Table 1 Autoimmune features of 54 IPAF patients according to criteria and domains
Clinical domain (n = 17) | |
|---|---|
Distal digital fissuring | 1 (5.9) |
Distal digital tip ulcerations | 0 (0) |
Inflammatory arthritis or polyarticular morning joint stiffness ≥60 min | 13 (76.5) |
Palmar telangiectasia | 0 (0) |
Raynaud’s phenomenon | 3 (17.6) |
Unexplained digital oedema | 3 (17.6) |
Unexplained fixed rash on the digital extensor surface | 0 (0) |
Serologic domain (n = 49) | |
ANA ≥1:320 titer, diffuse, speckled or homogeneous patterns, ANA nucleolar pattern (any titer), or ANA centromere pattern (any titer) | 31 (63.3) |
Rheumatoid factor > 2x upper limit of normal | 14 (28.6) |
Anti-CCP | 7 (14.3) |
Anti-dsDNA | 3 (6.1) |
Anti-Ro (SS-A) | 4 (8.2) |
Anti-La (SS-B) | 1 (2.0) |
Anti-topoisomerase (Scl-70) | 1 (2.0) |
Anti-ribonucleoprotein | 2 (4.1) |
Anti-Smith | 2 (4.1) |
Anti-tRNA synthetase, Anti-Pm-Scl, Anti-MDA-5 | 0 (0) |
Morphologic domain (n = 44) | |
Suggestive radiology patterns by HRCT | 39 (72.2) |
Nonspecific interstitial pneumonia | 34 (87.2)a |
Organising pneumonia | 3 (7.7)a |
Nonspecific interstitial pneumonia with organising pneumonia overlap | 2 (5.1)a |
Lymphoid interstitial pneumonia | 0 (0)a |
Histopathologic pattern | 12 (22.2) |
Nonspecific interstitial pneumonia | 0 (0)a |
Organising pneumonia | 4 (33.3)a |
Nonspecific interstitial pneumonia with organising pneumonia overlap | 1 (8.3)a |
Interstitial lymphoid aggregates with germinal centres | 5 (41.7)a |
Diffuse lymphoplasmacytic infiltration | 2 (16.7)a |
Multi-compartment involvement (in addition to interstitial pneumonia) | 11 (20.4) |
Unexplained pleural effusion or thickening | 5 (45.5)a |
Unexplained pericardial effusion or thickening | 3 (27.3)a |
Unexplained intrinsic airways diseases | 2 (18.2)a |
Unexplained pulmonary vasculopathy | 1 (9.1)a |