medical therapy of children with systemic juvenile idiopathic arthritis and tuberculosis screening among children receiving biologic medications. Arthritis Care Res (Hoboken). 2013;65:1551–63.
- Southwood TR. Paediatric rheumatic disease: Treatment of JIA in the biologic era: what are we waiting for? Nat Rev Rheumatol. 2014;10:132–4.
- Otten MH, Prince FH, Anink J, Ten Cate R, Hoppenreijs EP, Armbrust W, Effectiveness and safety of a second and third biological agent after failing etanercept in juvenile idiopathic arthritis: results from the Dutch National ABC Register. Ann Rheum Dis. 2013;72:721–7.
- Record JL, Beukelman T, Cron RQ. Combination therapy of abatacept and anakinra in children with refractory systemic juvenile idiopathic arthritis: a retrospective case series. J Rheumatol. 2011;38:180–1.
- Woerner A, Ritz N. Infections in children treated with biological agents. Pediatr Infect Dis J. 2013;32:284–8.
- Hashkes PJ, Uziel Y, Laxer RM. The safety profile of biologic therapies for juvenile idiopathic arthritis. Nat Rev Rheumatol. 2010;6:561–71.
- Abinun M. Haematopoietic stem cell transplantation for rheumatological conditions. Paediatr Child Health. 2011;21:558–62 .
- Abinun M, Flood TJ, Cant AJ, Veys P, Gennery AR, Foster HE, Autologous T cell depleted haematopoietic stem cell transplantation in children with severe juvenile idiopathic arthritis in the UK (2000-2007). Mol Immunol. 2009;47:46–51.
- Milanetti F, Abinun M, Voltarelli JC, Burt RK. Autologous hematopoietic stem cell transplantation for childhood autoimmune disease. Pediatr Clin North Am. 2010;57:239–71.
- Myers A, Clark J, Foster H. Tuberculosis and treatment with infliximab. N Engl J Med. 2002;346:623–6.
- Consolaro A, Ruperto N, Bracciolini G, Frisina A, Gallo MC, Pistorio A, ; Paediatric Rheumatology International Trials Organization (PRINTO). Defining criteria for high disease activity in juvenile idiopathic arthritis based on the juvenile arthritis disease activity score. Ann Rheum Dis. 2014;73:1380–3.
- Ravelli A, Minoia F, Davì S, Horne A, Bovis F, Pistorio A, 2016 classification criteria for MAS complicating SO-JIA: EULAR/ACR/PPRINTO Initiative. Arthritis Rheumatol. 2016;68:566–76.
- Mermel LA, Allon M, Bouza E, Craven DE, Flynn P, O’Grady NP, Clinical practice guidelines for the diagnosis and management of intravascular catheter-related infection: 2009 Update by the Infectious Diseases Society of America.[ [Erratum in: Clin Infect Dis. 2010;50:457. Erratum in: Clin Infect Dis. 2010;50:1079. ]. Clin Infect Dis. 2009;49:1–45.
- Minoia F, Davì S, Horne A, Demirkaya E, Bovis F, Li C, ; Pediatric Rheumatology International Trials Organization; Childhood Arthritis and Rheumatology Research Alliance; Pediatric Rheumatology Collaborative Study Group; Histiocyte Society. Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients. Arthritis Rheumatol. 2014;66:3160–9.
- Lovell DJ, Zaoutis TE, Sullivan K. Immunosuppressants, infection, and inflammation. Clin Immunol. 2004;113:137–9.
- Beukelman T, Xie F, Chen L, Baddley JW, Delzell E, Grijalva CG, ; SABER Collaboration. Rates of hospitalized bacterial infection associated with juvenile idiopathic arthritis and its treatment. Arthritis Rheum. 2012;64:2773–80.
- Kimura Y, Weiss JE, Haroldson KL, Lee T, Punaro M, Oliveira S, ; Childhood Arthritis Rheumatology Research Alliance Carra Net Investigators. Pulmonary hypertension and other potentially fatal pulmonary complications in systemic juvenile idiopathic arthritis. Arthritis Care Res (Hoboken). 2013;65:745–52.
- van de Vosse E, van Dissel JT, Ottenhoff TH. Genetic deficiencies of innate immune signalling in human infectious disease. Lancet Infect Dis. 2009;9:688–98.
- Abinun M. An overview of infectious complications in children on new biologic response-modifying agents. Ped Health. 2010;4:509–17 .
- Maródi L, Casanova JL. Primary immunodeficiencies may reveal potential infectious diseases associated with immune-targeting mAb treatments. J Allergy Clin Immunol. 2010;126:910–7.
- Puel A, Picard C, Lorrot M, Pons C, Chrabieh M, Lorenzo L, Recurrent staphylococcal cellulitis and subcutaneous abscesses in a child with autoantibodies against IL-6. J Immunol. 2008;180:647–54.
