Systemic sclerosis in adults. Part II: management and therapeutics

Jerjen, Rebekka; Nikpour, Mandana; Krieg, Thomas; Denton, Christopher P.; Saracino, Amanda

doi:10.1016/j.jaad.2021.10.066

Cited by 9 publications

(6 citation statements)

References 200 publications

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“…Bosentan has been shown to be effective in treating and preventing DU [ 11 - 13 ]. However, macitentan did not meet the primary outcome (the cumulative number of new DUs from baseline to week 16) in clinical trials (DUAL-1 and DUAL-2) [ 18 ].…”

Section: Discussionmentioning

confidence: 99%

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Macitentan for the Treatment of Refractory Digital Ulcers in Patients With Connective Tissue Diseases

Abánades¹,

Mozo²,

Hernán³

et al. 2023

Cureus

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Systemic sclerosis (SSc) is a chronic autoimmune disease with complex pathogenesis, characterized by vascular dysfunction and fibrosis. Digital ulcers (DUs) are a common and severe complication in SSc patients, negatively impacting their quality of life. This retrospective study evaluates the use of macitentan, an endothelin receptor antagonist, in six female patients with connective tissue disease (CTD) and sclerodermiform features (five SSc and one mixed connective tissue disease) for the treatment of refractory DUs. Macitentan demonstrated a safe and effective alternative to bosentan, reducing DU relapses, hospitalizations, and the use of systemic prostaglandin therapy. The findings suggest that macitentan may be a valuable therapeutic option in specific cases of recurrent or refractory DUs and warrant further investigation in larger, long-term studies.

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Section: Discussionmentioning

confidence: 99%

“…Currently, bosentan is the only ERA authorized in Europe for the prevention of the recurrence of DUs in patients with SSc [ 11 ]. This recommendation is based on two clinical trials that demonstrated the efficacy of bosentan for this indication [ 12 , 13 ].…”

Section: Introductionmentioning

confidence: 99%

Macitentan for the Treatment of Refractory Digital Ulcers in Patients With Connective Tissue Diseases

Abánades¹,

Mozo²,

Hernán³

et al. 2023

Cureus

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“…A detailed description of management is outside the scope of this review but can be found elsewhere. [9][10][11][12] Certain broad principles apply (Figure 1). Early diagnosis is key to allowing early identification, monitoring and treatment of different 'complications' of SSc before irreversible tissue injury has occurred or progressed.…”

Section: General Principles Of Managementmentioning

confidence: 99%

“…Discussion of these is outside the scope of this review, but can be found elsewhere. 12,105 Randomized controlled trials are challenging due to the rarity of early dcSSc and the problems inherent in current outcome measures; for example, the modified Rodnan skin score 106 (often the primary outcome measure) has a large interobserver variability. 107 Whenever possible, patients with dcSSc should be recruited into one of the ongoing clinical trials because only in this way will effective treatments be identified.…”

Section: Future Therapiesmentioning

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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“…Locally injected into the digit or the palmar skin, it may reduce severity of RP attacks. 5 Iloprost could be proposed as a second-line treatment after failure of calcium channel blockers – Correct. Iloprost is an analog of prostacyclin and may be used as a second-line treatment of RP, as it reduces the frequency and severity of the RP attacks.…”

mentioning

confidence: 99%