Musculoskeletal Rehabilitation in the Person with Scleroderma

Janet L. Poole

Disclosures

Curr Opin Rheumatol. 2010;22(2):205 

In This Article

Abstract and Introduction

Abstract

Purpose of Review: The purpose of this review is to examine current evidence for the efficacy of rehabilitation techniques for musculoskeletal impairments in persons with scleroderma.
Recent Findings: The current literature on rehabilitation techniques consists of studies evaluating the effectiveness of paraffin wax treatment, hand and face stretching exercises, connective tissue massage and joint manipulation, splints, and aerobic exercise and resistance training. Only four randomized controlled trials were found and except for those studies, the majority of studies involved small sample sizes and no control groups. However, except for splints, these studies show improvement in joint motion, hand function, and cardiopulmonary endurance.
Summary: Although the results from these small studies are promising, larger, randomized controlled studies are needed to fully determine the effectiveness of rehabilitation techniques for persons with scleroderma.

Introduction

Systemic sclerosis or scleroderma is a rare, autoimmune connective tissue disease of unknown cause characterized by skin thickening, vascular insufficiency, or Raynaud's phenomenon and fibrotic changes in the viscera, including the lungs, heart, kidneys, and gastrointestinal tract.[1] Scleroderma is classified into two subtypes: limited systemic sclerosis and diffuse systemic sclerosis.[1] In limited systemic sclerosis, there is gradual skin thickening limited to the distal extremities and face; later viscera is also involved.[1] In diffuse systemic sclerosis, the more severe subtype of the disease, skin thickening occurs more rapidly in the face, neck, and trunk and symmetrically in the fingers, hands, arms, and legs. There is significant early internal organ involvement of the lungs, heart, gastrointestinal tract, or kidneys.[1]

Musculoskeletal impairments in the upper extremities include arthralgia, stiffness, and tendon friction rubs in which fibrinous deposits on the tendon sheaths cause a creaky or rubbing sound when a joint moves.[2] Contractures and deformities in the hand are the most observable musculoskeletal impairment and consist of decreased flexion of the metacarpophalangeal (MCP) joint, decreased extension of the proximal interphalangeal (PIP) joint, and decreased abduction of the thumb.[3,4] At the tips of the digits, digital cutaneous atrophy and digital tuft resorption can occur. Numerous studies have documented hand impairments,[2,3,4,5] but few studies have examined lower extremity musculoskeletal involvement.[6,7] These studies showed that persons with scleroderma had foot ulcers, histories of foot surgery (primarily amputations, difficulty finding shoes that fit[6]) and arthralgias and flexion contractures in the feet;[7] however, the changes in the feet were less common and had a later onset than in the hands.[7]

Although the vascular and fibrotic damage to the organs are well recognized as causes of mortality and disability, the musculoskeletal impairments also lead to significant physical disability.[8,9,10,11,12,13] To date, there have been few clinical studies evaluating the effectiveness of rehabilitation techniques for musculoskeletal impairments; however, the studies that do exist show promising results. It is recognized that pharmaceutical and surgical management are also important in the management of musculoskeletal impairments. However, the purpose of this review is to examine current evidence for the efficacy of rehabilitation techniques for musculoskeletal impairments in persons with scleroderma. The interventions are grouped by technique rather than by the musculoskeletal impairments. Because there are very few studies, the review includes all studies since 1980 that could be found searching PubMed, CINAHL, PEDRO, OTDBASE, and PsychINFO. The search terms used were occupational therapy, physical therapy, exercise, range of motion, heat, stretching, paraffin wax, splints, orthotics, massage, hydrotherapy, therapeutic exercise, resistive exercise, and rehabilitation.

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