Clinical features and outcomes of juvenile dermatomyositis and other childhood onset myositis syndromes

Ramanan, A V; Feldman, Brian M.

doi:10.1016/s0889-857x(02)00024-8

Cited by 152 publications

(88 citation statements)

References 53 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The clinical spectrum is very variable, from mild disease that has minimal functional impact to a chronic, severely disabling condition (1)(2)(3)(4). Despite new therapies, juvenile DM remains chronically active in a large proportion of patients.…”

mentioning

confidence: 99%

Extranodal lymphoid microstructures in inflamed muscle and disease severity of new‐onset juvenile dermatomyositis

Padilla¹,

Vallejo²,

Lacomis³

et al. 2009

Arthritis & Rheumatism

View full text Add to dashboard Cite

Objective. Juvenile dermatomyositis (DM) is an autoimmune disease of childhood characterized by lesions in skin and muscle that are populated by plasmacytoid dendritic cells (PDCs) and lymphocyte infiltrates. We undertook this study to examine the cellular composition, organization, and molecular milieu of the cellular infiltrates in muscle in juvenile DM and to correlate the infiltrates with clinical disease manifestations.Methods. Since PDCs and lymphocyte foci express CCL19 and CCL21, we investigated for in situ formation of lymphoid microstructures that could be sites of extranodal immune activation.Results. Analyses of muscle biopsy samples from children with new-onset juvenile DM showed 3 categories of lesions: diffuse infiltrates, lymphocytic aggregates lacking follicle-like organization, and follicle-like structures. The last of these exhibited elements of classic lymphoid follicles, including networks of follicular dendritic cells and high endothelial venules. They also expressed high levels of CXCL13 and lymphotoxins known to support lymphoid organogenesis. There were also resident naive CD45RA؉ T cells and maternally derived B cells and PDCs. Patients with diffuse infiltrates or lymphocytic aggregates were responsive to standard therapy with steroids and methotrexate, but those with follicle-like structures tended to have severe disease that required additional agents such as intravenous Ig or rituximab.Conclusion. These data suggest that lymphoneogenesis is a component of the early disease process in juvenile DM. Ectopic lymphoid structures could indicate a severe course of disease; their early detection could be a tool for disease management.

show abstract

mentioning

confidence: 99%

Extranodal lymphoid microstructures in inflamed muscle and disease severity of new‐onset juvenile dermatomyositis

Padilla¹,

Vallejo²,

Lacomis³

et al. 2009

Arthritis & Rheumatism

View full text Add to dashboard Cite

show abstract

“…MRI is the most common diagnostic modality used in the pediatric population, as it is more likely to show abnormalities consistent with JDM compared to electromyography or muscle biopsy. [1][2][3] Our patient exhibited a rare presentation of JDM, as the predominant clinical feature prompting her to seek medical evaluation was diffuse subcutaneous edema. Although localized edema is common in JDM, generalized edema has been reported rarely in the literature.…”

Section: Discussionmentioning

confidence: 90%

“…JDM can also have gastrointestinal tract, pulmonary, and cardiac involvement. [2][3][4] The average age at onset is 7 years, and JDM has a female predominance of 2:1.…”

Section: Discussionmentioning

confidence: 99%

“…[1][2][3]14 Intravenous gamma globulin use ranges from 17% to 39% of cohorts and is typically used for more severe presentations of JDM or in symptomatic patients refractory to standard treatment. 15 Once the myositis improves and enzyme levels normalize, corticosteroid treatment can be slowly tapered.…”

Section: Discussionmentioning

confidence: 99%

“…15 Once the myositis improves and enzyme levels normalize, corticosteroid treatment can be slowly tapered. 3,14,15 With early initiation and aggressive treatment plans, the prognosis for JDM has significantly improved; the lifetime mortality rate is less than 2% today, as compared to 30% approximately 40 years ago. 6 Nevertheless, mortality still exists, and JDM patients can have significant disability from complications such as calcinosis, lipodystrophy, and severe muscle atrophy.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

A 5-Year-Old Girl with Fatigue, Diffuse Edema, and Weakness

Barsell¹,

Librizzi²

2015

Pediatr Ann

View full text Add to dashboard Cite

A 5-year-old previously healthy girl presented to our hospital with 10 days of periorbital edema, abdominal pain, muscle weakness, and progressive fatigue. Four days prior to admission, her pediatrician prescribed diphenhydramine and fexofenadine for a possible allergic reaction, and polyethylene glycol 3,350 for constipation, but the patient showed no signs of improvement. She became progressively more fatigued, and 1 day prior to admission mild extremity edema was noted. The patient denied fever, vomiting, diarrhea, cough, joint pain or stiffness, recent rash, or trauma. She had visited Mexico 1 month prior. There was no significant family history. Outpatient testing revealed a negative urinalysis.In the emergency department, the patient was noted to be tachycardic (heart rate of 132 beats per minute) but otherwise well-appearing. Physical examination was significant for mild hepatomegaly, mild non-pitting edema of the bilateral upper and lower extremities, mild periorbital edema, and bilateral erythematous patches (2 cm × 2 cm) on her knees (Figure 1). Initial emergency department testing was significant for hypoalbuminemia (2.9 g/dL), transaminitis (aspartate aminotransferase [AST] of 231 units/L, alanine aminotransferase [ALT] of 139 units/L), and a mildly elevated erythrocyte sedimentation rate (24 mm/h).Initial differential diagnosis included autoimmune hepatitis, viral hepatitis, protein-losing enteropathy, and viral myocarditis. Coagulation studies and glucose challenge test were normal (suggesting normal synthetic liver function), hepatitis panel was negative, and the AST to ALT ratio of 231:139 made a primary hepatic etiology less likely. Sustained tachycardia prompted further cardiology testing; however, electrocardiogram and echocardiogram revealed normal cardiac function.Generalized fatigue and subjective muscle weakness remained a prominent symptom, and on further questioning the family then disclosed a history of intermittent facial rash and erythema above her eyelids (Figure 2). Therefore, a systemic inflammatory etiology was explored.

show abstract