Correct!
4. 1 and 3
There are 3 types of alveolar proteinosis (1):
1. Congenital
2. Secondary (allogeneic bone marrow transplant, hematologic malignancy, infections (Pneumocystis), acute toxic inhalations [silica, aluminum])
3. Acquired (autoimmune).
About 90% of the cases of alveolar proteinosis are acquired. This occurs when due anti-GM-CSF antibodies are formed which result in impairment of the alveolar macrophage clearance of surfactant. The surfactant accumulates clogging the alveoli resulting in the dyspnea and the positive PAS staining. Our patient's serum was positive for antibodies to GM-CSF.
The treatment of choice for severely ill patients is whole lung lavage (1). She was also begun on GM-CSF.
After whole lung lavage she was weaned off ventilator. After discharge pulmonary function tests (PFTs) showed a FVC of 80% of predicted, a TLC of 79% of predicted, and a DLco of 74% of predicted. A thoracic CT scan marked improvement in her "crazy paving" (Figure 4).
Figure 4. Representative images from the repeat thoracic CT scan showing improvement in the "crazy paving" compared to Figure 1.
By November, 2009 she had completed 4 months of GM-CSF and was feeling well, not requiring oxygen. She was subsequently lost to follow-up. In February, 2014 she was referred by her obstetrician because she was now pregnant with increasing dyspnea. Repeat PFTs showed her FVC was 56% of predicted and her DLco was 42% of predicted. Chest x-ray showed diffuse consolidation.
Which of the following are indicated at this time? (Click on the correct answer to proceed to the fifth and last panel)