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Higher Risk Of Death Seen For PH With Combined Lung Disease

People with a combination of two lung diseases — specifically pulmonary fibrosis and emphysema, known as CPFE — who also have pulmonary hypertension (PH) were found to be at an increased risk of death in comparison to CPFE patients without PH, according to a new meta-analysis.

Having CPFE also was linked to a higher risk for PH among patients than was having either lung disease alone.

While the researchers stressed that more studies were needed "to further explore the risk of PH and its impact on survival in patients with CPFE," the findings of their meta-analysis — an analysis that combines the results of multiple scientific studies — "revealed a significantly increased risk of PH in patients with CPFE compared with those with [idiopathic pulmonary fibrosis] or emphysema alone," they wrote.

"Also, the presence of PH was a significant predictor of mortality," the team wrote.

The study, "An increased risk of pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema: a meta-analysis," was published in BMC Pulmonary Medicine.

Investigating the risk of PH in patients with other lung diseases

Chronic lung diseases such as chronic obstructive pulmonary disease (COPD) or idiopathic pulmonary fibrosis (IPF) can lead to the development of PH, a disorder in which the pressure in the blood vessels of the lungs, known as the pulmonary arteries, is elevated.

Such patients belong to Group 3 of the World Health Organization's PH classifications — the most commonly used classification system for the disease. Group 3 includes individuals with PH due to lung disease or chronic oxygen shortage.

Studies have shown that patients with combined pulmonary fibrosis and emphysema, dubbed CPFE, are at a particularly high risk of developing PH.

As the name suggests, CPFE is a disease characterized by the co-existence of IPF, in which scarring and inflammation in the lungs make breathing difficult, and emphysema, where damage to the air sacs of the lungs — often due to smoking — causes breathlessness.

It is not well established whether IPF or emphysema alone confer a similar risk for PH as does the combined disease. Moreover, for CPFE patients who do have PH, it is not known whether their mortality risk is increased.

To learn more, the scientists conducted a meta-analysis of 13 previously published studies on the topic. Together, these studies involved 1,596 participants, including 560 with CPFE, 720 with IPF, and 316 with emphysema. The studies, published between 2009 and 2021, were mostly conducted in East Asia.

Patients with CPFE were more likely to be male and have a history of smoking compared with IPF patients. They also were found to have the lowest diffusing capacity of the lungs for carbon monoxide, or DLCO — a measure that assesses the lungs' ability to transfer inhaled gas into the bloodstream.

Findings from a range of different lung function tests were consistent with the idea that CPFE patients show a mix of defects associated with either IPF or emphysema, according to the researchers.

Further study needed into risk of death for CPFE patients with PH

Patients with CPFE had a higher incidence of PH than patients with IPF alone, and were found to be at a 2.66 times higher risk of PH. Likewise, CPFE patients were at a 3.19 times higher risk relative to patients with emphysema only.

The severity of PH, reflected by an estimated measure of pulmonary artery pressure, also was higher in CPFE patients than in either group with only one lung condition. The researchers noted that the measures and cut-offs used to diagnose PH did vary between studies, limiting some head-to-head comparisons.

Scientists believe there are several reasons that might explain the risk of PH in CPFE patients. These may include blood vessel remodeling, inflammation, and blood vessel restrictions due to low oxygen levels.

"The natural course of CPFE might encompass the development of PH," the researchers wrote. "Additional studies focusing on the molecular mechanisms of PH in patients with CPFE are urgently required in the future."

Patients with CFPE who also had PH were found to have worse survival than patients with CFPE who did not have PH, with the risk of death more than six times higher in this population.

"Despite this dismal prognosis, there is currently no curative medication for the treatment of PH related to CPFE," the scientists wrote. They noted, however, that the natural course of PH-CPFE, "remains to be fully explored."

The researchers noted several issues with their analysis, most notably that there were no official diagnostic criteria for CPFE.

"Due to the limitations in this study, multi-center or prospective studies with a clear definition of CPFE should be conducted in the future," the team wrote, noting a need to examine survival outcomes among patients.

Chronic Obstructive Pulmonary Disease COPD

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African Americans' Lung Differences May Mean COPD Goes Undiagnosed

Many African Americans with clear symptoms of chronic obstructive pulmonary disease (COPD) don't get a formal diagnosis because current diagnostic tools might not work as well for them, a study finds.

