Musculoskeletal Involvement in Systemic Sclerosis: An Unexplored Aspect of the Disease

Abstract

Musculoskeletal (MSK) symptoms in patients with systemic sclerosis (SSc) include articular involvement (arthralgia, synovitis, contractures), which is often an early phenomenon and significantly contributes to the disability. Predominantly the hands are affected. Consensus in outcome measures of articular involvement is missing. Health Assessment Questionnaire Disability Index (HAQ-DI), Cochin Hand Function Scale (CHFS), Hand Mobility Index in Scleroderma (HAMIS), and Disease Activity Score of 28 Joints (DAS28) may be used for the assessment of different aspects of joint involvement. There is an unmet need for therapies confirmed by randomized controlled clinical trials (RCTs) to treat both synovitis and non-inflammatory joint involvement. The few rehabilitation studies that have been conducted have shown some promising efficacy. Muscle involvement may be an early symptom. The presence of clinically meaningful muscle involvement often heralds an unfavourable prognosis. The histology of muscle biopsy shows a variable picture including inflammation and necrosis. Besides, signs of acute neurogenic atrophy have been recently described as a previously underestimated contributor to muscle weakness. Similar to articular involvement, the lack of classification criteria on inflammatory and non-inflammatory SSc-associated myopathies, and the lack of validated core set of outcome measures makes it difficult to perform RCTs. The SSc-specific fibrinous tenosynovitis (tendon-friction rubs /TFRs/) is a frequent finding in SSc. Patients with TFR are at increased risk of developing renal, vascular, cardiac and gastrointestinal involvement and have reduced survival rates. Changes of fibrinous tenosynovitis can be objectively detected by ultrasound and may be used as an outcome measure in the treatment of MSK involvement.