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

Abstract

BackgroundTo 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.MethodsRetrospective 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.ResultsTwenty-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.ConclusionThe 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.

Opportunistic infections in immunosuppressed patients with juvenile idiopathic arthritis: analysis by the Pharmachild Safety Adjudication Committee

“…The best example is represented by herpes zoster (Tables 4 and 5). Varicella zoster presentation was included among the "confirmed OI," as suggested in the majority of the literature in this issue [49][50][51]. However, primary varicella infection, frequently observed in our population (155/682, 22.7%), was included among the "possible/patient and/or pathogen-related OI" rather than "definite OI" due to the high incidence in healthy non-vaccinated children and its usually non-complicated presentation.…”

Varicella Pneumonia in an Adult with Immunocompromised Status: Case Report

“…The patients on immunosuppressive therapy have more opportunistic infectious events, in which the most frequent (68%) were caused by HPV [10]. Likewise, the patients who accepted immunosuppressive treatment had a shorter time to catch varicella and develop varicella cellulitis, sepsis, and cerebellitis [11].One survey from the USA, the incidence of VZV was highest in patients with bone marrow or stem cell transplant (43.03 %) followed by solid organ transplant, human immunode ciency virus (HIV) infection, and systemic lupus erythematosus (SLE). VZV incidence rates were higher among persons on immunosuppressants/chemotherapy than among non-users [12].…”

How the COVID-19 pandemic has influenced pediatric rheumatology practice: Results of a global, cross-sectional, online survey

“…Previous studies have reported an association between immunosuppression and increased risk of serious infections in patients with rheumatic diseases [ 19 , 20 ]. On the other hand, active rheumatic disease is a significant risk factor for infection [ 21 , 22 ].…”