Hand disease in scleroderma: a clinical correlate for chronic hand transplant rejection

ABSTRACT: Chronic rejection remains a potential long-term consequence of hand composite tissue allotransplantation (CTA). Scleroderma has already been proposed as a model for chronic facial allograft rejection based on potential parallels of observed progression of disease and pathophysiology course. This study proposes a similar model for how chronic rejection may manifest itself in the context of hand CTA through the functional and psychological assessment of patients with scleroderma, should it occur. METHODS: 100 consecutive patients with a clinical diagnosis of scleroderma were recruited into the study. Subjective assessment of static hand disfigurement was carried out through the use of standardised digital photographs. Hand function was assessed through the measurement of active range of motion (AROM) and using the activities of daily living (ADL) and Disabilities of the Arm, Shoulder & Hand (DASH) questionnaire. Psychological and quality of life evaluation comprised the Hospital Anxiety Depression Scale (HADS) and the SF36 health survey. RESULTS: Examination of standardised digital photographs of subjects revealed a variety of hand changes characteristic of scleroderma, ranging from mild to moderate through to severe. Objective assessment of hand disfigurement did not correlate with duration of disease, nor psychological distress. However, individuals with worsening disfigurement demonstrated poorer AROM. Longitudinally no deterioration in terms of function was seen over time in terms of the DASH and ADL results. Nevertheless deterioration of function did have a significant impact on quality of life. Overall HADS showed 22% of individuals as suffering from clinical levels of anxiety and 10% from clinical depression. CONCLUSION: Chronic rejection has not yet occurred in any of the hand transplants performed to date. Scleroderma results in a spectrum of chronic functional and psychological disability that provides a model for the potential outcome of chronic hand allograft rejection. Findings from this study provide insight into the impact of this progressive disease for patients and contribute to the information and consent process for patients considering hand composite tissue transplantation.