Systemic sclerosis (scleroderma): an integrated challenge in rehabilitation

Abstract

Systemic sclerosis (SSc), a multisystem disease involving the microvascular system and the connective tissue, is considered one of the most difficult rheumatic diseases to treat. The natural history of the disease evolves from an edematous to a scleroatrophic phase following two different temporal patterns: acute or chronic. The former leads to early death, and the latter evolves slowly toward severe disability that deserves rehabilitative intervention. Despite the poor prognosis, recent improvements in diagnosis and treatment have led to longer patient survival, thus increasing the need to intervene against the development of tissue fibrosis and contractures by using appropriate integrated rehabilitation programs. This article does not review the medico-pharmacological management of visceral manifestations of the disease. Rather, it is divided into six parts, which include analyses of the changes in skin, joints and tendons, and muscle induced by SSc; examination of the existing literature on rehabilitation strategies and treatments; discussions of the pain and peripheral sensory-motor system involvement that are present to a greater or lesser extent in almost all patients and influence not only the duration and outcome of rehabilitation but also the patient's family, social life, and working ability; and consideration of ergonomic and occupational interventions. No controlled studies have been done on the few rehabilitation guidelines and specific protocols identified, so it must be emphasized that this article is a summary of opinions expressed in the literature and the authors' own findings. Particularly lacking are studies on such aspects as ergonomics, work intervention, or the management of sexual dysfunction. Experience gained in the rehabilitation of skin burns and other rheumatic diseases forms the basis for a logical approach to SSc patients.