Scleroderma Lung Disease
Pathophysiology: One of a group of collagen vascular
diseases (CVD). A multisystem disease involving the skin,
muscles, bones and the lungs.
In
the thorax: an immunologic small vessel vasculitis develops in
the alveolar septi and small lung vessels. May sometimes
involve serosal linings ie. pleura and pericardium. The process
leads to diffuse alveolar damage (DAD), interstitial fibrosis, loss of
lung compliance and may progress to an end stage lung.
Esophageal muscle involvement causes dilation of esophagus and
aspiration pneumonia may complicate the findings.
Clinical Clues: look for multiple system involvement,
esophageal dysmotility and with dilation and recurrent
aspiration
CXR/CT Findings:
-
linear interstitial changes in
the lower 2/3 of the lungs
-
absence of nodular changes; no
gross adenopathy
-
may be pleural reaction or
effusion (not typical), or pericardial effusion (uncommon)
-
dilated esophagus often with an
air fluid level especially seen on CT
-
fibrosis and volume loss in the
lower lung
-
may seen honeycombing and end
stage lung disease changes
“Aunt Sophies”: a gamut
of lower lung interstitial disease with volume loss
-
Idiopathic pulmonary fibrosis (UIP etc…)
-
Rheumatoid lung disease
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Chronic repeated aspiration and
fibrosis
-
Dermatomyositis (rare)
-
Drug reactions (usually not
lower lung specific)
-
Asbestosis
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