Table 4 Consensus criteria to stop or not start ERT

Non-compliance > 50% of infusions

Class I

Failure to attend regularly (according to local guidelines) at FU visits

Class I

Persistent life threatening or severe infusion reactions that do not respond to prophylaxis, e.g. anaphylaxis

Class I

Patient request

Class I

End stage renal disease, without an option for renal transplantation, in combination with advanced heart failure (NYHA class IV)

Class IIA

End stage FD or other comorbidities with a life expectancy of < 1 year

Class IIB

Severe cognitive decline of any cause

Class IIB

Lack of response for 1 year when the sole indication for ERT is neuropathic pain while receiving maximum supportive care*

Class IIB

Criteria for not starting ERT

Class of recommendation

Advanced cardiac disease with extensive fibrosis [37] if cardiac disease is the sole treatment indication^†

Class I

End stage renal disease, without an option for renal transplantation, in combination with advanced heart failure (NYHA class IV)

Class IIA

End stage FD or other comorbidities with a life expectancy of < 1 year

Class IIB

Severe cognitive decline of any cause

Class IIB