An international survey of developing classification criteria for juvenile dermatomyositis-scleroderma overlap

Khaosut, Parichat; Pilkington, Clarissa; Wedderburn, Lucy R.; Compeyrot-Lacassagne, Sandrine

doi:10.1093/rheumatology/kez226

Cited by 4 publications

(3 citation statements)

References 7 publications

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“…Anti-topoi-somerase I antibody was not positive in any of the 18 patients in whom this was tested. It may be important to carefully look at clinical features of other overlapping diseases with JSSc as this has been found to impact the therapeutic decisions (35). Two patients in the present series had abnormal PFT (moderate restriction) but CT chest showed no abnormalities.…”

Section: Discussionmentioning

confidence: 76%

Clinical profile, long-term follow-up and outcome of juvenile systemic scleroderma: 25 years of clinical experience from North-West India

Jindal

Patra

Guleria

et al. 2021

Clinical and Experimental Rheumatology

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Objective. To describe the clinical profile, long-term follow-up and outcome of juvenile systemic scleroderma (JSSc) from a tertiary care referral hospital in North-West India. Methods. A review of case records was performed and children with JSSc (disease onset <14 years of age) were analysed. Diagnosis was based on the Paediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for JSSc. Results. Forty patients (28 girls and 12 boys; F:M ratio= 2.3:1) were diagnosed with JSSc (including 22 children with overlap) in the last 25 years. Mean age at symptom onset was 7.75±3.19 years with a mean delay in diagnosis of 2.275±2.09 years. Raynaud's phenomenon was seen in 26/40 (65%) patients at presentation. Lung involvement was noted in 40% patients. Methotrexatewas the most commonly used therapy, followed by oral prednisolone. Patients without overlap had higher incidence of cutaneous ulcers as compared to patients with overlap (55% vs. 18%; p-value: 0.01). Patients with overlap required significantly higher oral prednisolone (81% vs. 22%), methotrexate (72% vs. 38%) and hydroxychloroquine (54% vs. 5%) while cyclophosphamide (13% vs. 44%) and azathioprine (9% vs. 44%) were used relatively less in this group. Mortality was 15% at a mean follow-up of 51.75 months. Infections were noted to be the most common cause of death. There was no significant difference in the mortality between patients with and without lung disease or patients with or without overlap. Conclusion.We describe the largest single-centre cohort with longest follow-up of juvenile systemic scleroderma from India.

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Section: Discussionmentioning

confidence: 76%

Clinical profile, long-term follow-up and outcome of juvenile systemic scleroderma: 25 years of clinical experience from North-West India

Jindal

Patra

Guleria

et al. 2021

Clinical and Experimental Rheumatology

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“…29 As for the low -Scl70 prevalence is common in patients with Myo-SSc seen in previous seroprevalence surveys. [28][29][30] Thus, it seems to be the serological behavior of this entity, although more robust studies are needed.…”

Section: Discussionmentioning

confidence: 99%

Patients with inflammatory myopathies overlapping with systemic sclerosis: A Brazilian-Japanese bicentric study

et al. 2022

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Objectives: This study aims to describe and compare the demographic, clinical, and laboratory characteristics and follow-up of representative samples of patients with myopathies and systemic sclerosis overlap syndromes (Myo-SSc) from two tertiary centers. Patients and methods: This is a cross-sectional and retrospective study conducted between January 2000 and December 2020. Fourty-five patients were analyzed with Myo-SSc (6 males, 39 females; mean age: 50.2±15.4 years; range, 45 to 65 years) from two tertiary centers (n=30 from Brazil and n=15 from Japan). Results: The median follow-up was 98 (range, 37 to 168) months. Muscle impairment started simultaneously with the diagnosis of systemic sclerosis in 57.8% (26/45) of cases. Muscle involvement occurred before the onset of systemic sclerosis in 35.5% (16/45) of cases, and after in 6.7% (3/45). Polymyositis was observed in 55.6% (25/45) of cases, followed by dermatomyositis in 24.4% (11/45) and antisynthetase syndrome in 20.0% (9/45). Concerning systemic sclerosis, the diffuse and limited forms occurred in 64.4% (29/45) and 35.6% (16/45) of the cases, respectively. Comparing the subgroups, Myo or SSc onset was earlier in Brazilian patients, and they had a higher frequency of dysphagia (20/45, [66.7%]) and digital ulcers (27/45, [90%]), whereas Japanese patients had higher modified Rodnan skin scores (15 [9 to 23]) and prevalence of positive anti-centromere antibodies (4/15 [23.7%]). The current disease status and mortality were similar in both groups. Conclusion: In the present study, Myo-SSc affected middle-aged women, and its manifestation spectrum varied according to geographic distribution.

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“…Currently, no unifying internationally accepted definition of overlap myositis exists as different CTDs can have similar clinical features. An international survey of clinical opinion on criteria for JDM–scleroderma overlap, which occurs in 15–20% of patients with JDM according to some reports 71 , proposed the use of the presence of two or more of the following criteria: Raynaud phenomenon, sclerodactyly and sclerodermatous skin changes in a child fulfilling criteria for JDM 72 . In a large US study of 1,718 patients with systemic lupus erythematosus (SLE) (451 paediatric and 1,267 adult patients), 6.3% of the patients had concurrent myositis 73 , whereas in a UK cohort of patients with JIIM, 2.5% of the patients were given a diagnosis of JDM–SLE overlap 15 .…”

Section: Clinical Phenotypesmentioning

confidence: 99%

Juvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care

et al. 2023

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The childhood-onset or juvenile idiopathic inflammatory myopathies (JIIMs) are a heterogenous group of rare and serious autoimmune diseases of children and young people that predominantly affect the muscles and skin but can also involve other organs, including the lungs, gut, joints, heart and central nervous system. Different myositis-specific autoantibodies have been identified that are associated with different muscle biopsy features, as well as with different clinical characteristics, prognoses and treatment responses. Thus, myositis-specific autoantibodies can be used to subset JIIMs into sub-phenotypes; some of these sub-phenotypes parallel disease seen in adults, whereas others are distinct from adult-onset idiopathic inflammatory myopathies. Although treatments and management have much improved over the past decade, evidence is still lacking for many of the current treatments and few validated prognostic biomarkers are available with which to predict response to treatment, comorbidities (such as calcinosis) or outcome. Emerging data on the pathogenesis of the JIIMs are leading to proposals for new trials and tools for monitoring disease.

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An international survey of developing classification criteria for juvenile dermatomyositis-scleroderma overlap

Cited by 4 publications

References 7 publications

Clinical profile, long-term follow-up and outcome of juvenile systemic scleroderma: 25 years of clinical experience from North-West India

Clinical profile, long-term follow-up and outcome of juvenile systemic scleroderma: 25 years of clinical experience from North-West India

Patients with inflammatory myopathies overlapping with systemic sclerosis: A Brazilian-Japanese bicentric study

Juvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care

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