Rare Lung Disease

Lymphangioleiomyomatosis

                                                                

      

 

 

 

 

 

Lymphangioleiomyomatosis (LAM) is a rare lung disease primarily affecting women. It is characterized by abnormal smooth-muscle cell infiltration and proliferation of the airway, which leads to a loss of lung function by progressive cystic formation and air trapping.  Hyperpolarized 129Xe MRI is utilized in research as a tool to investigate real time ventilation through the use of hyperpolarized Xe gas as a contrast agent.  With CT we can identify cystic lung and quantify individual cyst size and total cystic lung volume.  With HP 129Xe MRI can visualize regional lung ventilation and classify regional lung function.  So from these two imaging modalities we can determine cystic lung ventilation and compare it to lung function measures.                                                            
 

 

 

 

 

  

Pulmonary Alveolar Proteinosis

                                                                                                        Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by dense alveolar lipoproteinaceous surfactant accumulation and hypoxemic respiratory failure. Currently there is no standard measure for treatment efficacy and no non-invasive way to quantify surfactant burden or disease severity in PAP; thus a common methodology for practical clinical assessment is needed.  High resolution CT is an important tool in the diagnosis of PAP and the qualitative assessment of disease severity.  Although continuous advances in CT scanners permit higher resolution with greater volume coverage, there is still significant exposure to radiation and this is an especially undesirable risk for children and evaluation of patients in longitudinal studies. The advantage of magnetic resonance imaging (MRI) is that it does not involve ionizing radiation, and therefore reduces the risk of future radiation-induced cancers.  Through UTE MR image analysis, surfactant burden/severity and surfactant removal after treatment can be quantified in a pediatric population.  The calculated amount of surfactant removed, as quantified via both imaging modalities, correlates with the actual amount of sediment recovered from the WLL effluent.  UTE MRI demonstrates its potential as a complementary imaging tool for CT in the lifelong monitoring of disease status and treatment efficacy in this patient population.