Treatment Approaches to Juvenile Dermatomyositis (JDM) Across North America: The Childhood Arthritis and Rheumatology Research Alliance (CARRA) JDM Treatment Survey

Stringer, Elizabeth; Bohnsack, John F.; Bowyer, Suzanne L.; Griffin, Thomas A.; Huber, Adam M.; Lang, Bianca; Lindsley, Carol B.; Ota, Sylvia; Pilkington, Clarissa; Reed, Ann M.; Scuccimarri, Rosie; Feldman, Brian M.

doi:10.3899/jrheum.090953

Cited by 93 publications

(81 citation statements)

References 35 publications

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“…6 Nevertheless, mortality still exists, and JDM patients can have significant disability from complications such as calcinosis, lipodystrophy, and severe muscle atrophy. 15 Even with an aggressive treatment plan, more than half of JDM patients have continuous …”

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. 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.…”

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%

“…15,16 JDM has a largely unpredictable course, as one-third of patients recover spontaneously, one-third develop moderate to severe disability, and the remaining one-third die from the illness. [4][5][6] This case highlights a unique presentation of JDM with fatigue and diffuse edema as the prominent features rather than the classic skin manifestations.…”

Section: Continued From Page 471mentioning

confidence: 99%

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

Barsell¹,

Librizzi²

2015

Pediatr Ann

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Continued From Page 471mentioning

confidence: 99%

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

Barsell¹,

Librizzi²

2015

Pediatr Ann

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“…Intravenous immunoglobulin (IVIG) is commonly used for the treatment of a wide range of autoimmune diseases 1,2,3 . The available data also suggest that IVIG has a steroid-sparing effect: either enhancing the steroid sensitivity or reducing the steroid dose 4,5,6 .…”

Section: To the Editormentioning

confidence: 99%

Enhancement of the Affinity of Glucocorticoid Receptors as a Mechanism Underlying the Steroid-sparing Effect of Intravenous Immunoglobulin: Figure 1.

Pashov¹,

Delignat²,

Bayry³

et al. 2011

J Rheumatol

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Protocols for the initial treatment of moderately severe juvenile dermatomyositis: Results of a Children's Arthritis and Rheumatology Research Alliance Consensus Conference

Huber¹,

Giannini²,

Bowyer³

et al. 2010

Arthritis Care & Research

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109

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Objective. To use juvenile dermatomyositis (DM) survey data and expert opinion to develop a small number of consensus treatment protocols, which reflect current initial treatment of moderately severe juvenile DM. Methods. A consensus meeting was held in Toronto, Ontario, Canada on December 1-2, 2007. Nominal group technique was used to achieve consensus on treatment protocols, which represented typical management of moderately severe juvenile DM. Consensus was also reached as to which patients these protocols would be applicable (inclusion and exclusion criteria), which initial investigations should be done prior to initiating one of these protocols, which data should be collected to evaluate these protocols, and the concomitant interventions required or recommended. Results. Three protocols that described the first 2 months of treatment were developed. All protocols included corticosteroids and methotrexate. One protocol also included intravenous gamma globulin. Consensus was achieved for all issues that were addressed by conference participants, although there were some areas of controversy. Conclusion. Despite considerable variation in clinical practice, it is possible to achieve consensus on the initial treatment of juvenile DM. Once these protocols are extended beyond 2 months, these protocols will be available for clinical use. By using methods that account for differences between patients (confounding by indication), the comparative effectiveness of the protocols will be evaluated. In the future, the goal will be to identify the optimal treatment of moderately severe juvenile DM.

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Treatment Approaches to Juvenile Dermatomyositis (JDM) Across North America: The Childhood Arthritis and Rheumatology Research Alliance (CARRA) JDM Treatment Survey

Abstract: For the majority of North American pediatric rheumatologists, corticosteroids and MTX appear to be the standard of care for typical cases of JDM. There is variability, however, in the route of administration of corticosteroids and use of IVIG and hydroxychloroquine.

Cited by 93 publications

References 35 publications

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

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

Enhancement of the Affinity of Glucocorticoid Receptors as a Mechanism Underlying the Steroid-sparing Effect of Intravenous Immunoglobulin: Figure 1.

Protocols for the initial treatment of moderately severe juvenile dermatomyositis: Results of a Children's Arthritis and Rheumatology Research Alliance Consensus Conference

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