Three factors — the presence of digital ulcers, Raynaud’s phenomenon, and gastrointestinal (GI) symptoms — appear to predict a worsening over time in life quality for people with systemic scleroderma (SSc), a study in nearly 500 patients shows.
In addition, pulmonary arterial hypertension (PAH) was found to significantly contribute to declining quality of life among those with long-standing disease.
These findings may help to identify unmet needs among patients and appropriate interventions to address them, the researchers said.
Their study, “Health-related quality of life in patients with systemic sclerosis: evolution over time and main determinants,” was published in the journal Rheumatology.
Given its severe and bodywide (systemic) nature, systemic scleroderma has a clear negative impact on patients’ health-related quality of life (HRQoL). HRQoL, focused on the influence that health status has on life quality, is a patient-reported outcome that includes domains related to physical, mental, emotional, and social functioning.
Notably, pain, shortness of breath, digital ulcers, Raynaud’s phenomenon and GI involvement have been shown to negatively affect HRQoL in people with systemic scleroderma. Raynaud’s phenomenon is a condition in which the fingers and toes become numb, prickly, and frigid in response to cold temperatures or stress.
But data are limited as to how HRQoL changes over time in this patient population, and which disease-specific features mostly cause these changes.
To address this knowledge gap, a team of researchers at Leiden University Medical Center, in the Netherlands, assessed quality of life changes over time in all 492 SSc patients followed at their center between 2009 and 2019.
They analyzed patients’ demographic and clinical data, as well as HRQoL, which was measured annually using two validated measures — the Short Form-36 and the EuroQol Five-Dimensional descriptive system. Both measures include both physical and mental aspects of health-related quality of life.
Participants’ mean age was 55, most (79%) were female, and 24% had diffuse cutaneous systemic scleroderma (dcSSc). They had a median disease duration (since first non-Raynaud symptom) of three years, and 202 patients were classified as incident cases, meaning they had their first non-Raynaud symptom within the two years prior to study enrollment.
More than one-third (37%) had interstitial lung disease (ILD), 16% showed severe GI involvement, and 5% had PAH. Patients were followed for a median of 3.4 years.
At first evaluation (baseline), participants with dcSSc, a lower education level, shorter disease duration, and co-existing conditions affecting the heart and lungs had a worse quality of life, results showed.
Organ involvement, as well as Raynaud’s phenomenon and GI problems independently, were also significantly associated with a poorer quality of life in both incident and prevalent (long-standing) cases.
In addition, HRQoL worsened over time in both groups, with incident cases showing poorer quality of life during the first two years of follow-up, after which their HRQoL curves became generally similar to those of people with prevalent disease.
Notably, Raynaud’s phenomenon, GI symptoms, and digital ulcers were identified as significant risk factors for quality of life worsening over time in both patient groups.
Among functional difficulties, poorer walking abilities, reduced hand function, and lower maximum mouth opening were all found to be significantly associated with HRQoL worsening over time in both groups.
Skin and heart involvement, as well as poorer walking abilities, had a larger influence on HRQoL in the incident group, while PAH was found to be an important risk factor only in patients with long-standing disease.
To the researchers’ surprise, the presence of ILD did not significantly affect quality of life over time. They hypothesized that this may be due to such patients being treated more aggressively and earlier — which could have an overall beneficial impact on disease severity and quality of life — or to these patients learning to cope with their more severe disease.
These findings suggest that SSc patients’ daily life “is significantly affected by relatively less severe, but troublesome and difficult to control symptoms, including Raynaud’s and GI complaints,” the researchers wrote.
Data also highlight “the possibility that these factors may predict further HRQoL deterioration,” the team wrote, adding that “major attention should be paid to GI symptoms, Raynaud and [digital ulcers] as possible predictors of worsening HRQoL.”
“Deeper knowledge of factors significantly influencing not only HRQoL, but also changes of HRQoL over time, is of relevance to tailor most appropriate treatment strategies,” the team concluded.