The following is a summary of “Emphysema and interstitial lung disease in systemic sclerosis-related pulmonary hypertension,” published in the June 2023 issue of Pulmonology by Vakhshoorzadeh, et al.

Pulmonary hypertension (PH) is a significant cause of morbidity and mortality in individuals with systemic sclerosis (SSc). Patients with SSc-PH and coexisting interstitial lung disease (ILD) generally experience worse clinical outcomes compared to those with SSc-PH alone. 

Moreover, as the severity of ILD, as measured by forced vital capacity (FVC), increases, there is a tendency towards higher all-cause mortality. Reduced FVC is a common feature of both restrictive lung diseases, including ILD, and obstructive lung diseases, such as chronic obstructive pulmonary disease (COPD), which can also affect SSc patients. For a study, researchers sought to characterize the influence of FVC on clinical outcomes in SSc-PH, considering radiologic ILD/emphysema.

However, little was known about the impact of concurrent obstructive lung disease in SSc-PH, which may confound FVC measurements used as a marker for ILD severity and its contribution to overall mortality. The hypothesis posits that a decline in FVC associated with any hypoxic lung disease, irrespective of its etiology, is associated with an increased risk of transplant-free, all-cause mortality in individuals with SSc-PH.

Source: resmedjournal.com/article/S0954-6111(23)00221-4/fulltext