In a cohort of 92 people with FSHD1 (n=88) or FSHD2 (n=4), the number of participants with a restrictive syndrome increased from 41 to 48 over five years.
- Mean vital capacity (VC) decreased from 79% to 76.7%, with individual variations ranging from a decrease of 33.5% to an increase of 23.6%.
- The Tiffeneau coefficient increased from 86.3% to 89.7% in five years, with variations ranging from a decrease of 29% to an increase of 40.6%.
- Seventy-seven participants (83.6%) had stable CV over the five years, eleven had a CV decline of more than 10% and four had a decline of more than 20%, with no significant difference in their initial assessment of lung function.
- The 15 participants in the progressive subgroup (16.3%) were older (56.3 ± 13.2 years versus 46.8 ± 15.3 years), had been ill for a longer period of time (33.7 ± 16.9 years versus 18.9 ± 15.8 years) and had more severe initial muscle damage than the other participants.
