Table 2

Clinical course of physiological and radiological findings in IDPO

No of case and data during follow-up
Change of clinical symptoms
Development and deterioration of dyspnoea on exertion3 (14%)
Drug therapy* (n=20)2 (9%)
Oxygen therapy0
Deterioration of pulmonary ossification on HRCT (n=17)
 Stable2 (12%)
 Progression15 (88%)
Survival
 Alive20 (91%)
 Unknown2 (9%)
Changes of pulmonary function tests
FVC decline (n=17)
 ≥100 mL/year4 (24%)
 <100 mL/year and ≥50 mL/year6 (35%)
 <50 mL/year7 (41%)
FVC, %predicted relative decline (n=17)
 ≥5% /year2 (12%)
 <5% /year and ≥2.5% /year3 (18%)
 <2.5% /year12 (71%)
FEV1 decline (n=17)
 ≥100 mL/year4 (24%)
 <100 mL/year and ≥50 mL/year6 (35%)
 <50 mL/year7 (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.