Presence of Diffuse Cutaneous Disease Linked to Worse HRQOL in Systematic Sclerosis

A study presented at the ACR Convergence 2024 meeting found that, among patients with early systemic sclerosis, the presence of diffuse cutaneous disease, musculoskeletal manifestations, higher GIT score, and higher mRSS were linked to a worse health-related quality of life (HRQOL).

The first years of systemic sclerosis are the period with peak disease activity and are associated with greater pain, fatigue, sleep disruption, physical impairment, anxiety, and body image distress. Investigators sought to evaluate the physical and mental health of patients with systemic sclerosis.

“One of the biggest challenges of systemic sclerosis in the early years of disease is the very high disease activity, progression of skin, that really happens fast in the first couple of years,” said Ashima Makol, MD, MBBS, from Mayo Clinic, during an interview at ACR Convergence. “[It is] also associated with tightening of the skin, joint inflammation, a lot of contractures that patients develop over the course of the disease that can impact physical function. It can impact body image with all of that skin movement, which is very challenging for patients from a mental health perspective and adjusting to this new normal.”

Participants were enrolled in the Collaborative National Quality and Efficacy Registry (CONQUER), a multicenter US-based registry of patients with early limited cutaneous and diffuse cutaneous systemic sclerosis. They all met the 2013 ACR-EULAR classification criteria for systemic sclerosis and had a disease duration of < 5 years from their first non-Raynaud systemic sclerosis symptom. The sample had a mean age of 53.7 years, with most White (69.7%), followed by Hispanic (10.8%), Black (10.8%), Asian (6.7%), and Other (1.9%).

To evaluate health-related quality of life, participants completed the Patient-Reported Outcomes Measurement Information System-29 version 2.0 (PROMIS-29v2). Physical and mental health was assessed in 7 domains: physical function, social function, pain interference, fatigue, anxiety/fear, sleep disturbance, and depression. The team compared the domains for patients with limited cutaneous systemic sclerosis and those with diffuse cutaneous systemic sclerosis.

More than half of the participants (68.7%) had diffuse cutaneous systemic sclerosis. The mean disease duration was 2.6 years, with similar durations for limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis (P < .001). Moreover, patients with diffuse cutaneous systemic sclerosis had significantly worse physical pain (P < .001), social function (P < .001), and pain interference (P = .002) but no significant differences for fatigue (P = .075), anxiety or fear (P = .726), sleep disturbance (P = .632), and depression (P = .063).

Investigators saw greater GIT scores, which indicated worse GI functioning, were linked to greater fatigue, pain interference, and sleep disturbance. Meanwhile, greater GI scores were related to poorer physical and social function.

Furthermore, musculoskeletal involvement, particularly with large joint contractures (P < .001), small joint contractures (P < .001), and tendon friction rubs (P < .001), demonstrated statistically significant associations with the PROMIS-29v2 domain scores. The presence of small joint contractures and the use of ambulatory aids were linked to worse scores in several domains.

“We didn’t see… differences in anxiety or depression, and that was somewhat surprising,” Makol said, “but it seems like limited and diffusion cutaneous patients are impacted as much as each other, which is why differences in them don’t really come out as well, but they are doing much worse compared to… the general population.”

Relevant disclosure for Makol includes GENZYME Corporation.

References

^{Makol, A, Lamba, I, Khanna, D. Clinical Correlates of Physical and Mental Health in Early Systemic Sclerosis: Data from the Collaborative National Quality and Efficacy (CONQUER) Registry. American College of Rheumatology. Presented at ACR Convergence 2024 in Washington DC from November 14 – 19, 2024.}