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Forty-year-old woman presents to ER with dyspnea.

Contributed from the case records of the 
Indiana University Department of Radiology

History:  Forty-year-old woman presents to ER with dyspnea.
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Which choice is most appropriate?

Pulmonary embolism.Intracardiac mass.Pericardial thickening.Anomalous pulmonary venous return.No significant abnormality.
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Forty-year-old woman presents to ER with dyspnea.

Here are some lung-window images from the same scan. Click to enlarge.

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The patient is NOT a smoker

What is in your differential diagnosis? What is your favored diagnosis? (Discussed further on next page).

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Forty-year-old woman presents to ER with dyspnea.

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Findings:  Diffuse cystic lung disease seen symmetrically and bilaterally

Differential diagnosis for cystic lung disease:

  • Langerhans cell granulomatosis (EG)
  • Lymphangioleiomyomatosis (LAM)
  • Emphysema (centrilobular)
  • Idiopathic pulmonary fibrosis
  • Pneumocystis
Diagnosis:  Actual diagnosis unknown (patient lost to followup). LAM was favored over EG.
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Forty-year-old woman presents to ER with dyspnea.


Key points:
 

  • LAM is hamartomatous proliferation of smooth muscle around lymphatics and blood vessels that progress to replace the normal lung with cysts. Usually found in women of childbearing age.
  • Lymphocytic interstitial pneumonia is from chronic antigenic stimulus eliciting lymphoproliferative response. My be idiopathic or secondary to autoimmune etiology, viral infection (especially HIV), and Castleman disease.
  • Langerhans cell granulomatosis is likely secondary to allergic reaction to smoke. It may regress, resolve completely, become stable, or progress to advanced cystic disease.
  • Desquamative interstitial pneumonia is associated with smoking 90% of the time. Predominant in the lower lungs. Small well-defined cysts. Best clue is smoker with ground glass opacities.

References:

  1. https://my.statdx.com/get.summary?dxId=desquamative_interstitial_pneumonia.
  2. https://my.statdx.com/get.summary?dxId=lymphangiomyomatosis_dx.
  3. https://my.statdx.com/get.summary?dxId=lymphocytic_interstitial_pneumonitis_dx.
  4. https://my.statdx.com/get.summary?dxId=langerhans_cell_granulomatosis__pulmonary.
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Forty-year-old woman presents to ER with dyspnea.


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