Features suggesting CTD | |
Demographics | Female sex Age <50 years |
Clinical manifestations | Any patient with extra-thoracic manifestations highly suggestive of CTD: |
Raynaud’s phenomenon, oesophageal hypomotility, inflammatory arthritis of the metacarpophalangeal joints or wrists, digital oedema, symptomatic keratoconjonctivitis sicca | |
Lung histopathology | All cases of NSIP, LIP or ILD patterns with histopathology features that might suggest CTD: |
Extensive pleuritis, dense perivascular collagen, lymphoid aggregates with germinal centre formation, prominent plasmacytic infiltration | |
Biology | Patients with positive high-titre antinuclear antibodies (>1:320) or rheumatoid factor (>60 IU·mL−1), nucleolar staining antinuclear antibodies or specific antibodies (anti-cyclic citrullinated peptide, anti-Scl70, anti-Ro, anti-La, anti-dsDNA, anti-Smith, anti-RNP, anti-tRNA synthetase) |
CTD: connective tissue disease; NSIP: nonspecific interstitial pneumonia; LIP: lymphoid interstitial pneumonia; ILD: interstitial lung disease; RNP: ribonucleoprotein. Adapted from [37].