Management of Raynaud’s phenomenon in systemic sclerosis—a practical approach

Fernández‐Codina, Andreu; Cañas-Ruano, Esperanza

doi:10.1177/2397198318823951

Cited by 17 publications

(5 citation statements)

References 43 publications

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“…The focus of this review is recent advances: current treatment of Raynaud's phenomenon and digital ulceration are described fully elsewhere [39][40][41][42][43]. However, to orientate the reader, a stepwise approach to management is summarized in Fig.…”

Section: Treatmentmentioning

confidence: 99%

Raynaud's phenomenon and digital ulcers: advances in evaluation and management

Herrick¹

2021

Current Opinion in Rheumatology

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Purpose of reviewThe aim of this review is to give an update on advances in evaluation and management of systemic sclerosis (SSc)-related Raynaud's phenomenon and digital ulceration, focusing on reports from the last 18 months. The increasing recognition of the huge impact of Raynaud's phenomenon and of digital ulceration on the everyday lives of patients with SSc has sparked enthusiasm internationally to develop better outcome measures and better treatments, and so a review is timely.Recent findingsThere have been recent advances in the development of patient reported outcome instruments [e.g. the Hand Disability in Systemic Sclerosis-Digital Ulcers (HDISS-DU) instrument] and also in noninvasive imaging techniques, including thermography and laser Doppler methods. Improved outcome measures will facilitate future clinical trials, both early phase proof-of-concept and later phase trials. New insights have been gained into mechanisms of action and methods of administration of ‘conventional’ therapies, for example phosphodiesterase inhibitors and intravenous prostanoids. New treatment approaches are being investigated, including topical and procedural therapies.SummaryClinicians can look forward to seeing these advances translating into clinical benefit over the next 5 years. To help ensure this, they should strive whenever possible to recruit patients with SSc-related digital vasculopathy into observational studies and clinical trials.

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Section: Treatmentmentioning

confidence: 99%

Raynaud's phenomenon and digital ulcers: advances in evaluation and management

Herrick¹

2021

Current Opinion in Rheumatology

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“…In secondary prevention of digital ulcers, bosentan could also be of interest in adults. 38 Children with head and neck morphea should have ophthalmologic examinations to monitor for asymptomatic involvement that may lead to irreversible damage.…”

Section: Therapeutic Approachmentioning

confidence: 99%

Skin involvement of juvenile scleroderma

Gacem,

Hadef,

Samir Ladj

2023

JDC

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Pediatric scleroderma is a rare chronic inflammatory disease in children. It includes two major clinical entities, systemic sclerosis (SSc) and localized scleroderma (LS). The two forms have a common mechanism but their clinical manifestations differ. Skin involvement occupies an important place in diagnostic classifications due to the richness of dermatological clinical manifestations and their suggestive aspect of the disease. If the vital prognosis is often not compromised, the functional prognosis is strongly affected, sometimes with deleterious aesthetic problems. Very few pediatric publications have been produced. In this article, we report the different skin manifestations inherent to this disease, and their management in children.

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“…Meta-analyses have suggested benefit in SSc-related RP 26,27 and most clinicians would now recommend PDE5 inhibitors as second line after calcium channel blockers, with which they can be used in combination. 28 Although PDE5 inhibitors have been less studied for the treatment of digital ulcers than for RP, it is likely that they confer some benefit. The Establish the diagnosis of SSc as early as possible, including subtype and autoantibody status (both of which predict disease trajectory)…”

Section: Advances In Managementmentioning

confidence: 99%

Advances in the Treatment of Systemic Sclerosis

Herrick¹

2022

Rheumatology

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Although systemic sclerosis (SSc) is currently incurable, there have been recent advances in treatment. This review article begins by providing a brief background to SSc in terms of disease subtyping and autoantibodies, because both predict disease trajectory and help clinicians to select appropriate monitoring and treatment protocols. Broad principles of management are then described: ‘disease-modifying’ therapies and therapies directed at reducing disease burden and/or progression of SSc-related digital vascular disease and of internal organ involvement. Next, advances in the management of digital vasculopathy, pulmonary arterial hypertension (PAH), interstitial lung disease (ILD) and early diffuse cutaneous SSc are discussed in turn, for example: (a) increased use of phosphodiesterase inhibitors and endothelin receptor antagonists for digital vasculopathy; (b) early recognition and treatment of PAH, including with combination therapies; (c) increased use of mycophenolate mofetil and of nintedanib in ILD; and (d) immunosuppression now as standard practice in early diffuse cutaneous SSc, and autologous haematopoietic stem cell transplantation for highly selected patients with progressive diffuse disease. Finally, future challenges are discussed, including ensuring that all patients with SSc are monitored and treated according to best practice guidelines, and whenever possible giving patients the opportunity to participate in clinical trials.

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Management of Raynaud’s phenomenon in systemic sclerosis—a practical approach

Cited by 17 publications

References 43 publications

Raynaud's phenomenon and digital ulcers: advances in evaluation and management

Raynaud's phenomenon and digital ulcers: advances in evaluation and management

Skin involvement of juvenile scleroderma

Advances in the Treatment of Systemic Sclerosis

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