RT Book, Section
A1 Fitzpatrick, Meghan E.
A1 Prendergast, Niall T.
A1 Rivera-Lebron, Belinda
A2 Papadakis, Maxine A.
A2 McPhee, Stephen J.
A2 Rabow, Michael W.
A2 McQuaid, Kenneth R.
SR Print(0)
ID 1186012944
T1 Pulmonary Alveolar Proteinosis
T2 Current Medical Diagnosis & Treatment 2022
YR 2022
FD 2022
PB McGraw-Hill Education
PP New York, NY
SN 9781264269389
LK accessmedicine.mhmedical.com/content.aspx?aid=1186012944
RD 2024/04/19
AB Pulmonary  alveolar  proteinosis  is  a  rare  disease  in  which  periodic  acid-Schiff  (PAS)-positive  phospholipids  accumulate  within  alveolar  spaces.  The  condition  may  be  primary  (idiopathic)  or  secondary  (occurring  in  immunodeficiency;  hematologic  malignancies;  inhalation  of  mineral  dusts;  or  following  lung  infections,  including  tuberculosis  and  viral  infections).  Progressive  dyspnea  is  the  usual  presenting  symptom,  and  chest  radiograph  shows  bilateral  alveolar  infiltrates  suggestive  of  pulmonary  edema  (eFigure  9–16).  The  diagnosis  is  based  on  demonstration  of  characteristic  findings  on  BAL  (milky  appearance  and  PAS-positive  lipoproteinaceous  material)  in  association  with  typical  clinical  and  radiographic  features.  In  secondary  disease,  an  elevated  anti-GM-CSF  (anti-granulocyte-macrophage  colony-stimulating  factor)  titer  in  serum  or  BAL  fluid  is  highly  sensitive  and  specific.  In  some  cases,  transbronchial  or  surgical  lung  biopsy  (revealing  amorphous  intra-alveolar  phospholipid)  is  necessary.