Table 1.
—Proposed Provisional Criteria for Lung-Dominant CTD
1. NSIP, UIP, LIP, OP, and DAD (or DIP if no smoking history), as determined by surgical lung biopsy specimen or suggested by high-resolution CT and | |
2. Insufficient extrathoracic features of a definite CTD to allow a specific CTD designation and | |
3. No identifiable alternative etiology for IP and | |
4. Any one of the following autoantibodies or at least two of the histopathology features: | |
Autoantibodies | Histopathology features |
a. High-titer ANA (> 1:320) or RF (> 60 IU/mL) | (a) Lymphoid aggregates with germinal centers |
b. Nucleolar-ANA | (b) Extensive pleuritis |
c. Anti-CCP | (c) Prominent plasmacytic infiltration |
d. Anti-Scl-70 | (d) Dense perivascular collagen |
e. Anti-Ro | |
f. Anti-La | |
g. Anti-dsDNA | |
h. Anti-Smith | |
i. Anti-RNP | |
j. Anti-tRNA synthetase (eg, Jo-1, PL-7, PL-12, and others) | |
k. Anti-PM-Scl | |
l. Anticentromere |
ANA = antinuclear antibody; CCP = cyclic citrullinated peptide; CTD = connective tissue disease; DAD = diffuse alveolar damage; DIP = desquamative interstitial pneumonia; IP = interstitial pneumonia; LIP = lymphocytic interstitial pneumonia; NSIP = nonspecific interstitial pneumonia; OP = organizing pneumonia; RF = rheumatoid factor; RNP = ribonucleoprotein; UIP = usual interstitial pneumonia.