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Alveolar Proteinosis (diff names)
last updated: April 11, 2016
General
a syndrome caused by defects in the generation or degeneration of surfactant that lead to abnormal accumulation of surfactant-derived components (proteinaceous material) in the alveoli and terminal bronchioli
Architecture of lung untouched, normal intraalveolar septum
Golgi: involved in secretory pathway
Rare, mostly men, 72% smokers, make predominance lost with nonsmokers; smoking accelerates the disease progression
3 classifications
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Congenital
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deficiency beta surfactant protein
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Primary (acquired) (autoimmune)
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mostly (90% of cases)
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more patchy, crazy paving
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Secondary
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associated with certain systemic diseases
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“With the benefit of hindsight”
1990s: mice deficiency in GM-CSF autoantibodies
Symptoms
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Dyspnea
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resting dyspnea?
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Weight loss
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Fever
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Clubbing ?
Physiology
Surfactant recycled by macrophages and type II cells
Pathophysiology
GMCSF from type II cells allows maturation of the macrophages
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antibodies to the GMCSF does not allow it to bind to its macrophage receptor and younger foamy macrophages
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defect in the breakdown of surfactant
Diagnosis
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Latix agglutinin test for GMCSF (BAL levels correlate better)
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Milky, waxy, foamy macrophages (also think usually amiodarone)
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PAS+ pink stain inside the cell
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PFT: restrictive pattern, low DLCO
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X-ray: widespread patchy airspace disease more at bases
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CT: crazy paving pattern (not specific)
Treatment
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Whole lung lavage
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double lumen tube
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1L saline instilled with percussion (about 30L per lung)
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many bottles
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Looks like a paper weight in snow storm when shaken
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usually lavaged once is enough (on average lasts about 15 months)
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usually symptoms get better few days after 2nd lavage
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usually not diuresed afterwards
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Duke does lavage under hyperbaric chamber
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GMCSF
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given for autoimmune disease usually
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usually if becomes refractory to whole lung lavage
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Plasmapheresis, retuximab, etc not really done yet; steroids have been shown to be detrimental
Reference


