Agata Gierlotka, Ireneusz Bielecki
Systemic bevacizumab appears to be an effective and well-tolerated disease-modifying therapy for children with severe JoRRP. The available evidence supports its use as an adjuvant treatment in patients with aggressive or pulmonary disease; however, prospective multicenter studies are needed to establish optimal treatment protocols and long-term outcomes.
OBJECTIVE: To systematically evaluate the efficacy and safety of systemic bevacizumab in children with JoRRP.
METHODS: A systematic literature review was conducted according to the PRISMA 2020 guidelines. MEDLINE, Embase, ScienceDirect, and the Cochrane Library were searched from database inception to June 30, 2026. Studies reporting pediatric patients (<18 years) with JoRRP treated with intravenous bevacizumab were included. Data regarding patient characteristics, treatment protocols, clinical outcomes, and adverse events were extracted.
RESULTS: Twenty-two studies involving 74 pediatric patients met the inclusion criteria. Systemic bevacizumab consistently reduced disease burden, prolonged intervals between surgical procedures, and decreased the need for repeated operative interventions. Complete clinical remission was reported in six children, and successful tracheostomy decannulation was achieved in four patients. Regression of pulmonary lesions was observed in several cases with distal airway involvement. Treatment was generally well tolerated. Adverse events were predominantly mild and transient, with no permanent treatment discontinuation due to toxicity.
CONCLUSIONS: Systemic bevacizumab appears to be an effective and well-tolerated disease-modifying therapy for children with severe JoRRP. The available evidence supports its use as an adjuvant treatment in patients with aggressive or pulmonary disease; however, prospective multicenter studies are needed to establish optimal treatment protocols and long-term outcomes.