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Table 2 Clinical, functional, radiological and histopathological characteristics of CPFE patients according to their immunologic profile

From: Increased incidence of autoimmune markers in patients with combined pulmonary fibrosis and emphysema

Variable

ANA + ANCA+

ANA + ANCA-

ANA-ANCA-

Total Number

7

10

23

FEV1%pred

69,2 ± 17,1

67,8 ± 14,3

68,4 ± 18,7

FVC%pred

72,87 ± 26,6

68,96 ± 22,9

69,54 ± 21,6

DLCO%pred

35,95 ± 12,3

35,79 ± 10,7

33,9 ± 10,4

Histopathology pattern

UIP

UIP

UIP

CD20 + cells/mm2

51.6 ± 6.9*

49.6 ± 7.1*

6.2 ± 3.6

Survival (median-range) months

51 (12 – 96)*

38 (16 – 61)

Radiology pattern

UIP

UIP

UIP

sPAP

36,9 ± 13,1

38,2 ± 12,8

37,1 ± 11,5

Hemoptysis

7

0

0

Hematuria

7

0

0

Arthralgias

0

3

0

Raynaud’s phenomenon

0

4

0

Dysphagia

0

1

0

  1. Abbreviations: ANA: anti-nuclear antibodies, ANCA: antineutrophil cytoplasm antibodies (ANCAs), DL CO : Diffusing capacity of lung for carbon monoxide, FVC: Forced Vital Capacity, sPAP: systolic pulmonary artery pressure, UIP: Usual Interstitial Pneumonia. *p < 0.05.