Table 1

Interstitial lung diseases associated with connective tissue diseases

Rheumatic disease

Frequency of ILD (%)


Systemic sclerosis

45 (clinically significant)

More common in diffuse disease; topoisomerase-1 antibodies

Rheumatoid arthritis

20 to 30

Increased risk with cigarette smoking


20 to 50a

More common with anti-synthetase antibodies

Sjögren's syndrome

Up to 25


Systemic lupus erythematosus

2 to 8

Usually in patients with multisystem disease

Mixed connective tissue disease

20 to 60


ILD, interstitial lung disease. aFrequency may be higher based on recent studies.

Castelino and Varga Arthritis Research & Therapy 2010 12:213   doi:10.1186/ar3097