Results of the Health Assessment Questionnaire for Japanese patients with systemic sclerosis - Measuring functional impairment in systemic sclerosis versus other connective tissue diseases

Objective: To evaluate the physical functional impairment in patients with systemic sclerosis (SSc) using the Health Assessment Questionnaire (HAQ) and to estimate the correlation of HAQ scores with the severity of SSc. Method: One hundred and twenty-four outpatients with connective tissue disease, including 50 patients with SSc, were evaluated using the HAQ. Twelve patients were classified as having diffuse cutaneous SSc (dSSc) and 38 limited cutaneous SSc (lSSc). The severity classification and the guidelines for treatment (2004) were applied to Japanese SSc patients in order to evaluate the relationship between HAQ scores and disease activity in patients with multiple organ involvement. Results: In dSSc the HAQ category scores for eating, walking, grip, activity and the HAQ-disability index (HAQ-DI) showed the greatest deficits in all disease groups. The severity of disease activity correlated significantly with the scores for walking, reach, and the HAQ-DI. The severity of joint, heart, and pulmonary hypertension were correlated independently with the HAQ-DI score by multiple linear regression analysis. Conclusion: Patients with dSSc suffer greater functional impairment than patients with other connective tissue diseases, and improvements in hand use and walking represent very important targets for both drug development and rehabilitation. As improvement in organ involvement (joints, heart as well as pulmonary hypertension) can lead to reduced functional impairment, they constitute an important target for therapy in SSc.