Pleuroparenchymal Fibroelastosis: A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias

Table 1

Anthropometric and functional characteristics of two cases with pleuropulmonary fibroelastosis.

	Case	Case
Age (yr)	25	40
BMI (m/kg^2)	21	19
FVC (%pred)	35	37
FEV1 (%pred)	44	34
FEV1/FVC (%)	98	75
TLC (L)	4.44	3.22
TLC (%pred)	67	61
IC (L)	1.08	0.77
IC (%pred)	32	31
RV (L)	2.45	1.92
RV (%pred)	150	113
RV/TLC (%)	227	179
IC/TLC ratio	24	23
(%pred)	49	ND
KCO	88	ND
6 MWT (mt)	268	518
Resting SatO2	91	97
Post-6 MWT SatO2	81	83

BMI, body mass index; FVC, forced vital capacity; FEV₁, forced expiratory volume in 1 s; FEV₁/FVC, forced expiratory volume in 1 s/forced vital capacity ratio; TLC, total lung capacity; RV, residual volume; RV/TLC, residual volume/total lung capacity ratio; IC, inspiratory capacity; IC/TLC, inspiratory capacity/total lung capacity ratio; , diffusing capacity for carbon monoxide; KCO, carbon monoxide transfer coefficient for alveolar volume; ND: not done; 6 MWT, six-minute walk test; SatO₂: oxygen saturation.