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Table 4 Clinical, functional, survival and baseline data of patients with CPFE and ANA + ANCA + immunologic profile with and without positive renal biopsy for MPA

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

Variable

ANA + ANCA + with positive renal biopsy for MPA

ANA + ANCA + without positive renal biopsy for MPA

Patients (n)

3

4

Renal function

Renal failure

normal

Hematuria (n)

3

4

Hemoptysis (n)

2

0

Gas exchange impairment

Respiratory failure

Mild

Treatment

Pulses of methyprednisolone (1gr for 3 consecutive days) and cyclophosphamide (1gr for one day) plus oral cyclophosphamide (60 mgr/day) and oral prednisolone (60 mg/day) for 7 months

Azathioprine (2mg/kgr/day), prednisolone (20mgr/day), NAC (1800mgr/day) for 12 months

Death

1

0

FVC%pred. before Rx (∫)

60,5 ± 18,8

76,2 ± 24,5

FVC%pred. after Rx (§)

57,8 ± 17,1

70,3 ± 21,8

DLCO%pred. before Rx (∫)

29,5 ± 9,8

37,3 ±11,2

DLCO%pred. after Rx (§)

26,5 ± 10,1

35,9 ± 10,9

  1. Abbreviations: ANA: anti-nuclear antibodies, ANCA: antineutrophil cytoplasm antibodies (ANCAs), DL CO : Diffusing capacity of lung for carbon monoxide, FVC: Forced Vital Capacity, MPA: Microscopic polyangiitis, NAC: N-acetylcysteine, Rx: Treatment. *p < 0.05.
  2. ∫ Pulmonary function tests 3 months before treatment initiation.
  3. § Pulmonary function tests after treatment initiation (7 and 12 months).