This is a 55 year old female who has sustained 2 pneumothoracies and had bilateral pleurodesis. Otherwise she is mildly short of breath. PFT’s show an FEV1 of 69% predicted an FEV1/FVC ratio of .50. Her DlCO is 79% of predicted. She has not smoked and the alpha-1 level is normal
Please offer a differential diagnosis
April 21, 2008 at 1:51 am |
CT shows thin walled cysts uniformly distributed in upper and lower lobes. Differentials to be considered include lymphangioleiomyomatosis, PLCH and A1AT deficiency. PLCH is unlikely given nonsmoker status, absence of nodules and absence of upper lobe predominance of cysts. A1AT levels are normal. I favor LAM, although patient is slightly older than the usual age group expected in this disease.