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Table 2 Final clinical diagnoses and distribution of positive autoimmune serologies and suggestive clinical signs or symptoms of autoimmune disease

From: Utility of autoimmune serology testing in the assessment of uncharacterized interstitial lung disease: a large retrospective cohort review

ILD type

Serology obtained (N = 605)

Positive serologyb

Positive clinical signs or symptoms suggestive of autoimmune diseasec

Duration of disease cohort follow-up, months (median (IQR))

Subsequent development of CTD after initial visit, N (%)

IPF (N, (%))

197 (32.6)

51 (25.8)

19 (6.9)

9.2 (1.05–35.1)

0

Unclassifiable ILD (N, (%))

146 (24.1)

46 (31.5)

24 (10.8)

12.6 (0.3–47.5)

3

Other IIP (N, (%))

92 (15.2)

24 (26.1)

21 (15.9)

9.7 (0.6–52.8)

1

Hypersensitivity pneumonitis (N, (%))

70 (11.6)

22 (31.4)

10 (9)

9 (0.6–44.7)

0

CTD-ILD ((N, (%))

42 (6.9)

41(98)

38 (90)

14.8 (0.9–66.6)

-

Drug induced/Environmental (N, (%))

15 (2.5)

4 (26.7)

0

6.2 (1.7–50)

0

Rare ILDa (N, (%))

11 (1.8)

3 (27)

2 (7.1)

16.2 (0.1–59.7)

0

IPAF (N, (%))

18 (2.9)

14 (78)

13 (72)

17.3 (1.8–55.1)

0

Sarcoidosis (N, (%))

5 (0.82)

2 (40)

1 (9)

4.1 (0.6–33.8)

0

CPFE (N, (%))

4 (0.66)

2 (50)

0

28.4 (3.3–55.1)

0

Aspiration-related fibrosis (N, (%))

1 (0.16)

0 (0)

1 (16.6)

55.7 (−)

0

ANCA vasculitis related ILD (N, (%))

4 (0.66)

4 (100)

4 (80)

80.9 (25–96.7)

0

  1. Abbreviations: ANCA anti-neutrophilic cytoplasmic antibodies, CTD-ILD connective tissue disease-related interstitial lung disease, CPFE combined pulmonary fibrosis and emphysema, IIP idiopathic interstitial pneumonia, ILD interstitial lung disease, IPAF interstitial pneumonia with autoimmune features, IPF idiopathic pulmonary fibrosis, IQR interquartile range
  2. aRare ILD includes lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, pulmonary amyloidosis, pulmonary alveolar proteinosis, Williams-Campbell syndrome, IgG4 sclerosing lung disease, ILD in dyskeratosis congenita, Birt-Hogg-Dubé syndrome
  3. bpercentages are positive laboratory findings over those with obtained serology
  4. cpercentages are for positive review of systems for the disease type, inclusive of serology screened and non-screene