Combined pulmonary fibrosis and emphysema (CPFE) impairs lung function and decreases survival rates of systemic sclerosis (SSc) patients, a retrospective analysis shows.
The study “Overall mortality in combined pulmonary brosis and emphysema related to systemic sclerosis” was published in the journal Rheumatic and Musculoskeletal Diseases.
SSc is caused by an excessive production of the protein collagen, leading to tissue thickening and scarring. In the lungs, accumulation of scar tissue can lead to interstitial lung disease (ILD), a general category that includes many different lung conditions, including pulmonary fibrosis.
Emphysema is a lung disease in which the lung’s air sacs, called alveoli, are destroyed, causing severe breathing problems, such as shortness of breath and chronic cough.
CPFE is associated with shorter survival among idiopathic pulmonary fibrosis (IPF) patients. However, it remains unknown whether this also is the case in SSc patients.
To address this question, a team of researchers in Italy performed a retrospective analysis of data from 470 patients with SSc (mean 59.2 years), followed for a median period of 4.2 years. Seventy-three patients died during follow-up.
Researchers reviewed patient’s pulmonary function tests — forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLco) — along with computerized tomography (CT) chest scans.
Based on chest CT images, researchers divided the patients into four subgroups: SSc with CPFE; SSc with ILD; SSc with emphysema only, and; other-SSc subtypes.
CPFE had the highest prevalence in male SSc patients, and was found in 18% of patients with ILD, and 7% of patients who never smoked.
Compared to the three other groups, those with SSc and CPFE had increased lung impairment, as shown by extended fibrosis and decreased pulmonary function.
Moreover, a statistical analysis showed that SSc patients with CPFE had the worst survival rate of the four groups.
Overall, the results showed that “CPFE increases the mortality risk in SSc along with a highly impaired lung function,” researchers wrote.
The team argued that emphysema and pulmonary fibrosis analysis should be included in radiological exams, like CT chest scans, in patients with SSc.
“CPFE increases the risk of mortality and it should always be sought in order to better outline the prognosis in patients with SSc” the team concluded.