Abstract

Results Fever at onset, high painVAS and parental VAS scores highly correlated with risk of relapse disease course. Treatment: effectiveness of NSAID only 3/10 (30%), SSZ - 1/5 (20%), corticosteroids - 0/3 (short-term effect only), MTX - 4/7 (57.1%), pamidronate (PAM) with partial response 2/12 (16.7%) and with complete response 10/12 (83.3%). Biologics - adalimumab and etanercept were effective in 3/4 (75%) patients, who fail to NSAID, MTX, PAM and SSZ. During disease course treatment lead to decreasing sings of disease activity, such as: parental VAS (p = 0.015), pain VAS (p = 0.026), MDVAS (p = 0.026), CRP (p = 0.0008), WBC (p = 0.006), ESR (p = 0.00024), PLT (0.014). The main effectiveness belonged to PAM (p = 0.003) and biologics (p = 0.07) in decreasing of pain VAS (-100% and -80%), parental VAS (-92% and -74%) and MD VAS (-93% and -70%, respectively). We calculated the cumulative probability of survival (event of interest: CNO flare) in the entire patient sample, depending the kind of treatment (PAM, MTX and NSAID) obtained by the Kaplan-Meier method. Significant difference was proved comparing 3 therapeutical branches (p = 0.028). MTX treatment was effective (p = 0.04), as well as PAM (p = 0.01) than NSAID. Only flulike syndrome during PAM treatment was in 10/12 (83.3%). No any others side effects were reported. All patients who had flu-like syndrome on first infusion had complete response to PAM, vice verse patients, who had no such complication had only partial response to this treatment.

Highlights

  • Pediatric chronic nonbacterial osteomyelitis (CNO) is a sterile inflammatory bone disorder in which innate and adaptive immunity dysfunction involved

  • Biologics - adalimumab and etanercept were effective in 3/4 (75%) patients, who fail to non-steroid anti-inflammatory drugs (NSAIDs), MTX, PAM and SSZ

  • During disease course treatment lead to decreasing sings of disease activity, such as: parental VAS (p = 0.015), pain VAS (p = 0.026), MDVAS (p = 0.026), CRP (p = 0.0008), WBC (p = 0.006), ESR (p = 0.00024), PLT (0.014)

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Summary

Introduction

Pediatric chronic nonbacterial osteomyelitis (CNO) is a sterile inflammatory bone disorder in which innate and adaptive immunity dysfunction involved. The modern treatment modalities include non-steroid anti-inflammatory drugs (NSAIDs), steroids, sulfasalazine (SSZ), methotrexate (MTX), bisphosphonates and biologic drugs TNFa and IL1b-antagonists, with limited data

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