A long-term follow-up study of methotrexate in juvenile localized scleroderma (morphea)

Zulian, Francesco; Vallongo, Cristina; Patrizi, Annalisa; Fortina, Anna Belloni; Cutrone, Mario; Alessio, Maria; Martino, Silvana; Gerloni, Valeria; Vittadello, Fabio; Martini, Giorgia

doi:10.1016/j.jaad.2012.03.036

Cited by 114 publications

(98 citation statements)

References 13 publications

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“…According to literature, MTX has been proved to be an effective and well-tolerated treatment for achieving disease remission in pediatric patients with LS 15,18–20. However, few studies have been conducted in adult population.…”

Section: Discussionmentioning

confidence: 99%

Methotrexate: an effective monotherapy for refractory generalized morphea

Platsidaki

Tzanetakou

Kouris

et al. 2017

CCID

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IntroductionMorphea is an inflammatory skin disorder characterized by excessive collagen deposition. Although treatment algorithms for morphea subtypes have been suggested, no consistent recommendations are available. This study attempts to evaluate the clinical efficacy of methotrexate (MTX) as monotherapy in refractory generalized morphea.MethodsIt is a retrospective study, including 20 patients who had already been treated with various topical and systemic therapies with minimal clinical improvement. Patients received orally MTX at a of dosage 15 mg once weekly. Duration of the use, dosage of MTX, and adverse events were recorded. Clinical assessment of skin lesions was performed and documented.ResultsThe mean disease duration was 27 months before the initiation of MTX treatment. After 12 months of therapy, very good response was achieved in 6 patients (30%), good response in 10 patients (50%), and fair response in 2 patients (10%), while 2 patients (10%) had failed treatment. Patients were followed up for a mean time interval of 21 months. No serious adverse event was recorded.ConclusionMTX has been already proved to be an effective and well-tolerated treatment in pediatric patients with morphea. The majority of the group of adult patients showed very good and good improvement when treated with MTX. Although this is an uncontrolled study, MTX monotherapy was considered a safe and effective treatment for the management of this specific clinical subset of morphea in adults.

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Section: Discussionmentioning

confidence: 99%

Methotrexate: an effective monotherapy for refractory generalized morphea

Platsidaki

Tzanetakou

Kouris

et al. 2017

CCID

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“…In contrast to the high short-term response rates, (long-term) disease recurrences are reported in 28–44% of the patients after MTX discontinuation [1, 25, 182, 184]. A prospective long-term follow-up study described that MTX treatment duration was a predictor for relapse, suggesting that longer treatment duration prevents disease relapse [186]. Lastly, some of the retrospective studies report superior response rates for combination therapy with MTX plus SCS compared with MTX monotherapy [4, 185].…”

Section: Treatmentmentioning

confidence: 99%

Morphea and Eosinophilic Fasciitis: An Update

et al. 2017

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Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. The exact driving mechanisms behind morphea and EF pathogenesis remain to be elucidated. However, extensive extracellular matrix formation and autoimmune dysfunction are thought to be key pathogenic processes. Likewise, these processes are considered essential in systemic sclerosis (SSc) pathogenesis. In addition, similarities in clinical presentation between morphea and SSc have led to many theories about their relatedness. Importantly, morphea may be differentiated from SSc based on absence of sclerodactyly, Raynaud’s phenomenon, and nailfold capillary changes. The diagnosis of morphea is often based on characteristic clinical findings. Histopathological evaluation of skin biopsies and laboratory tests are not necessary in the majority of morphea cases. However, full-thickness skin biopsies, containing fascia and muscle tissue, are required for the diagnosis of EF. Monitoring of disease activity and damage, especially of subcutaneous involvement, is one of the most challenging aspects of morphea care. Therefore, data harmonization is crucial for optimizing standard care and for comparability of study results. Recently, the localized scleroderma cutaneous assessment tool (LoSCAT) has been developed and validated for morphea. The LoSCAT is currently the most widely reported outcome measure for morphea. Care providers should take disease subtype, degree of activity, depth of involvement, and quality-of-life impairments into account when initiating treatment. In most patients with circumscribed superficial subtypes, treatment with topical therapies suffices. In more widespread disease, UVA1 phototherapy or systemic treatment with methotrexate (MTX), with or without a systemic corticosteroid combination, should be initiated. Disappointingly, few alternatives for MTX have been described and additional research is still needed to optimize treatment for these debilitating conditions. In this review, we present a state-of-the-art flow chart that guides care providers in the treatment of morphea and EF.

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“…In general, patients with active deep linear morphea, active deep mixed variant morphea, active deep generalized morphea and active pansclerotic morphea should be treated with systemic steroids (a 3 month taper) and methotrexate (ideally for a duration of 2 years) in an attempt to prevent new lesion formation and functional impairment (22–25). Patients with active superficial circumscribed morphea may be treated with topical therapy or localized phototherapy (24,25).…”

Section: Partmentioning

confidence: 99%