Table 2 IPAF Classification criteria of patients from the study: clinical domain; serological domain and morphological domain

Clinical domain (n = 54), n (%)

 Raynaud’s phenomenon

19 (35, 8)

 Arthralgia / arthritis

25 (46, 2)

 Distal digital fissuring

5 (9, 3)

 Unexplained digital edema

4 (7, 4)

 Palmar telangiectasia

1 (1, 85)

Serological domain, n (%)

 ANA ≥ 1/320 (n = 73)

59 (80.8)

 Anti-Ro (n = 72)

18 (25)

 RF ≥ 40 (n = 65)

19 (29.2)

 Anti-CCP (n = 50)

6 (12)

 Anti-RNP (n = 69)

4 (5.7)

 Anti-PM Scl (n = 62)

1 (1.6)

Morphological domain (n = 79)

 Radiologic patterns by HRCT, n (%)

  NSIP

37 (46.8)

  NSIP/COP

2 (2.5)

  COP

1 (1.3)

  LIP

1 (1.3)

  UIP

29 (36.7)

  Others

9 (11.4)

 Histopathological patterns by lung biopsy, n = 18 (HCSC y FJD), n

  NSIP

3

  UIP

5

  Interstitial lymphoid aggregates with germinal centers

5

  OP

1

  NSIP/OP

2

  Others

2

 Multi-compartment involvement, n (%)

  Unexplained pulmonary vasculopathy (n = 54)

4 (7,4)

  Unexplained intrinsic airways disease (n = 54)

3 (5,5)

  Unexplained pulmonary vasculopathy (n = 54)

4 (7,9)

  Unexplained pericardial/pleural effusion/thickening (n = 54)

3 (5,5)