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Table 1 Demographic, laboratory, physiologic, and radiologic data and treatments in patients with AE-SAID-ILD

From: Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases

 

AE-SAID-ILD (n = 25)

Age, year

72 (55, 85)

Sex, male / female

17 / 8

Smoking, never / ex / current

6 / 18 / 1

Pack-year of smoking

27.5 (0, 144)

Surgical lung biopsy, +/-

6 / 17

Period from ILD diagnosis to first AE, mo

74 (0, 226)

Period from SAID diagnosis to first AE, mo

64 (0, 365)

ILD preceding SAID, +/- 

7 / 18

Observation period, day

60 (0, 228)

Data before AE a

 FVC, % pred

79.1 (49, 111)

 DLCO, % pred

62.1 (39.3, 82.0)

 PaO2 at rest, Torr

77.3 (61.3, 86.4)

 UIP pattern on HRCT, +/- / unknown

12 / 10 / 3

 Extent scores on HRCT (full score: 25)

12 (2, 20)

 JRS severity grade, I / II / III / IV / unknown

6 / 0 / 3 / 1 / 15

 The GAP staging system, I / II / III / unknown

8 / 3 / 0 / 14

 Preceding immunosuppressive treatments, +/- 

20 / 5

 Preceding antifibrotic treatments, +/- 

1 / 24

 Preceding oxygen therapy, +/- 

4 / 21

Data at AE

 Peripheral blood WBC, × 103/μL

11.6 (7.4, 22.8)

 Peripheral blood Neut, × 103/μL

10.7 (6.3, 19.4)

 Serum CRP, mg/dL

10.2 (1.5, 31.8)

 Serum LDH, IU/L

405 (202, 815)

 Serum KL-6, U/mL

1463 (340, 4341)

 Serum SP-D, ng/mL

329 (74, 2950)

 P/F ratio

180 (57, 328)

 Extent scores on HRCT at AE (full score: 25)

18.5 (10, 25)

 HRCT pattern at AE, peripheral / multifocal / diffuse / unknown

2 / 3 / 19 / 1

 Administration of steroid pulse therapy b, +/- 

25 / 0

 Duration from admission to starting treatments for AE, days

2 (0, 13)

 Administration of Immunosuppressants, +/- 

13 / 12

 Treatment with PMX-DHP, +/- 

8 / 17

 Duration from admission to starting PMX-DHP, days

2 (0, 16)

 Intubation, +/- 

6 / 19

  1. Data presented as median (range) or n (%)
  2. Abbreviations: AE Acute exacerbation, SAID-ILD Systemic autoimmune disease-related interstitial lung disease, FVC Forced vital capacity, DLCO Diffusion lung capacity for carbon monoxide, UIP Usual interstitial pneumonia, HRCT High-resolution computed tomography, JRS Japanese Respiratory Society, GAP Gender, age, and physiology, PF-ILD Progressive fibrotic interstitial lung disease, WBC White blood cells, Neut Neutrophils, CRP C-reactive protein, LDH Lactate dehydrogenase, KL-6 Krebs von den Lungen-6, SP-D Surfactant protein D, P/F PaO2/FiO2, PMX-DHP Direct haemoperfusion with a polymyxin B-immobilised fibre column
  3. a Pulmonary function tests and severity scores were evaluated within 12 months before AE-SAID-ILD
  4. b Methylprednisolone 1,000 mg/day for 3 days