Jerjen, Rebekka; Nikpour, Mandana; Krieg, Thomas; Denton, Christopher P; Saracino, Amanda M; (2022) Systemic sclerosis in adults. Part II: Management and therapeutics. Journal of the American Academy of Dermatology , 87 (5) pp. 957-978. 10.1016/j.jaad.2021.10.066.

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Abstract

The management of systemic sclerosis (SSc) is complex, evolving and requires a multidisciplinary approach. At diagnosis and throughout the disease course, clinical assessment and monitoring of skin involvement via the modified Rodnan Skin Score, patient reported outcomes and new global composite scores (such as the CRISS, which also considers lung function), is vital. Immunomodulation is the mainstay of skin fibrosis treatment, with mycophenolate mofetil considered first line. Meanwhile vasculopathy related manifestations (Raynaud's phenomenon, digital ulcers) and calcinosis, require general measures combined with specific pharmacological (calcium channel blockers, phosphodiesterase type 5 inhibitors and prostanoids) and non-pharmacological (digital sympathectomy and botulinum toxin injections), and often multifaceted, management approaches. Patients should be specifically screened for systemic manifestations at the time of diagnosis, and regularly thereafter and treated accordingly. Numerous targeted therapeutic options for SSc, including skin fibrosis, are emerging and include B-cell depletion, anti-IL-6, JAK and TGF-β inhibition. The second article in this continuing medical education (CME) series discusses these key aspects of SSc assessment and treatment, with particular focus on skin involvement. It is vital that dermatologists play a key role in the multidisciplinary approach to SSc management.

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