Vaccinations in juvenile chronic inflammatory diseases: an update

Vaccination is a powerful tool to reduce the burden of infectious diseases in paediatric patients with chronic rheumatic diseases. Live attenuated vaccines are not recommended for profoundly immunosuppressed patients, but nonlive vaccines have adequate safety and efficacy profiles in the few (admittedly underpowered) studies published to date. No severe vaccine-specific or disease-specific adverse events have been observed in patients with juvenile idiopathic arthritis (JIA) or childhood-onset systemic lupus erythematosus (SLE) who have been vaccinated with live or nonlive agents. The immune response to live vaccines is variable in these patients but generally adequate, despite concomitant use of immunosuppressive and biologic agents. The proposal that onset of autoimmune rheumatic diseases could be induced by vaccination is controversial and primarily based on case reports; however, patients with mevalonate kinase deficiency can experience febrile attacks after immunizations. Adequately powered studies of live and nonlive vaccination in patients with paediatric rheumatic diseases are necessary to clarify safety and efficacy issues. This narrative Review discusses vaccination in patients with JIA, childhood-onset SLE, juvenile dermatomyositis, juvenile systemic sclerosis, primary vasculitis and autoinflammatory syndromes. Vaccine safety, short-term and long-term changes in disease parameters, and the immunogenicity and influence of immunosuppressive agents are outlined for each combination of disease and vaccine.