Thread by @gretchemaben

Here’s my tweet-ucation from the “Updates on Diffuse and Orphan Lung Diseases” session!
#CHEST2020

The next session is "Incidence, Mortality, and Characteristics of Patients Admitted with Pulmonary Alveolar Proteinosis in the US" with Rodrigo Garcia-Tome #CHEST2020

PAP is a rare disease. PAP has a variable clinical course, sometimes with spontaneous resolution but other times with progressive respiratory insufficiency. #CHEST2020

The objective of this study was to descrbe the hospital admission incidence, inpatient mortality, demographic characteristic, LOS, and hospital costs of PAP in the US. #CHEST2020

They used the HCUP and the national inpatient sample for this study and did a retrospective cohort study. #CHEST2020

Patient characteristics. Out of 500 patients 45% were women and 58% were white. #CHEST2020

There was a 5% inpatient mortality. #CHEST2020

Mean length of stay was 6.24 days. #CHEST2020

Whole lung lavages were performed 195 times. #CHEST2020

The hospitalization cost per admission was almost $30K. #CHEST2020

Summary #CHEST2020

The next session is "Natural History of ILD and Response to TreatmentRegimens in Patients with Idiopathic Inflammatory Myopathies" with Dr. Hema Balina! #CHEST2020

The aim of this study was to evaluate the pattern of IIM-ILD diagnosis and assess responsiveness to the regimen. #CHEST2020

Study methods #CHEST2020

Results: A total of 32 patients were included. Females were more common. 61% of patients with AA/black. #CHEST2020

First line treatment was Azathioprine in 55% of the patients, with Mycophenolate Modefil second. Prednisone was used in 84%. #CHEST2020

Treatment was changed in 67% of patients, mainly due to lack of response and side effect intolerance. 37% of patients had worsening of disease and 3/32 had died by the end of the follow-up period. #CHEST2020

Conclusions #CHEST2020

The next session is Dr. Dan Culver with "Baseline Characteristics of Patients in the Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry” #CHEST2020

The IPF-PRO registry enrolled 1002 patients with IPF. #CHEST2020

The IPF-PRO is designed to enrol patients with newly-diagnosed IPF within 6 months to collect retrospective data and a set of data at enrollment and every 6 months after. #CHEST2020

These are the demographics of the patients in the registry. #CHEST2020

65% had definite IPF vs probable or possible IPF. #CHEST2020

Lung function averages are seen here. Because the registry has very mild and very severe patients it is a broader sample than some. #CHEST2020

These are the patient-reported outcomes of the patients in the registry. These patients are suffering a good bit. #CHEST2020

These patients not only have comorbidities, but they can impact their outcomes. The most common comorbidity was GERD. #CHEST2020

Most of these patients were enrolled after the US approval of antifibrotic therapies. #CHEST2020

Other medications patients were on included statins, PPIs, supplemental oxygen, and more. Note that oral steroids weren't being used much, which is good given what we know about steroids in IPF. #CHEST2020

Conclusions #CHEST2020

The next session is "Clinical Significance of Radiological Markers of Pulmonary HTN in Combined Pulmonary Fibrosis and Emphysema" with Dr. Fatima Zeba! #CHEST2020

This study aimed to assess whether there is a relationship between radiological markers of PH, presence of PH, and mortality in CPFE. #CHEST2020

Study methods #CHEST2020

82 patients met inclusion criteria. #CHEST2020

A PA/AA ratio >1 was a/w decreased survival. #CHEST2020

Presence of honeycombing, traction bronchiectasis, and ground glass changes were associated with increased mortality. #CHEST2020

The RIPV diameter and LVSA diameter were associated with development of pulmonary HTN in the CPFE group.
#CHEST2020

Conclusions #CHEST2020

Clinical implications #CHEST2020

The next session is "Uncarboxylated Matrix G1a protein (ucMGP) as a predictor of lung function" with Jenna McNeill! #CHEST2020

Matrix G1a protein is expressed in elastin rich tissue and is a potent inhibitor of calcification and subsequently of elastin degradation. #CHEST2020

Previous studies showed that ucMGP is higher in COPD patients. #CHEST2020

Study design #CHEST2020

You can see here that over 6000 patients were assessed. #CHEST2020

ucMGP was associated with baseline lung function with a lower FEV1 and FVC with higher levels. Higher levels of ucMGP were associated with a higher odds of restrictive lung disease. #CHEST2020

Typically elastin degradation is associated with obstructive lung disease, but it has also been associated with IPF. #CHEST2020

Conclusions #CHEST2020

Thanks to all of our fantastic speakers for this session! #CHEST2020