Scleroderma with interstitial fibrosis
Citation, DOI, disclosures and case data
At the time the case was submitted for publication Natalie Yang had no recorded disclosures.
View Natalie Yang's current disclosuresPresentation
Shortness of breath.
Patient Data
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There are characteristic changes of pulmonary fibrosis, particularly in the bases and in the subpleural lung. Honeycomb change, intralobular septal thickening and traction bronchiectasis are all present. No pleural effusion or obvious nodal enlargement is visible.
The esophagus is dilated and air filled.
6 case questions available
Q: What is the striking abnormality within the chest? show answer
A: A honeycomb appearance with traction bronchiectasis consistent with pulmonary fibrosis.
Q: Is there another finding that narrows the potential differential diagnosis? show answer
A: The distal oesophagus is dilated.
Q: What is the likely diagnosis? show answer
A: Scleroderma with interstitial pulmonary fibrosis.
Q: What percentage of patients with this underlying disease are symptomatic for pulmonary disease? show answer
A: 25%
Q: What other mechanism of pulmonary injury frequently co-exists in this condition? show answer
A: Aspiration pneumonitis due to oesophageal involvement.
Q: What is the typical distribution of lung changes? show answer
A: Lower zone / subpleural.
Case Discussion
The combination of basal pulmonary fibrosis and a dilated esophagus, is certainly suggestive of scleroderma. Although not pathognomonic it should be considered highly likely, especially in a woman.
In this case, the patient had clinically established scleroderma.
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