DeSScipher Project Reveals Physicians and Patients Value Different Aspects of Disease Severity

Ana Pamplona, PdD avatar

by Ana Pamplona, PdD |

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localized scleroderma

Recent data from the DeSScipher Project within the EUSTAR (European Scleroderma Trials and Research) group highlights the different perceptions on disability between patients with systemic sclerosis (SSc) and physicians, and identifies the main factors of disability perceived by SSc patients.

The study, “Functional disability and its predictors in systemic sclerosis: a study from the DeSScipher project within the EUSTAR group,” was published in the journal Rheumatology.

The DeSScipher Project (an acronym for deciphering the optimal management of systemic sclerosis) is a multinational, longitudinal study included in the EUSTAR group database. This project includes five observational trials focusing on digital ulcers, hand arthritis, interstitial lung disease, pulmonary hypertension, and severe heart disease, without focusing on any specific organ manifestations.

SSc is an unusual and highly heterogeneous disease that affects several organs. The evaluation of SSc severity and its impact on the patient’s daily life depends on various factors due to the multiorgan nature of the disease and the different perception between patients and physicians.

Therefore, the most important objective of care when dealing with SSc patients is to improve symptoms, disability, health-related quality of life (QoL), and functional ability.

In the study, 944 SSc patients from the prospective DeSScipher cohort completed the scleroderma health assessment questionnaire (SHAQ), a disability score combines a health assessment questionnaire and five visual analogue scales. This is a self-reported questionnaire with 20 questions covering several areas such as rising, eating, walking, hygiene, dressing, reach, grip, and usual activities.

The authors found that 59% of the patients were in the mild to moderate disability category, 34% in the moderate to severe, and 7% in the severe to very severe category.

The study showed significant impaired functional capacity in a large number of SSc patients. Dyspnea (shortness of breath), pain, digital ulcers, muscle weakness, and gastrointestinal symptoms were the most significant symptoms recorded by the patients that were associated with high disability scores.

Key findings also revealed that patients and physicians emphasize different aspects in the evaluation of SSc severity. While patients focus on gastrointestinal symptoms, pain, dyspnea, digital ulcers, and weakness, the doctors were primarily focused on objective measures of function, like pulmonary function tests.

In clinical practice, objective measurements of gastrointestinal symptoms, muscle weakness, and digital ulcers are rarely performed.

The team suggests that more studies are needed to better understand and overcome the main factors of disability in systemic sclerosis to improve patients’ quality of life.