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Presented by Peter Illei, M.D. and prepared by Safia Salaria, M.B.B.S.
Case 3: 49-year-old female from the Bahamas with no significant past medical history.
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1. Question
Week 498: Case 3
49-year-old female from the Bahamas with no significant past medical history,who presented for evaluation of chronic cough and abnormal CT scan and open lung biopsy. The patient states that she was doing quite well and was free of any respiratory symptoms until 2 years ago. At that time she developed a cough that lasted for several months. She received empiric therapy for many different ailments including sinus symptoms, reflux, and inhaler therapy. She is unable to recall the precise medications used, but states that these did not have any improvement in her symptoms. Subsequently she underwent a CT scan, which demonstrated few small subpleural nodules, nodular densities in each lung, and minimal fibrosis in the bilateral apices. This was followed expectantly and demonstrated no changes on serial CT scans. Because of persistent symptoms, she underwent an open lung biopsy.images/1Alex/100311case3image1.jpg
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images/1Alex/100311case3image5.jpgCorrect
Answer: Thrombotic pulmonary hypertension
Histology:
Discussion: Sections show lung with intraalveolar hemorrhage, focal subpleural scarring and multiple foci of eccentric intimal fibrosis of pulmonary arteries. Background lung shows emphysematous change and foci of alveolar hemorrhage. No granulomas, malignancy or vasculitis are identified. The vascular changes are peculiar and show eccentric intimal fibrosis and most consistent with thrombotic pulmonary artery hypertension. There is no evidence of tissue response to the alveolar hemorrhage and no significant/increased hemosiderin deposition is noted and therefore this may represent an intraoperative event. There is only focal interstitial scarring with mild emphysematous change in the background.
Incorrect
Answer: Thrombotic pulmonary hypertension
Histology:
Discussion: Sections show lung with intraalveolar hemorrhage, focal subpleural scarring and multiple foci of eccentric intimal fibrosis of pulmonary arteries. Background lung shows emphysematous change and foci of alveolar hemorrhage. No granulomas, malignancy or vasculitis are identified. The vascular changes are peculiar and show eccentric intimal fibrosis and most consistent with thrombotic pulmonary artery hypertension. There is no evidence of tissue response to the alveolar hemorrhage and no significant/increased hemosiderin deposition is noted and therefore this may represent an intraoperative event. There is only focal interstitial scarring with mild emphysematous change in the background.
