Table 1 Characteristics of the study population

Demographic and clinical characteristics

Patients (n = 121)

 Women: number (%)

64 (53)

 Age at examination in years: median (range)

53 (23–79)

 Disease duration^a at examination in years: median (range)

16 (0.2–50)

 Wheelchair^b use at examination: number (%)

54 (45)

 Respiratory support at examination: number (%)

32 (26)

Physical outcomes

Median (range)

 FVC upright (% pred)

77 (10–117)

 FVC supine (% pred)

63 (13–107)

 HHD sum score (% max)

81 (21–100)

 MRC sum score (% max)

85 (39–100)

 6MWT (% pred)

67 (6–120)

PROMs

Median (range)

 SF-36 PCS score (norm-based)

33 (17–63)

 SF-36 MCS score (norm-based)

48 (19–72)

 RHS score

29 (11–36)

 R-PAct score

60 (7–100)