Changes in Disease Activity and Damage Over Time in Patients With Morphea

O’Brien, Jack C.; Nymeyer, Hugh; Green, Allison; Jacobe, Heidi

doi:10.1001/jamadermatol.2020.0034

Cited by 25 publications

(48 citation statements)

References 22 publications

(30 reference statements)

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“…Although >90% of patients with morphoea achieve disease remission with standard therapy, there are certain drawbacks in the long‐term course of affected patients 4 . Current data consistently confirm a relapse rate of 25%–48% after stopping treatment 4,10,12,23 …”

Section: Long‐term Outcomementioning

confidence: 95%

“…5). 4,18 Guiding therapy remains challenging due to the lack of reliable and easy‐to‐use parameters of disease activity. Currently, treating physicians mainly rely on clinical examination and photographs and may use the Localized Scleroderma Cutaneous Assessment Tool to document changes 22 …”

Section: Adequate Therapymentioning

confidence: 99%

“…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 onset occurs during childhood 4 . The reported incidence of morphoea is 0.3–3 per 100 000 children; however, in paediatric dermatology centres this condition is quite regularly seen, and contributes to frequent and long‐term follow‐up visits 5 .…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Long‐term Outcomementioning

confidence: 95%

Section: Adequate Therapymentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Diagnosis and management of morphoea in children: an overview

Weibel

2021

Clin Exp Dermatol

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“…Damage that results after active lesions progress to an inactive state include atrophy, pigment changes and functional impairment. Damage tends to remain stable or increase after successful management of active disease (61). Once lesions are clinically inactive, treatment centers around improving quality of life by addressing cosmetic and functional concerns.…”

Section: Diagnosis and Treatmentmentioning

confidence: 99%

“…Until recently, morphea was thought to be unresponsive to therapy largely because existing outcomes did not measure the effect of treatment on activity. Many previously reported outcome measures in morphea exclusively measured damage, and therefore remained unchanged even with successful immunosuppressive treatment, which exclusively improves activity and stabilizes damage (61). Patientreported outcomes (PRO), which are critical to assessing the effect of therapy on patients, are equally challenging to quantify in morphea.…”

Section: Clinical Outcome Measuresmentioning

confidence: 99%

Morphea: progress to date and the road ahead

et al. 2021

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Morphea is a rare autoimmune condition causing inflammation and sclerosis of the skin and underlying soft tissue. It is characterized by periods of activity (inflammation admixed with fibrosis), ultimately resulting in permanent damage (pigment change and tissue loss). Damage resulting from unchecked activity can lead to devastating, permanent cosmetic and functional sequelae including hair loss; cutaneous, soft tissue and bony atrophy; joint contractures; and growth restriction of the affected body site in children. This makes the early identification of activity and initiation of appropriate treatment crucial to limiting damage in morphea. To this end, recent investigative work has focused on validation of clinical, biomarker, imaging, and histologic outcomes aimed at accurately quantifying activity and damage.Despite promising results, further work is needed to better validate these measures before they can be used in the clinic and research settings. Although there has been recent approval of less toxic, targeted therapies for many inflammatory skin conditions, none have been systematically investigated in morphea.The mainstays of treatment for active morphea are corticosteroids and methotrexate. These are often limited by substantial toxicity. The paucity of new treatments for morphea is the result of a lack of studies examining its pathogenesis, with many reviews extrapolating from research in systemic sclerosis. Recent studies have demonstrated the role of dysregulated immune and fibrotic pathways in the pathogenesis of morphea, particularly interferon (IFN) gamma related pathways. Active morphea lesions have been foundto display an inflammatory morphea signature with CXCR3 receptor ligands, as well as a distinct fibrotic signature reflecting fibroblast activation and collagen production. CXCL9 and 10 have been associated with increased measures of disease activity. While immune dysfunction is thought to play the primary role in morphea pathogenesis, there are other factors that may also contribute, including genetic predisposition, environmental factors, and vascular dysregulation. There remains an essential need for further research to elucidate the pathogenesis of morphea and the mode of action of dysregulated upstream and downstream immune and fibrotic pathways. These studies will allow for the discovery of novel biomarkers and targets for therapeutic development.

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At the Leading Edge in Morphea—New Insights Into Disease Course and Management Options

Cruz-Diaz

Haemel

2020

JAMA Dermatol

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Changes in Disease Activity and Damage Over Time in Patients With Morphea

Cited by 25 publications

References 22 publications

Diagnosis and management of morphoea in children: an overview

Diagnosis and management of morphoea in children: an overview

Morphea: progress to date and the road ahead

At the Leading Edge in Morphea—New Insights Into Disease Course and Management Options

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