Close Monitoring Needed for SSc Patients With Rare Type of Interstitial Lung Disease, Study Shows

Evidence of pleuroparenchymal fibroelastosis (PPFE) — a rare type of interstitial lung disease — in people with systemic sclerosis (SSc) carries a negative prognosis, a study shows.

Therefore, SSc patients with PPFE should be closely monitored and receive regular follow-up by their healthcare team.

The study, titled “Pleuroparenchymal fibroelastosis in patients affected by systemic sclerosis,” was published in the journal Medicine.

Interstitial lung disease (ILD) is a term used to describe a large group of disorders that cause scarring (fibrosis) of the lungs. Since SSc is characterized by fibrosis of internal organs, ILD is a common finding among SSc patients.

PPFE is a rare and recently discovered ILD. It is characterized by fibrosis and thickening of the visceral pleura — the delicate membrane that covers the surface of each lung — and the nearby parenchymal areas of the upper portion of lungs.

A study showed that there was a high prevalence of PPFE-like lesions — as detected by an imaging technique called chest high-resolution computed tomography (HRCT) — in a group of patients with ILD associated with connective tissue disorders, such as SSc.

Because PPFE-like lesions are associated with a negative prognosis, it is important to identify and learn more about them in people with SSc, the team of researchers said.

The team therefore explored the clinical and radiological characteristics of PPFE-like lesions. They analyzed a group of 113 patients with ILD associated with connective tissue disorders, namely SSc, rheumatoid arthritis, dermatomyositis/polymyositis, and Sjögren’s syndrome.

The median age of the group was 62 years. Patients were observed for almost 24 months, and their clinical and functional details were analyzed retrospectively.

PPFE-like lesions were found in 21 people (19%), and were relatively frequent in those with SSc (6 out of 14 patients; 43%), and primary Sjögren’s syndrome (4 out of 14 patients; 29%).

Researchers found that those with PPFE-like lesions were significantly older, and had lower body mass index than those without lesions.

Furthermore, PPFE-like lesions were associated with an increased risk of pneumothorax, or a collapsed lung, and pneumomediastinum, or the abnormal presence of air or other gas in the region located between the lungs — 38% versus 15% in those without lesions.

People with PPFE-like lesions also experienced a more rapid lung function decline, and had a poor prognosis.

In fact, at the 2-year follow-up, patients were found to have reduced functionality of the lungs and decreased diffusion capacity for carbon monoxide (DLCO), which measures the ability of the lungs to transfer gas from inhaled air to the blood.

During the study period, progression of PPFE-like lesions was observed in 13 out of 21 patients. Among them, six patients died (29%), and their PPFE-like lesions were commonly progressive.

Next, researchers analyzed bronchoalveolar lavage fluid, and quantified the presence of immune cells in the lungs. Bronchoalveolar lavage findings showed that there were elevated numbers of neutrophils and eosinophils — two types of immune cells — due to the presence of inflammation, which is harmful to lung cells.

Based on the results, the team concluded that the “study indicates the clinical importance of radiological evidence of PPFE in a cohort of patients with ILD-SSc.”

“Radiological PPFE-like lesions were associated with increased risk of pneumothorax and pneumomediastinum, rapid lung function decline, and poor prognosis. Most radiological alterations involved the upper lobes and were readily detected,” the researchers said.

These findings highlight the need for people with ILD-SSc with evidence of PPFE to have close follow-up, they said.

Clinicians “should be aware of this new radiological finding which is accompanied by a negative prognosis, especially when associated with a progressive course. Patients with this radiological pattern need to be monitored with particular attention,” the team concluded.