Systemic sclerosis patients with interstitial lung disease (SSc-ILD) have a great deal of symptoms and comorbidities (other diseases), with a high impact on their overall biological and physical stability, or frailty.
The study, “Severity and features of frailty in systemic sclerosis-associated interstitial lung disease,” was published in the journal Respiratory Medicine.
It shows that the biological age of these patients often surpasses patients’ actual age, highlighting the high cumulative impact of these symptoms, which is known as frailty.
It suggests that recognizing frailty in these patients can help identify those who may benefit from supportive management to improve their independence.
ILD is a common complication of scleroderma and a main cause of mortality. More than half of patients with diffuse cutaneous SSc, and more than one third of patients with limited cutaneous SSc, also have ILD.
But this connective tissue disease can affect many other organs and tissues, including the skin, kidneys, and the gastrointestinal system.
Frailty — defined as the accumulation of multiple deficits (physiological, psychological, and social) that lead to increased vulnerability to biological stress and adverse health outcomes — can be used to describe the cumulative impact of the symptoms of SSc-ILD and its comorbidities.
“The individual items of the [Frailty Index] measure how many deficits a patient has and thus how well a patient can tolerate additional biological stress,” the researchers wrote.
Patients with SSc-ILD can be frail due to physical disabilities secondary to ILD, non-respiratory symptoms of SSc, or even unrelated ailments and diseases. Previous studies have shown that being frail can be a predictive factor of mortality in SSc and other chronic diseases. However, it has not been characterized previously in patients with SSc-ILD.
To help shed light on this issue, researchers from the University of British Columbia, Canada, evaluated 86 patients with SSc-ILD and 167 patients with fibrotic ILD unrelated to connective tissue disease, and compared the prevalence, severity, and components of frailty in each group.
Using the 42-item Frailty Index, which is based on the presence or absence of specific deficits, the researchers found that 55% of patients in the SSc-ILD group met the criteria.
“The most frequently reported deficits in SSc-ILD were restriction of activity due to poor health (71%); less activity at home or school (57%); always feeling tired (57%); need for help for heavy household chores (57%); and arthritis or rheumatism (50%),” the researchers reported.
Further analysis showed the factor that contributed most in this patient population was dyspnea, or shortness of breath. This comes in the wake of previous reports that found a correlation between dyspnea and frailty in ILD unrelated to connective tissue disease and chronic obstructive pulmonary disease (COPD).
The researchers also found that 21% of the SSc-ILD patients could be identified as pre-frailty. This finding suggests that nearly three quarters of SSc-ILD patients could be at risk of other ailments and could benefit from supportive management to preserve their independence.
“Frailty in SSc-ILD is predominantly related to independence and self-care deficits, while frailty in non-CTD ILD and other elderly populations is characterized by more frequent comorbidities,” researchers wrote.
Overall, this study showed that frailty is highly prevalent in SSc-ILD patients and may help identify those less likely to tolerate potentially toxic pharmaceutical therapies or lung transplant. However, additional studies are still required to confirm these findings and identify potential contributing factors in SSc-ILD patients.