Hand Impairment in Systemic Sclerosis: Various Manifestations and Currently Available Treatment

Abstract

OPINION STATEMENT Systemic sclerosis (SSc) is an autoimmune disease initially recognized by hand involvement due to characteristic Raynaud's phenomenon (RP), puffy hands, skin thickening, and contractures resembling claw deformities. SSc contributes to hand impairment through inflammatory arthritis, joint contractures, tendon friction rubs (TFRs), RP, digital ulcers (DU), puffy hands, skin sclerosis, acro-osteolysis, and calcinosis. These manifestations, which often co-exist, can contribute to difficulty with occupational activities and activities of daily living (ADL), which can result in impaired quality of life. However, despite this knowledge, most diagnostic and treatment principles in SSc are focused on visceral manifestations due to known associations with morbidity and mortality. Treatment of inflammatory arthritis is symptom based and involves corticosteroids ≤10mg daily, methotrexate, tumor necrosis factor inhibitors, tocilizumab, and abatacept. Small joint contractures are managed by principles of occupational hand therapy and rarely surgical procedures. TFRs may be treated similar to inflammatory arthritis with corticosteroids. All patients with RP and DU should keep digits covered and warm and avoid vasoconstrictive agents. Pharmacologic management of RP begins with use of calcium channel blockers, but additional agents that may be considered are fluoxetine and phosphodiesterase 5 (PDE5) inhibitors. DU management also involves vasodilators including calcium channel blockers and PDE5 inhibitors; bosentan has also been shown to prevent DU. In patients with severe RP and active DU, intravenous epoprostenol or iloprost can be used and surgical procedures, such as botulinum injections and digital sympathectomies, may be considered. For those with early diffuse cutaneous SSc needing immunosuppression for skin sclerosis, methotrexate or mycophenolate mofetil can be used, but the agent of choice depends on co-existing manifestations, such as inflammatory arthritis and/or lung involvement. Various pharmacologic agents for calcinosis have been considered but are generally ineffective; however, surgical options, including excision of areas of calcinosis, can be considered. Overall management of hand impairment for all patients with SSc should include occupational hand therapy techniques such as range of motion exercises, paraffin wax, and devices to assist in ADL. Thus, treatment options for the various manifestations contributing to hand impairment in SSc are limited and often modestly efficacious at best. Robust studies are needed to address the manifestations of SSc that contribute to hand impairment.

Possible pro‐inflammatory role of heparin‐binding epidermal growth factor‐like growth factor in the active phase of systemic sclerosis

“…Other dermal vascular symptoms include nailfold bleeding and telangiectasia. Contracture of phalanges occurs as a result of fibrosis of the skin and peritendonious tissue and is seen already in the early years after disease onset . In clinical practise, both KL‐6 and surfactant protein D are well known to be serum markers for ILD .…”

Inter‐rater reliability of modified hand mobility in scleroderma test

“…mHAMIS is a short, inexpensive and practical test developed to evaluate the hand mobility of patients with SSc . Because of these advantages, it is frequently used in clinical studies including hand‐related evaluations in individuals with SSc …”

Responsiveness to volume change of calcinosis cutis utilizing dual-energy computed tomography in a patient with diffuse cutaneous systemic sclerosis treated with alendronate

“…Calcinosis cutis is defined as the deposition of insoluble calcium salt in the skin and subcutaneous soft tissues, which is frequently manifested in patients with systemic sclerosis (Ssc) [1,2]. It occurs in about 25% of patients with SSc and can occur in any subset of SSc but is often seen in limited cutaneous systemic sclerosis [3].…”