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Answer: 4. Most patients with pulmonary alveolar proteinosis are asymptomatic and the disorder is discovered incidentally

Pulmonary alveolar proteinosis is associated with a number of conditions, including hematological malignancies, such as leukemia and lymphoma. A number of occupational exposures are associated with the development of pulmonary alveolar proteinosis, or histopathologically similar syndromes, such as mineral dusts and fumes, including aluminum dust, titanium dioxide, cement dust, fibrous insulation material, and nitrogen dioxide, and inorganic dusts, such as silicon dioxide. The appearance of crazy paving on thoracic CT, particularly high-resolution CT, is typical of pulmonary alveolar proteinosis, but a number of other conditions have been noted to produce this CT pattern as well. A significant number of patients with pulmonary alveolar proteinosis are asymptomatic at presentation- up to one-third- but the majority of patients present with dyspnea on exertion, progressive shortness of breath, cough, or less commonly, chest pain.

References

  1. Johkoh T, Itoh H, Muller NL, Ichikado K, Nakamura H, Ikezoe J, et al. Crazy-paving appearance at thin-section CT: spectrum of disease and pathologic findings. Radiology 1999; 211:155-160.
  2. Wang BM, Stern EJ, Schmidt RA Pierson DJ. Diagnosing pulmonary alveolar proteinosis. A review and an update. Chest 1997;111:460-466.
  3. Goldstein LS, Kavuru MS, Curtis-McCarthy P, Christie HA, Farver C Stoller JK. Pulmonary alveolar proteinosis: Clinical features and outcomes. Chest 1998;114:1357-1362.
  4. Kitamura T, Tanaka N, Watanabe J, Uchida K, Kanegasaki S,Yamada Y, Nakata K. Idiopathic pulmonary alveolar proteinosis asan autoimmune disease with neutralizing antibody against granulocyte/macrophage colony stimulating factor. J ExpMed 1999;190:875–880.
  5. Khan A, Agarwal R, Aggarwal AN. Effectiveness of granulocyte-macrophage colony-stimulating factor therapy in autoimmune pulmonary alveolar proteinosisTherapy for Pulmonary Alveolar Proteinosis: A meta-analysis of observational studies. Chest May 2012;141:1273-1283.

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