The treatment of PAP varies from case to case depending upon the form of PAP and the age of an affected individual and severity of the disease. About one-third of individuals with idiopathic PAP (of unknown cause) will improve without treatment (spontaneous remission).
Patients with moderate or severe respiratory problems due to autoimmune PAP can be treated by a whole lung lavage, a procedure in which one lung is cleansed with a salt solution while the other is pumped with pure oxygen. In some cases, the procedure may need to be performed once; in others it may need to be repeated many times over several years. Generally, one lung is lavaged in the first procedure, followed by lavage of the opposite lung one to two weeks later.
A treatment using recombinant granulocyte macrophage-colony stimulating factor (GM-CSF) given by nose, or with a subcutaneous injection is not approved for use in autoimmune PAP yet, but soon may be an effective option.
Lung transplantation has been used to treat adults with PAP as a last resort. According to the medical literature, in some cases, PAP has recurred in adults who have received lung transplantation.
Treatment options for patients with secondary and hereditary PAP are less clear and depend in part on the specific cause.In secondary PAP, removal and avoidance of the causative agent (e.g., silica exposure) or treatment of the underlying disorder may improve symptoms.For neonates with severe congenital PAP, lung transplantation is the only treatment that appears to improve outcome.
Last updated: 1/27/2017
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