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.
['Leuvenink|Raphael|R|', 'Aeschlimann|Florence|F|', 'Baer|Walter|W|', 'Berthet|Gerald|G|', 'Cannizzaro|Elvira|E|', 'Hofer|Michael|M|', 'Kaiser|Daniela|D|', 'Schroeder|Silke|S|', 'Heininger|Ulrich|U|', 'Woerner|Andreas|A|']
D000212:Acyclovir D000293:Adolescent D018501:Antirheumatic Agents D000998:Antiviral Agents D001171:Arthritis, Juvenile D002644:Chickenpox D002648:Child D002675:Child, Preschool D000068800:Etanercept D005260:Female D006562:Herpes Zoster D006801:Humans D016867:Immunocompromised Host D007166:Immunosuppressive Agents D007555:Isoxazoles D000077339:Leflunomide D008297:Male D008727:Methotrexate D012189:Retrospective Studies D016896:Treatment Outcome D000077483:Valacyclovir D014633:Valine D055815:Young Adult
Varicella zoster virus Rheumatic autoimmune disease Immunosuppression Pediatric