CTICasesDesquamative Interstitial Pneumonia in Scleroderma
Imaging findings
Chest radiographs demonstrate low lung volumes and subtle reticular opacities at the lung bases. High-resolution CT shows extensive, bilateral, basal-predominant ground glass opacities containing fine cystic or bubbly lucencies, mild reticulation, and patchy spared areas. Concurrently, mild esophageal dilatation is noted. Surgical lung biopsy of this lifelong non-smoker demonstrates a diffuse intra-alveolar accumulation of macrophages, establishing a confident histopathologic diagnosis of desquamative interstitial pneumonia (DIP).
Key takeaways
Although desquamative interstitial pneumonia is classically associated with cigarette smoking, it can occasionally arise in non-smokers in the context of connective tissue diseases, particularly systemic sclerosis (scleroderma). Pathologically, DIP is characterized by a diffuse accumulation of intra-alveolar macrophages, which can stain variably for hemosiderin and must be distinguished from non-specific interstitial pneumonia (NSIP). Recognizing that DIP-like reactions can occur as a manifestation of autoimmune lung injury helps guide appropriate clinical management, which may include immunosuppressive therapy rather than simple smoking cessation.
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