Long-term methotrexate efficacy in juvenile localized scleroderma

Zulian, Francesco; Vallongo, Cristina; Vittadello, Fabio; Zanon, G F; Giuliotto, Stephanie; Martini, Giorgia

doi:10.1186/1546-0096-9-s1-o16

Cited by 5 publications

(11 citation statements)

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“…Morphea is usually thought to be a self-limiting disease, but extracutaneous involvement is reported in ∼22% of patients, especially in the linear and generalized subtypes in the series of Zulian et al 4 Extracutaneous involvement was mainly articular 4,5 and presented as oligoarthritis or polyarthritis, related to the skin lesion site in three-quarters of patients. However, the remaining patients had arthritis completely unrelated to the site of the skin lesion, and 30% were positive for rheumatoid factors, raising the suspicion of a systemic, rather than local, pathologic process.…”

Section: Discussionmentioning

confidence: 97%

Fibrous Arthropathy Associated With Morphea: A New Cause of Diffuse Acquired Joint Contractures

et al. 2017

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Etiologies for childhood-onset diffuse joint contractures encompass a large group of inherited disorders and acquired diseases, in particular a subtype of juvenile idiopathic arthritis called "dry polyarthritis," dermatomyositis, and systemic sclerosis. We report on 2 boys, aged 5 and 8 years, who developed acquired symmetric painless joint contractures preceding the development of superficial plaques of morphea by 7 to 13 months. There was no other clinical involvement, biological inflammation, or autoantibodies. No urinary mucopolysaccharidosis was seen. In both patients, wrist MRI showed no joint effusion, no bone erosion, and no or mild synovial thickening with slight enhancement after gadolinium infusion. One patient underwent a synovial biopsy, which showed dense fibrosis with a sparse inflammatory infiltrate, similar to the pathologic pattern observed in the skin biopsy. With methotrexate and systemic steroids, joint contractures slowly improved in the first patient and remained stable in the second. These 2 cases suggest that fibrous synovitis should be considered in children with acquired diffuse, symmetric, painless contractures and without elevation of acute-phase reactants, even in the absence of cutaneous manifestations. Articular MRI with gadolinium and careful cutaneous examination at onset and during follow-up should provide clues for diagnosing this entity.

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Section: Discussionmentioning

confidence: 97%

Fibrous Arthropathy Associated With Morphea: A New Cause of Diffuse Acquired Joint Contractures

et al. 2017

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“…The annual incidence of LS is 1 to 3 per 100,000 children; incidence of SSc is 1 per million children. 5,55,56…”

Section: Sclerodermamentioning

confidence: 99%

“…In SSc, any organ may be affected, in children mainly vascular (Raynaud's phenomenon), cutaneous (skin thickening), gastrointestinal, pulmonary, and musculoskeletal involvement, and rarely cardiac, renal, and neurologic symptoms (►Table 3). 5, [55][56][57] Compared with adult-onset SSc, musculoskeletal involvement is more common in pediatric SSc. Approximately 25 to 40% have inflammatory arthritis (joint effusions), in addition to the typical so-called dry synovitis caused by the fibrosis of tendons traversing the joints that can cause limited range of motion.…”

Section: Systemic Sclerosismentioning

confidence: 99%

“…Acro-osteolysis and subcutaneous calcifications can also occur in a later stage of the disease and are better visualized on plain radiography. 5,55,56 Diagnostic criteria were described for diagnosing SSc with "skin induration or sclerosis proximal to the metacarpal-(MCP) or metatarsophalangeal (MTP) joints" as major criterion; an additional two minor criteria should be present as well (►Table 3). ►Table 3 also lists different organ involvement and possible clinical manifestations in SSc.…”

Section: Systemic Sclerosismentioning

confidence: 99%

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MR Imaging of Rheumatic Diseases Affecting the Pediatric Population

Schiettecatte

Jans

Jaremko

et al. 2021

Semin Musculoskelet Radiol

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This article reviews the application of magnetic resonance imaging (MRI) to pediatric rheumatic diseases. MRI can detect early manifestations of arthritis, evaluate the extent of disease, and monitor disease activity and response to treatment.Juvenile idiopathic arthritis (JIA) is the most common pediatric rheumatic disorder, representing a diverse group of related diseases that share a definition of joint inflammation of unknown origin with onset before 16 years of age and lasting > 6 weeks. JIA may lead to significant functional impairment and is increasingly imaged with MRI to assess for active inflammation as a target for therapy. This is particularly true for juvenile spondyloarthritis that includes multiple subgroups of JIA and primarily involves the spine and sacroiliac joints.Other less common pediatric rheumatic diseases considered here are chronic recurrent multifocal osteomyelitis and collagen vascular diseases including polymyositis, dermatomyositis, scleroderma, and juvenile systemic lupus erythematosus.

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“…Localized scleroderma, also known as morphea, primarily affects the skin on the trunk, limbs, and head, but can also involve the underlying organs, as well as joints. 65 Systemic scleroderma causes symmetric skin changes and fibrous changes in other organs, including cardiac, gastrointestinal, renal, and pulmonary systems. Raynaud's phenomenon, telangiectasias, calcinosis, arthritis/arthralgias, myositis/myalgias, dysphagia, and dyspnea are also commonly seen in patients with systemic scleroderma.…”

Section: Sclerodermamentioning

confidence: 99%

Central Nervous System Involvement in Pediatric Systemic Autoimmune Diseases

Stanton

Knight

2017

J Pediatr Neurol

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Central nervous system (CNS) involvement occurs in many pediatric systemic autoimmune diseases. These manifestations are often associated with increased morbidity and mortality and can have adverse long-term effects on neurodevelopmental, health-related, and quality-of-life outcomes. In this review, we summarize the existing literature on CNS involvement in pediatric systemic autoimmune diseases, compare diagnostic findings, and describe current treatments. CNS manifestations of pediatric systemic autoimmune disease present diagnostic and management challenges due to their relatively rare occurrence in children, the difficulty in confirming direct CNS involvement as opposed to secondary symptoms occurring as a consequence of systemic inflammation, and the need to define treatment strategies effective for CNS involvement.

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Long-term methotrexate efficacy in juvenile localized scleroderma

Abstract: Recent studies reported that methotrexate (MTX), appears beneficial in juvenile localized scleroderma (JLS) but little is known about its long-term efficacy. We assessed the long-term efficacy of MTX in a cohort of patients with JLS.

Cited by 5 publications

References 0 publications

Fibrous Arthropathy Associated With Morphea: A New Cause of Diffuse Acquired Joint Contractures

Fibrous Arthropathy Associated With Morphea: A New Cause of Diffuse Acquired Joint Contractures

MR Imaging of Rheumatic Diseases Affecting the Pediatric Population

Central Nervous System Involvement in Pediatric Systemic Autoimmune Diseases

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