- Swart JF, de Roock S, Wulffraat NM. What are the immunological consequences of long-term use of biological therapies for juvenile idiopathic arthritis? Arthritis Res Ther. 2013;15:213.
- Fitch PG, Cron RQ. Septic abscess in a child with juvenile idiopathic arthritis receiving anti-tumor necrosis factor-alpha. J Rheumatol. 2006;33:825, author reply 826–7.
- Renaud C, Ovetchkine P, Bortolozzi P, Saint-Cyr C, Tapiero B. Fatal group A Streptococcus purpura fulminans in a child receiving TNF-α blocker. Eur J Pediatr. 2011;170:657–60.
- Toussi SS, Pan N, Walters HM, Walsh TJ. Infections in children and adolescents with juvenile idiopathic arthritis and inflammatory bowel disease treated with tumor necrosis factor-α inhibitors: systematic review of the literature. Clin Infect Dis. 2013;57:1318–30.
- Elwood RL, Pelszynski MM, Corman LI. Multifocal septic arthritis and osteomyelitis caused by group A Streptococcus in a patient receiving immunomodulating therapy with etanercept. Pediatr Infect Dis J. 2003;22:286–8.
- Henderson C, Goldbach-Mansky R. Monogenic IL-1 mediated autoinflammatory and immunodeficiency syndromes: finding the right balance in response to danger signals. Clin Immunol. 2010;135:210–22.
- Cole TS, Rogerson E, Collins J, Galloway A, Clark J. Central venous catheter-related blood stream infections in children undergoing hematopoietic stem cell transplant for primary immunodeficiency and other nonmalignant disorders. Pediatr Infect Dis J. 2011;30:1098–100.
- Eleftheriou D, Melo M, Marks SD, Tullus K, Sills J, Cleary G, Biologic therapy in primary systemic vasculitis of the young. Rheumatology (Oxford). 2009;48:978–86.
- Russell RK, Wilson ML, Loganathan S, Bourke B, Kiparissi F, Mahdi G, A British Society of Paediatric Gastroenterology, Hepatology and Nutrition survey of the effectiveness and safety of adalimumab in children with inflammatory bowel disease. Aliment Pharmacol Ther. 2011;33:946–53.
- Radhakrishna SM, Reiff AO, Marzan KA, Azen C, Khemani RG, Rubin S, Pediatric rheumatic disease in the intensive care unit: lessons learned from 15 years of experience in a tertiary care pediatric hospital. Pediatr Crit Care Med. 2012;13:e181–6.
- Shulman AI, Punaro M. Critical care of the pediatric patient with rheumatic disease. Curr Opin Pediatr. 2011;23:263–8.
- Thomas E, Symmons DP, Brewster DH, Black RJ, Macfarlane GJ. National study of cause-specific mortality in rheumatoid arthritis, juvenile chronic arthritis, and other rheumatic conditions: a 20 year followup study. J Rheumatol. 2003;30:958–65.
- Hashkes PJ, Wright BM, Lauer MS, Worley SE, Tang AS, Roettcher PA, Mortality outcomes in pediatric rheumatology in the US. Arthritis Rheum. 2010;62:599–608.
- Lionetti G, Kimura Y, Schanberg LE, Beukelman T, Wallace CA, Ilowite NT, Using registries to identify adverse events in rheumatic diseases. Pediatrics. 2013;132:e1384–94.
- Ringold S, Hendrickson A, Abramson L, Beukelman T, Blier PR, Bohnsack J, Novel method to collect medication adverse events in juvenile arthritis: results from the childhood arthritis and rheumatology research alliance enhanced drug safety surveillance project. Arthritis Care Res (Hoboken). 2015;67:529–37.
- Ruperto N, Brunner HI, Quartier P, Constantin T, Wulffraat N, Horneff G, ; PRINTO; PRCSG. Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis. N Engl J Med. 2012;367:2396–406.
- De Benedetti F, Brunner HI, Ruperto N, Kenwright A, Wright S, Calvo I, ; PRINTO; PRCSG. Randomized trial of tocilizumab in systemic juvenile idiopathic arthritis. N Engl J Med. 2012;367:2385–95.
- van Royen-Kerkhof A, Vastert BS, Swart JF, Wulffraat NM. Biologic treatment of pediatric rheumatic diseases: are we spoilt for choice? Immunotherapy. 2014;6:1–3.
- Wahezi DM, Ilowite NT. Juvenile idiopathic arthritis: an update on current pharmacotherapy and future perspectives. Expert Opin Pharmacother. 2013;14:975–89.
1Current affiliation: Leeds General Infirmary, Leeds, UK.