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

Abstract

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.

Keywords: Raynaud's phenomenon; acro-osteolysis; arthralgias; calcinosis; digital ulcers; hand impairment; hand involvement; inflammatory arthritis; joint contractures; puffy hands; scleroderma; skin sclerosis; systemic sclerosis; tendon friction rubs.

Figure 1

Comparison of the various manifestations of hand impairment in SSc based on disease subtype and appearance during disease course.

Figure 2

Hand contractures in SSc (claw hand deformity). Patient with dcSSc with severe contractures including hyperextension of MCP joints (red arrow), flexion contractures of PIP joints (green arrow) and flexion contractures of DIP joints (yellow arrow).

Figure 3

Importance of TFRs in SSc [2, 28, 29, 30, 32]

Figure 4

Vascular complications of SSc. Cyanotic phase of RP (blue arrow). Active digital ulcer (red arrow).

Figure 5

Phases of skin involvement in SSc. A. Initial edematous phase of SSc manifested as puffy hands. B. Sclerodactyly of the digits of the hand due to skin sclerosis resulting in tight skin and loss of skin folds of the fingers. Contractures of PIP and DIP joints are also present in this patient. C. A patient with severe dcSSc with areas of atrophy beginning over MCP joints but no appearance of wrinkles, so this is the initial phase of skin atrophy. Contractures of PIP and DIP joints are also present. D. A patient with severe dcSSc with prior skin thickening but now is in the later stages of atrophy with thin skin and reappearance of wrinkles resembling normal skin. Contractures of PIP and DIP joints are also present.

Figure 6

Acro-osteolysis. A. Plain radiographs with resorption of bilateral 1^st, 2^nd and 3^rd distal phalanxes (red arrows). Also areas of calcinosis (blue arrows) and erosions (yellow) noted. B. Acro-osteolysis can be seen on physical examination here with loss of the 2^nd and 3^rd distal phalanges (arrows).

Figure 7

Calcinosis. A. and B. Extruded calcium salts from the digits of a patient with calcinosis. C. Calcinosis with associated skin breakdown on the 1^st digit of the hand (arrow). D. Calcinosis on radiographs near the IP joint and 2^nd PIP of the left hand (arrows). E. Calcinosis involving 2^nd digit of the right hand (arrow).