Idiopathic pulmonary fibrosis and lymphocytic interstitial pneumonitis

Case contributed by Melbourne Uni Radiology Masters


Shortness of breath.

Patient Data

Age: 84-year-old
Gender: Male

There is extensive interstitial opacity predominately involving the subpleural lung involving all lung zones without a basal predominance. Minor areas of traction airway dilatation are seen peripherally. No definite honeycomb change. There is widespread mosaic attenuation with no evidence of air trapping consistent with widespread hazy groundglass opacity. Extensive well-defined lung cysts are identified bilaterally measuring up to 27mm in diameter. The central airways are normal.

The pulmonary artery is dilated measuring 3.4cm. The heart size is enlarged. Minor calcification of the aortic bowel are seen. Extensive coronary artery calcification is identified.

The pleural spaces are clear.

Numerous mediastinal lymph nodes are seen, the largest in the right upper paratracheal region measuring 14mm and a subcarinal node measuring 24mm.

The right lobe of the thyroid gland appears enlarged but without retrosternal extension.

A small well-defined low-density lesion superiorly in segment VIII of the liver is likely to be a cyst.

No suspicious bony abnormalities seen.

Conclusion: The combination of interstitial abnormality, ground-glass opacity and lung cysts favors a diagnosis of lymphocytic interstitial pneumonia.

Case Discussion

Biopsy proved LIP in this patient. 

The combination of findings of fibrosis and multiple lung cysts is rarely encountered except in patients with LIP.

Cystic lung syndromes such as PLCH and LAM are not associated with fibrosis.

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