Lung structure differences among African Americans that result in scores higher than the cutoff used to diagnose COPD appear to be behind the misdiagnoses.

In African Americans, "COPD is often missed because they are frequently exposed to various kinds of deprivation that seems to result in smaller lungs with comparatively better airflow," Elizabeth Regan, MD, PhD, the study's first author and a researcher at National Jewish Health in Denver, said in a hospital press release. "Now that we've revealed the gap, we can start to bridge it," Regan said, adding "better diagnostic tools will lead to better care for all of our patients, regardless of background."

The study, "Use of the Spirometric "Fixed-Ratio" Underdiagnoses COPD in African-Americans in a Longitudinal Cohort Study," was published in the Journal of General Internal Medicine.

COPD is a chronic inflammatory lung disease marked by airway blockage that causes persistent cough and wheezing, and shortness of breath. To diagnose COPD, doctors usually check for a block in airflow by using a spirometry, a test that measures how much and how quickly air can flow in and out of the lungs.

Shortcomings of ratio criteria in African Americans' COPD diagnosis

It involves two measurements: the volume of air that can be breathed out in one second (FEV1) and the volume of air that can be forced out of the lungs after taking the deepest breath possible (FVC). If the ratio of FEV1 to FVC (FEV1/FVC) is less than 0.7, it suggests COPD.

"African Americans are less often diagnosed with COPD," the scientists wrote, but the reasons behind this remain largely unclear, leading researchers in the U.S. To evaluate whether the current fixed FEV1/FVC ratio criteria for COPD diagnosis was accurate for African Americans.

They drew on data from the COPDgene study (NCT00608764), a large ongoing U.S. Study that seeks to understand how genetics influence the risk of developing COPD among smokers, and analyzed data from 3,366 African Americans and 6,766 non-Hispanic white people from 21 centers across the U.S. All were smokers or former smokers. Those with a preexisting lung disease other than COPD, except for asthma, were excluded.

The researchers reviewed demographic factors, socioeconomic characteristics, respiratory symptoms, lung imaging data, and mortality.

Using the current spirometry criteria, more African Americans were classified as not having COPD than non-Hispanic white people (70% vs. 49%). This means they had a FEV1/FVC ratio of 0.7 or higher. African Americans were younger (55 vs. 62) and more likely to be current smokers (80% vs. 39%), but their risk of dying in 12 years was similar to that of non-Hispanic white people.

They also had a bigger reduction of FVC relative to that of FEV1, increasing the FEV1/FVC ratio and excluding a COPD diagnosis.

"When the two aspects of spirometry are combined in a mathematic ratio (airflow/lung volume) the number is higher than would otherwise be expected, leading to the conclusion that they do not have COPD or other lung problems," Regan said.

COPD symptoms in African Americans

Higher FEV1/FVC ratios among African Americans were seen despite the fact that many had symptoms suggestive of COPD and some even had signs of emphysema on computed tomography scans. Emphysema is a severe form of COPD marked by the destruction of the tiny air sacs in the lungs where gas exchanges occur.

To ensure a fair comparison, the researchers matched by age, sex, and smoking status the African Americans and the non-Hispanic white people who didn't get a formal COPD diagnosis.

Again, African Americans had worse spirometry results, had more or more severe symptoms, and worse diffusing capacity for carbon monoxide (DLCO), which measures how well the lungs can exchange gases.

They scored significantly higher on the BODE index, a measure of body mass, airflow obstruction, shortness of breath, and exercise capacity. Higher scores indicate a lower chance of survival.

African Americans were also more likely to live in areas of poorer socioeconomic conditions than non-Hispanic white people, which may lead to differences in lung development and higher FEV1/FVC ratios.

The findings suggest the current way of diagnosing COPD might not be accurate for African Americans and adds to data from previous COPDGene's analyses that suggest diagnostic criteria should include more than just spirometry data.

"Disproportionate reductions in FVC relative to FEV1 leading to higher FEV1/FVC were identified in these participants and associated with deprivation [lower socioeconomic conditions]," the researchers wrote. "Broader diagnostic criteria for COPD are needed to identify the disease across all populations."

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