Clinical course and therapeutic approach to varicella zoster virus infection in children with rheumatic autoimmune diseases under immunosuppression

Background: to analyze the clinical presentation and complications of varicella zoster virus (vzv) infection in children with rheumatic diseases treated with immunosuppressive medication such as biological disease-modifying antirheumatic drugs (bdmards) and/or conventional disease-modifying antirheumatic drugs (cdmards),and to analyze the therapeutic approach to vzv infections with respect to the concomitant immunosuppressive treatment. methods: retrospective multicenter study using the swiss pediatric rheumatology registry. children with rheumatic diseases followed in a swiss center for pediatric rheumatology and treated with cdmard and/or bdmard with a clinical diagnosis of varicella or herpes zoster between january 2004 and december 2013 were included. results: twenty-two patients were identified,of whom 20 were treated for juvenile idiopathic arthritis,1 for a polyglandular autoimmune syndrome type iii,and 1 for uveitis. of these 22 patients,16 had varicella and 6 had herpes zoster. median age at vzv disease was 7.6 years (range 2 to 17 years),with 6.3 years (range 2 to 17 years) for those with varicella and 11.6 years (range 5 to 16 years) for those with herpes zoster. the median interval between start of immunosuppression and vzv disease was 14.1 months (range 1 to 63 months). two patients had received varicella vaccine (1 dose each) prior to start of immunosuppression. concomitant immunosuppressive therapy was methotrexate (mtx) monotherapy (n = 9) or bdmard monotherapy (n = 2),or a combination of bdmard with prednisone,mtx or leflunomide (n = 11). four patients experienced vzv related complications: cellulitis in 1 patient treated with mtx,and cellulitis,sepsis and cerebellitis in 3 patients treated with biological agents and mtx combination therapy. six children were admitted to hospital (range of duration: 4 to 9 days) and 12 were treated with valaciclovir or aciclovir. conclusion: the clinical course of varicella and herpes zoster in children under immunosuppression is variable,with 4 (18 %) of 22 children showing a complicated course. thorough assessment of vzv disease and vaccination history and correct vzv vaccination according to national guidelines at diagnosis of a rheumatic autoimmune disease is essential to minimize vzv complications during a later immunosuppressive treatment. © 2016 leuvenink et al.