Table 2

Patient evaluation and treatment at diagnosis, at 18 months and at 4–5 years of follow-up

At diagnosisAt 18 months follow-upAt 4–5 years of follow-up
Age groups and number of patients (n)<10 years group
(14)
≥10 years group
(27)
<10 years group
(14)
≥10 years group
(24)
<10 years group
(10)
≥10 years group
(19)
Lung function tests
Mean values (SD)
 FVC % pred62 (±26)61 (±22)76 (±12)76 (±15)80 (±12)74 (±13)
 CLdyn % pred45 (±15)43 (±1)66 (±30)65 (±13)100, 80 and 34*55 and 74*
 TLCO % pred96 (±48)61 (±19)82 (±12)76 (±21)78 (±8)79 (±23)
Treatment
Number of patients (%)
 Steroids
  Oral14 (100%)23 (85%)5 (36%)12 (50%)4 (40%)8 (42%)
  Intravenous pulses11 (79%)11 (41%)†7 (50%)4 (17%)7 (70%)5 (26%)
Immunosuppressive drugs003 (21%)12 (50%)3 (30%)7 (37%)
Outcome
Number of patients (%)
 Recovery7 (50%)7 (29%)5 (50%)8 (42%)
 Stabilisation3 (21%)3 (13%)2 (20%)2 (10%)
 Relapses4 (29%)14 (58%)†3 (30%)9 (47%)
  • Pulmonary function tests were expressed as a percentage of the predicted values (% pred) for height-matched children (±SD). Qualitative values are expressed as number of children (%).

  • *Only 3 patients tested in the <10 years old group and 2 in the >10 years old group.

  • †p Value ≤0.05.

  • CLdyn, dynamic lung compliance; TLCO, lung transfer factor for carbon monoxide.