Early diffuse cutaneous systemic sclerosis (scleroderma) is highly associated with fatigue, pain, and disability, according to researchers who evaluated data from the European Scleroderma Observational Study (ESOS; ClinicalTrials.gov identifier: NCT02339441). More specifically, disability appears to be strongly correlated with degree of skin thickening by way of its effect on hand function.
Because clinicians are so often focused on identifying early internal organ involvement, the burden of functional disability has been somewhat overlooked in the literature, particularly when examining early disease. Therefore, investigators were interested in describing the extent of such disability in terms of dermatologic and musculoskeletal impairment.
Participants in ESOS filled out various questionnaires at 0, 12, and 24 months, including the Short Form-36 Health Survey, the Functional Assessment of Chronic Illness Therapy fatigue score, the Cochin Hand Function Scale (CHFS), and the scleroderma-specific Health Assessment Questionnaire with disability index (HAQ-DI). The modified Rodnan skin score, digital ulcer status, and internal organ involvement were also considered. Pain was assessed using a visual analogue scale from 0 to 100.
There were 326 patients with early diffuse cutaneous systemic sclerosis (mean duration, 11.9 months) analyzed at 50 medical centers worldwide. Participants had high disability levels (mean HAQ-DI, 1.1; SD± 0.83), particularly in the “grip” and “activity” categories (49% and 39.0% of respondents, respectively). CHFS scores revealed similar results in terms of disability (mean, 18.7; SD±20.7), with fine finger movements proving especially difficult. These elevated HAQ-DI and CHFS scores were associated with increased modified Rodnan skin score (ρ, 0.34 [P <.0001] and ρ, 0.35 [P <.0001], respectively), indicating a positive correlation between disability and skin thickening.
Not unexpectedly, all the measures used (HAQ-DI, CHFS, Functional Assessment of Chronic Illness Therapy, and Short Form-36) were highly associated, with the strongest correlation existing between the HAQ-DI and CHFS (ρ, 0.84; P <.0001). In terms of clinical associations, the HAQ-DI was highest in patients with muscle involvement (P =.002), cardiac dysfunction (P =.005), pulmonary fibrosis (P =.005), and digital ulcers (P =.004). Pain was also associated with skin thickening, as well as all disability indices. Considering trends in disability, investigators found that deteriorating HAQ-DI scores across 12 months were highly correlated with decreasing hand function (ρ, 0.57; P <.0001), rising fatigue (ρ, −0.53; P <.0001), and increasing modified Rodnan skin score (ρ, 0.40; P <.0001).
The authors noted that their results likely indicate the importance of skin thickening in contributing to the impairment of hand function (secondary to stiff, painful hands), which leads to increased overall disability. They strongly recommend that patients with early diffuse cutaneous systemic sclerosis be treated with a multidisciplinary approach that includes occupational therapy, physiotherapy, and pain management to achieve optimal results and the best quality of life.
Peytrignet S, Denton CP, Lunt M, et al. Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study [published online November 30, 2017]. Rheumatology (Oxford). doi: 10.1093/rheumatology/kex410