Background: Systemic sclerosis (SSc) is a chronic disabling disease, inducing functional impairment by the definitory cutaneous manifestation, as well as by musculo-articular and cardio-pulmonary involvement. The Scleroderma Health Assessment Questionnaire (SHAQ), a tool designed to assess functional impairment, has recently been validated into Romanian.
Objectives: This paper aims to compare, from the point of view of disability, the two disease subsets of SSc defined by LeRoy, limited cutaneous SSc (lc-SSc) and diffuse cutaneous SSc (dc-SSc), and to assess the relation between disability and each organ/system involvement of SSc.
Methods: Fifty-four SSc patients were included (47 women, 7 men), mean age 49.8±13.3 years and mean disease duration from first non-Raynaud symptom 5.6±6.5 years. Twenty-four patients (45%) belonged to the dc-SSc subset and 30 (55%) to the lc-SSc subset. All patients were extensively investigated, self-assessed pain and fatigue (on 100 mm VAS, where 0 is for no pain and no fatigue respectively) and completed the SHAQ questionnaire.
Results: SHAQ scores had a broad spectrum (from 0, meaning no disability, to 3, meaning greatest possible disability), with a mean±DS of 1.13±0.78. Patients of the dc-SSc subset had significantly higher SHAQ scores (1.25±0.87 vs. 1.02±0.71; p=0.03 by Mann-Whitney U test) and a tendency towards more joint involvement (63% vs. 53%, 2 =0.46, p=0.50) and more pulmonary involvement (70% vs. 43%, 2=3.6, p=0.057) than lc-SSc patients. Multiple linear regression showed that pain (due mostly to joint involvement), fatigue, hand impairment (measured by finger-to-palm distance in cm) and dyspnoea (expressed as NYHA class) are the main causes of disability, assessed by the SHAQ index.

Conclusion: SSc-related disability is clinically significant and is mostly related to joint, cutaneous and cardio-pulmonary involvement, as well as to the disease-induced fatigue.