Case 412 -- A caucasian woman in her late 20s with progressive shortness of breath

Contributed by Craig Horbinski, MD, PhD, and Sanja Dacic, MD, PhD
Published on line in January, 2005



The patient was a Caucasian woman smoker (10 pack-years) in her late 20s who complained of chronic cough and shortness of breath. The CXR showed mediastinal lymphadenopathy. Bronchial washings were essentially nondiagnostic, showing only reactive bronchial cells and inflammatory infiltrate. FNA showed epithelioid cell clusters and giant cells, consistent with a granulomatous inflammatory process. PFTs were as follows:

The patient quit smoking, but symptoms progressively worsened despite balloon dilatation of her airways and prednisone.


PFT results:

The patient underwent bilateral lung transplantation in August 2004.


The pleura of both lungs was covered with multiple, firm, patchy-white plaques, ranging in diameter from 0.1 to 2.5 cm [Image 1]. The bronchi and blood vessels were grossly patent. Cut surface revealed innumerable intraparenchymal lesions similar to those on the pleura [Image 2]. The hilar bronchi were grossly patent, but constricted. In contrast to the left lung, the right lung was full of mucopurulent material obstructing the distal airways in all lung fields [Image 3].


The histologic sections demonstrated a markedly remodeled lung parenchyma. Pleural, subpleural, and paraseptal fibrosis were associated with well-formed, epithelioid, hyalinized, coalescing granulomas distributed along lymphatic routes in the pleura and septa and along bronchovascular structures [Images 4, 5, 6, 7, 8, 9 and 10]. Focal fibrinoid necrosis in the center of a few granulomas was present [Image 11], but special stains for acid-fast bacilli and fungal organisms were negative. Invasion of vessel walls was frequent, but there was no necrosis [Images 8 and 9]. There was an inconspicuous interstitial mononuclear cell infiltrate. Non-necrotizing granulomas were also present in the hilar lymph nodes. An asteroid body was seen in one of the giant cells. [Image 12, arrow]


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