No of case and data during follow-up | |
Change of clinical symptoms | |
Development and deterioration of dyspnoea on exertion | 3 (14%) |
Drug therapy* (n=20) | 2 (9%) |
Oxygen therapy | 0 |
Deterioration of pulmonary ossification on HRCT (n=17) | |
Stable | 2 (12%) |
Progression | 15 (88%) |
Survival | |
Alive | 20 (91%) |
Unknown | 2 (9%) |
Changes of pulmonary function tests | |
FVC decline (n=17) | |
≥100 mL/year | 4 (24%) |
<100 mL/year and ≥50 mL/year | 6 (35%) |
<50 mL/year | 7 (41%) |
FVC, %predicted relative decline (n=17) | |
≥5% /year | 2 (12%) |
<5% /year and ≥2.5% /year | 3 (18%) |
<2.5% /year | 12 (71%) |
FEV1 decline (n=17) | |
≥100 mL/year | 4 (24%) |
<100 mL/year and ≥50 mL/year | 6 (35%) |
<50 mL/year | 7 (41%) |
DLCO, %predicted decline ≥15% /year (n=11) | 0 |
Data are n of 22 IDPO cases (%), except for the indicated subjects. The average follow-up period was 6.36 (5.85) years.
*Drug; inhaled steroid and long-acting β2 stimulants:1, expectorant and antitussive:1.
DLco, diffusing capacity of the lung for carbon monoxide; FEV1, forced expiratory volume in 1 s; FVC, forced vital capacity; HRCT, high-resolution CT; IDPO, idiopathic dendriform pulmonary ossification.