Table 1 Main serum markers, clinical features and radiological patterns of autoimmune lung CTDs
CTD | Serum markers | Clinical features | HRCT features |
|---|---|---|---|
RA | Anti-CCP, RF | Erosive and symmetric inflammatory arthropathy, subcutaneous nodules, skin ulceration Lung involvement: exertional dyspnea non-productive cough, bibasilar inspiratory crackles (edema, cyanosis and signs of pulmonary hypertension) | UIP, NSIP patterns Airway disease with obliterative and follicular bronchiolitis Rheumatoid nodules |
SSc | ACA Anti-Scl70, anti-RNA polymerase III | Skin thickening, telangiectasias, fingertip ulcers, gastrointestinal diseases, myocardial diseases Lung involvement: exertional dyspnoea, non-productive cough fatigue, “velcro” crackles | NSIP pattern, UIP pattern with straight-edge sign and/or “Four Corners” sign PH |
IIM | Increased CK levels; positivity for MSAs/MAAs | Muscle weakness, systemic involvement and specific cutaneous manifestations (for DM heliotrope rash and Gottron papules) Lung involvement: (a) rapidly progressive dyspnea and respiratory failure. (b) insidious onset of dyspnoea | Hypoventilation and respiratory failure due to respiratory muscles involvement; aspiration pneumonia NSIP or OP pattern |
SLE | ANA Anti dsDNA antibodies; anti-Sm; lupus anticoagulant Ro/SSA antibodies; hypocomplementemia | Rheumatological, dermatological and renal abnormalities Lung involvement: acute symptoms (dyspnoea, respiratory failure)—in cases of hemorrhage of ALP; dyspnea and cough in interstitial pneumonitis | Pleuritis, pleural effusions, pulmonary infections, chronic interstitial lung diseases (with NSIP pattern), ALP, DAH |
pSS | ANA, anti-Ro60kD; anti-Ro52kD; anti-La | Xerostomia and xerophthalmia, focal lymphocytic sialadenitis Lung involvement: it could be asymptomatic In symptomatic cases, cough and dyspnea are often present | NSIP pattern LIP with diffuse interstitial and peribronchiolar infiltration of lymphoplasma cells OP pattern |
MCTD | Anti-U1 RNP, anti-Ro52 antibodies | Patients are often asymptomatic Dysphagia and RP, diffuse hand edema (puffy hands) | NSIP, UIP; OP and DAD have been occasionally described |