Joint and tendon involvement predict disease progression in systemic sclerosis: a EUSTAR prospective study

Avouac, Jérôme; Walker, Ulrich A.; Hachulla, É.; Riemekasten, Gabriela; Cuomo, Giovanna; Carreira, Patrícia; Caramaschi, Paola; Ananieva, L.; Matucci‐Cerinic, Marco; Czirják, László; Denton, Christopher P.; Ladner, Ulf Müller; Allanore, Yannick

doi:10.1136/annrheumdis-2014-205295

Cited by 118 publications

(84 citation statements)

References 25 publications

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“…This association was not found in a previous study,7 which might be explained by two major differences in our study design. Only patients with ILD extent <20% lung involvement on HRCT were included in our cohort, which was different from the cohort selection of overall SSc-ILD or patients with SSc in previous studies.…”

Section: Discussioncontrasting

confidence: 88%

Prediction of progression of interstitial lung disease in patients with systemic sclerosis: the SPAR model

et al. 2018

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Section: Discussioncontrasting

confidence: 88%

Prediction of progression of interstitial lung disease in patients with systemic sclerosis: the SPAR model

et al. 2018

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Section: Discussionmentioning

confidence: 93%

“…TFR were associated with diffuse and reduced survival in 1301 patients with SSc 32. This item was predictive of worsening of skin fibrosis and scleroderma renal crisis in the EUSTAR cohort 32. CRP levels were increased in early disease and were associated with activity, skin, lung, kidney disease and poor survival in 1043 patients with SSc from the CSRG Registry 33…”

Section: Discussionmentioning

confidence: 99%

The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index

Valentini

Iudici

Walker³

et al. 2016

Ann Rheum Dis

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“…In the European League Against Rheumatism (EULAR) Scleroderma Trials and Research group (EUSTAR) registry, synovitis had a prevalence of 16% [3]. Synovitis occurred more often in dcSSc and was a predictive factor for dcSSc, pulmonary hypertension, muscle weakness, new DU, and decreased left heart function [3, 4]. …”

Section: Arthralgias and Inflammatory Arthritismentioning

confidence: 99%

“…TFRs were initially discovered in a patient with dcSSc in 1876 and recently have been shown to have a prevalence of 11% according to the EUSTAR registry [3, 4, 28]. They are described as leathery crepitus on palpation of moving joints, which is thought to be due to fibrinous deposits on the surface of tendon sheaths and overlying fascia [2].…”

Section: Tendon Involvementmentioning

confidence: 99%

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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Joint and tendon involvement predict disease progression in systemic sclerosis: a EUSTAR prospective study

Abstract: Joint synovitis and TFRs are independent predictive factors for disease progression in patients with early SSc. These easily detected clinical markers may be useful for the risk stratification of patients with SSc.

Cited by 118 publications

References 25 publications

Prediction of progression of interstitial lung disease in patients with systemic sclerosis: the SPAR model

Prediction of progression of interstitial lung disease in patients with systemic sclerosis: the SPAR model

The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index

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

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