Recommendations with grade of recommendation and Delphi score on agreement among experts
| RC | Grade | Delphi score (mean (SD)) | |
|---|---|---|---|
| IMMUNOSUPPRESSIVE DRUGS | |||
| 1 | When indicated according to national guidelines*, non-live vaccines can be administered to PaedRD using glucocorticosteroids, DMARDs and/or anti-TNFα therapy. | C | 9.8 (0.4) |
| 2 | To assure adequate immune responses, it is recommended to determine pathogen-specific antibody concentrations after vaccination in all PaedRD on high-dose glucocorticosteroids† (≥2 mg/kg or ≥20 mg/day for ≥2 weeks) or on rituximab. Measuring pathogen-specific antibody concentrations can be considered in patients on anti-TNFα treatment. | C | 8.4 (2.9) |
| 3 | In patients with an indication for pneumococcal or influenza vaccination, it is recommended to vaccinate prior to rituximab use whenever possible. | C | 9.8 (0.6) |
| 4 | In PaedRD with a contaminated wound, it is suggested to administer tetanus immunoglobulin to those patients treated with rituximab in the past 6 months, since responses to TT vaccination can be reduced. | D | 9.6 (0.9) |
| 5 | To assure adequate immune responses, it is recommended to determine pneumococcal strain-specific antibody concentrations after the PPV23 in PaedRD on methotrexate at time of vaccination. | C | 7.9 (3.4) |
| LIVE-ATTENUATED VACCINES | |||
| 6 | Until more data are available, it is recommended to withhold live-attenuated vaccines in patients on high-dose DMARD,† high-dose glucocorticosteroids† or biological agents. However, vaccination can be considered on a case-to-case basis weighing the risk of infections vs the hypothetical risk of inducing infections by vaccination. | D | 9.2 (0.9) |
| 7 | It is recommended to adhere to national vaccination guidelines for live-attenuated vaccines in PaedRD unless patients are on high-dose DMARD,† high-dose glucocorticosteroids† or biological agents. Boosters vaccinations against VZV, MMR and YFV can be considered in patients on methotrexate <15 mg/m2/week or low-dose glucocorticosteroids. | C | 8.9 (1.5) |
| 8 | It is recommended to withhold BCG vaccination during active Kawasaki disease. | C | 9.5 (0.9) |
| 9 | It is recommended to assess VZV infection and vaccination history in PaedRD, especially in those patients anticipating high-dose immunosuppressive therapy† or biologicals. In case of a negative history for VZV infection or vaccination, VZV vaccine should be considered, ideally before initiation of immunosuppressive therapy.‡ | D | 9.2 (1.2) |
| NON-LIVE VACCINES | |||
| 10 | The TT vaccine should be administered to patients with juvenile SLE and JIA according to the national vaccination guidelines. | B | 9.8 (0.6) |
| 11 | It is recommended to adhere to national vaccination guidelines* for vaccination against hepatitis B virus, tetanus, diphtheria, pertussis, Hib, pneumococci and meningococci in PaedRD. | C | 9.8(0.6) |
| 12 | It is recommended to adhere to national vaccination guidelines* for vaccination against hepatitis A virus, poliovirus, Japanese encephalitis, typhoid fever, rabies, cholera or tickborne encephalitis in PaedRD. | D | 8.9 (1.8) |
| 13 | Annual influenza vaccination should be considered in all PaedRD. | D | 8.4 (2.2) |
| 14 | In the case vaccinations against Hib, pneumococci and meningococci are not included in the national vaccination programmes*, these vaccinations are recommended for PaedRD with low complement levels or functional asplenia. These vaccinations can be considered in patients on high-dose immunosuppressive drugs† or biological agents before therapy.‡ | D | 9.3 (1.6) |
| 15 | It is recommended to adhere to national vaccination guidelines* for vaccination against HPV in PaedRD. Given the higher risk of HPV infection in female SLE patients, these patients should be advised to be vaccinated in the adolescence. However, physicians should be vigilant on potential thromboembolic events. | D | 9.2 (1.6) |
↵* National vaccination guidelines worldwide can be found on http://apps.who.int/immunization_monitoring/en/globalsummary/scheduleselect.cfm.
↵† High-dose DMARD are defined as intravenous pulse therapy, cyclosporine >2.5 mg/kg per day, sulphasalazine >40 mg/kg per day or 2 g/day, azathioprine >3 mg/kg, cyclophosphamide orally >2.0 mg/kg per day, leflunomide >0.5 mg/kg per day, or 6-mercaptopurine >1.5 mg/kg per day. High-dose glucocorticosteroids are dosages ≥2 mg/kg or ≥20 mg/day for 2 weeks or more. In patients chronically treated with 20 mg/day glucocorticosteroids, dosages below 2 mg/kg per day are also considered high dosages.
↵‡ Generally 2–4 weeks is recommended before immunosuppressive therapy is commenced.
BCG, bacillus Calmette–Guérin; DMARD, disease-modifying antirheumatic drugs; Hib, Haemophilus influenzae type B; HPV, human papillomavirus; JIA, juvenile idiopathic arthritis; MMR, measles, mumps, rubella; PaedRD, paediatric patients with rheumatic diseases; PPV23, 23-valent pneumococcal polysaccharide; RC, recommendation; SLE, systemic lupus erythematosus; TNF, tumour necrosis factor; TT, tetanus toxoid; VZV, varicella zoster virus; YFV, yellow fever virus.