Table 3

Treatment and clinical course

Case	Diagnostic delay^3-150	Treatment	Duration of follow up (months)	Symptomatic change	FEV₁ change (%)^3-151	Tlco change (%)^3-152	Final result
2	2 years	MP + tamoxifen	13	Deterioration	−17.9	−2	Progression
3	12 months	MP + tamoxifen	43	Deterioration	−59.7	−47	Progression
4	3 years	MP + tamoxifen	29	Deterioration	−59.3	−2	Progression
5	2 years	MP + tamoxifen	44	No change	20.8	−18	Stable
6	3 years	MP	54	Deterioration	−46.2	−20	Death from respiratory insufficiency
8	9 years	MP + tamoxifen	25	Deterioration	−29.5	9	Death from infection following transplantation
15	3 years	MP	15	Deterioration	−34.9	−4	Progression
17	4 years	Tamoxifen	12	Deterioration^3-153	NE	NE	Death from respiratory insufficiency
20	2 months	MP	12	No change	3.0	NE	Stable
21	7 years	MP	12	No change	−8.3	−3	Progression

MP = medroxyprogesterone; FEV₁ = forced expiratory volume in one second; Tlco = carbon monoxide lung transfer factor; NE = not examined.
↵3-150 The time between symptom onset and diagnosis.
↵3-151 FEV₁ change (%) = percentage of (final FEV₁— initial FEV₁)/initial FEV₁.
↵3-152 Tlco change (%) = final Tlco (% predicted) — initial Tlco (% predicted).
↵3-153 She experienced three pneumothoraces, two of which were during pregnancy.