Ischemic Digital Ulcers Affect Hand Disability and Pain in Systemic Sclerosis

Mouthon, Luc; Carpentier, Patrick; Lok, C.; Clerson, Pierre; Gressin, Virginie; Hachulla, É.; Bérezné, Alice; Diot, Élisabeth; Kien, A. Khau Van; Jégo, Patrick; Agard, C.; Duval-Modeste, A.-B.; Sparsa, A.; Puzenat, E.; Richard, M.‐A.; Investigators, Eclipse

doi:10.3899/jrheum.130900

Cited by 62 publications

(58 citation statements)

References 18 publications

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“…Health assessment questionnaire-disability index (HAQ-DI) and Scleroderma HAQ (SHAQ), which has 5 additional SSc-specific visual analog scales (VAS) compared to HAQ-DI, are measures of disability that assess difficulties with ADL and have been validated in SSc. Cochin Hand Function Scale (CHFS) assesses hand function and has also been validated in SSc [5, 11*]. HAMIS administration requires the use of occupational and/or physical therapists for the assessments whereas SHAQ and CHFS are self-reported measures.…”

Section: Arthralgias and Inflammatory Arthritismentioning

confidence: 99%

“…Forty-four to 60% of patients with SSc have had a DU during their disease course [11*]. DU are associated with early onset RP, dcSSc, anti-topoisomerase I antibody, and late and worsening SSc patterns on nailfold videocapillaroscopy [40].…”

Section: Vascular Involvementmentioning

confidence: 99%

“…DU can be complicated by infection, gangrene and amputation. Various studies have shown an association of DU with pain, disability, decreased quality of life, more hospitalizations, progressive cardiovascular disease, and decreased survival [11*, 41]. In a prospective, multicenter, longitudinal study (ECLIPSE), the effect of recurrent ischemic DU on hand disability was assessed in patients who had at least one DU within the previous year and were on treatment with bosentan.…”

Section: Vascular Involvementmentioning

confidence: 99%

“…In a prospective, multicenter, longitudinal study (ECLIPSE), the effect of recurrent ischemic DU on hand disability was assessed in patients who had at least one DU within the previous year and were on treatment with bosentan. The results of this study suggest DU often affect multiple digits on both hands and are significantly associated with pain and hand disability [11*]. In a prospective, observational study using the Digital Ulcers Outcome (DUO) registry, four categories of DU were studied over 2 years: no DU, episodic (only 1 follow up visit with DU), recurrent (at least 2 follow up visits with DU but 1 follow up visit without DU) and chronic (DU present at every follow up visit) [41].…”

Section: Vascular Involvementmentioning

confidence: 99%

“…To prevent DU, patients should be advised to minimize cold exposure, avoid tobacco use, avoid vasoconstrictive over-the-counter and prescription medication use, and avoid hand injuries, including microtrauma [11*]. Pharmacologic treatment for DU is focused on vasodilatory agents including calcium channel blockers, PDE5 inhibitors, such as sildenafil and tadalafil, bosentan, a dual endothelin receptor antagonist, and intravenous prostacyclins, such as epoprostenol or iloprost [11*, 43].…”

Section: Vascular Involvementmentioning

confidence: 99%

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

Young

Namas

Dodge

et al. 2016

Curr Treat Options in Rheum

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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.

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Section: Arthralgias and Inflammatory Arthritismentioning

confidence: 99%

Section: Vascular Involvementmentioning

confidence: 99%