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Scleroderma
 

• 90% of patients with disease have lung disease but only about 25% have abnormal chest x-rays

• 3:1 female to male ratio

• Rheumatoid factor found in 35%

• Increased incidence of lung cancer: alveolar cell

• Pulmonary fibrosis is uncommon in CREST but pulmonary arterial hypertension can occur
  

X-ray

• Diffuse reticular interstitial disease, primarily at bases

• Proceeds from fine to coarse reticulation

• Alveolar infiltrates 2° aspiration from disturbed esophageal motility may occur

• Honeycombing can be found

• Progressive volume loss (unlike other causes of diffuse fibrosis except Hamman-Rich)

• Pleural disease is rare (unlike RA and lupus)

WH/’91

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