Abatacept Improves Skin-score and Reduces Lesions in Patients with Localized Scleroderma: A Case Series

Fage, Simon; Arvesen, Kristian Bakke; Olesen, Anne Braae

doi:10.2340/00015555-2878

Cited by 28 publications

(10 citation statements)

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“…This can dampen B cell:T-cell interactions and T-cell functional activation, which in turn would lead to less inflammatory driven fibrosis and potentially less auto-antibody production. A few cases reports have described successful treatment of morphea with abatacept, especially widespread disease or deeper disease (monitored by MRI in some) (63–65). Further clinical studies in trial format are warranted to further understand the utility of these biologic agents in treating morphea.…”

Section: Discussionmentioning

confidence: 99%

Autoantibodies in Morphea: An Update

et al. 2019

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Skin autoimmune conditions belong to a larger group of connective tissue diseases and primarily affect the skin, but might also involve underlying tissues, such as fat tissue, muscle, and bone. Autoimmune antibodies (autoantibodies) play a role in autoimmune skin diseases, such as localized scleroderma also termed morphea, and systemic scleroderma, also called systemic sclerosis (SSc). The detailed studies on the biological role of autoantibodies in autoimmune skin diseases are limited. This results in a few available tools for effective diagnosis and management of autoimmune skin diseases. This review aims to provide an update on the detection and most recent research on autoantibodies in morphea. Several recent studies have indicated the association of autoantibody profiles with disease subtypes, damage extent, and relapse potential, opening up exciting new possibilities for personalized disease management. We discuss the role of existing autoantibody tests in morphea management and the most recent studies on morphea pathogenesis. We also provide an update on novel autoantibody biomarkers for the diagnosis and study of morphea.

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Section: Discussionmentioning

confidence: 99%

Autoantibodies in Morphea: An Update

et al. 2019

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“…However, cases refractory to MMF have been observed, and the treatment of recalcitrant morphoea remains largely empirical. Recently abatacept, a recombinant fusion protein interfering with the T‐cell costimulatory pathway, has been reported to be effective in two case reports and three small series of adult and paediatric patients (total of 18 cases) 27–30 . My group shared this good empirical experience with abatacept and presented an abstract of a study on three paediatric patients at the European Society for Pediatric Dermatology Meeting in 2019 31 .…”

Section: Second‐line Therapy For Difficult‐to‐treat Casesmentioning

confidence: 99%

Diagnosis and management of morphoea in children: an overview

Weibel

2021

Clin Exp Dermatol

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Summary Paediatric morphoea (localized scleroderma) is an inflammatory sclerosing disorder of the skin and subcutis associated with tissue atrophy. It is thought that the disease develops on the background of genetic predisposition (e.g. mosaicism for the common linear variant) initiated by various trigger factors, and that detected autoantibodies and inflammatory cytokines represent secondary epiphenomena. In contrast to the common belief that morphoea is a benign self‐limiting disorder, long‐term data indicate that its chronicity, relapsing nature and extracutaneous complications lead to significant morbidity, particularly when the disease starts in early childhood. Early recognition may be challenging, and the most important clinical clues are band‐like distribution, atrophy of underlying tissue, skin sclerosis, and localized loss of body/scalp hair, eyelashes or eyebrows. Extracutaneous manifestations occur in up to 20% of patients, with arthritis/arthralgia and neurological symptoms being most frequently observed, followed by ophthalmological complications such as uveitis. Corticosteroids and methotrexate are highly effective as first‐line therapy in morphoea, leading to partial reversal of skin manifestations. However, the development of atrophy is not sufficiently prevented by standard therapy. There is a relapse rate of 25%–48% within the first years after stopping treatment, thus long‐term follow‐up is warranted. Mycophenolate mofetil seems to be a beneficial second‐line therapy, and a new drug, abatacept, also seems to be a promising and well‐tolerated second‐line treatment option. Additionally, autologous fat injections are beneficial and may be used as an adjunct to ongoing therapy.

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“…4 Subsequent cases have been reported demonstrating improvement in morphea treated with IV abatacept, with some patients subsequently later being switched to subcutaneous abatacept. 5…”

Section: Case Descriptionmentioning

confidence: 99%

A case of recalcitrant linear morphea responding to subcutaneous abatacept

Talia

Bitar

Wang

et al. 2021

Journal of Scleroderma and Related Disorders

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Introduction: Linear morphea is an inflammatory condition that is often treated with systemic glucocorticoids and methotrexate, with mycophenolate mofetil being used as an alternative agent. However, there are few published reports on beneficial effect of abatacept for refractory disease. We present a case of a woman in her 30s who presented with linear morphea on her scalp, with a notable response following the addition of subcutaneous abatacept. Methods: Computational analysis was performed comparing the immune cell scores of skin biopsies from five morphea skin biopsies from three additional unique patients and 15 healthy control skin biopsies. Value of p < 0.05 was considered statistically significant. Results: Immune cell scores demonstrated a statistically significant enrichment of activated CD4 memory T cells, M1 macrophages, monocytes, and memory B cells comparing skin biopsies of morphea versus healthy controls (p < 0.05 for all). Discussion: Abatacept may be considered for recalcitrant cases of morphea. Our computational analysis supports a well-designed study is needed to assess abatacept as first-line therapy.

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Abatacept Improves Skin-score and Reduces Lesions in Patients with Localized Scleroderma: A Case Series

Cited by 28 publications

References 0 publications

Autoantibodies in Morphea: An Update

Autoantibodies in Morphea: An Update

Diagnosis and management of morphoea in children: an overview

A case of recalcitrant linear morphea responding to subcutaneous abatacept

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