Clinical features and disease course of patients with juvenile dermatomyositis

Gowdie, Peter; Allen, Roger C.; Kornberg, Andrew J.; Akikusa, Jonathan

doi:10.1111/1756-185x.12107

Cited by 62 publications

(61 citation statements)

References 19 publications

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“…Nine muscle MRIs were done as part of the diagnostic evaluation of children; all in the latter half of the period under review. Thus MRI seems to have gained prominence as a diagnostic tool for JDM consistent with findings from other studies [19, 26, 27]. However, in Africa and many other resource constrained economies, MRI may not be available in many centres and even where it is available, long waiting times, technical issues with reporting and cost implications may prove prohibitive.…”

Section: Discussionsupporting

confidence: 74%

Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study

et al. 2016

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BackgroundJuvenile dermatomyositis (JDM) is a rare idiopathic inflammatory childhood myopathy of uncertain aetiology. The demographic and clinical presentation of JDM may differ by race and geographic regions. Few studies have described the characteristics of JDM patients from Africa.MethodsWe conducted a retrospective observational study to determine clinical characteristics and outcomes of patients satisfying the Bohan and Peter criteria for probable JDM seen between 2004 and 2013 in three hospitals in Cape Town, South Africa.ResultsTwenty five cases were identified: 16 female and 9 male; thirteen (52 %) were of indigenous African, eleven (44 %) mixed and one (4 %) European ancestry. The median ages at disease onset and diagnosis were 6.75 (range 2.0–9.7) and 7.9 (range 3.4–9.75) years respectively. Eleven patients had calcinosis while the mortality was 2/25 (8 %). Only 40 % of the patients had clinically inactive disease by PRINTO criteria (modified) at last review. There was no statistically significant difference in racial distribution (p-value = 1), age at disease onset (p-value = 0.87) and disease duration prior to treatment initiation (p-value = 0.75) between patients who had clinically active and inactive disease.ConclusionThe demographic characteristics of children with JDM were similar to that from most other regions of the world with female predominance and similar age at onset. Majority of the patients remained with clinically active disease, which put them at risk of further disease complications. Long term follow up and use of appropriate treatment guidelines may be indicated in management of JDM patients for optimum treatment outcomes.

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Section: Discussionsupporting

confidence: 74%

Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study

et al. 2016

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“…In many situations, MRIs have decreased the need for invasive procedures such as EMG and muscle biopsy [12, 13]. In our short report the muscle MRI was used in 89% at the time of diagnosis, while EMG and muscle biopsy were used in 17 and 24%, respectively.…”

Section: Discussionmentioning

confidence: 99%

Muscle MRI at the time of questionable disease flares in Juvenile Dermatomyositis (JDM)

Abdul-Aziz

Adler

et al. 2017

Pediatr Rheumatol

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BackgroundThe course of JDM has improved substantially over the last 70 years with early and aggressive treatments. Yet it remains difficult to detect disease flares as symptoms may be mild; signs of rash and muscle weakness vary widely and are often equivocal; laboratory tests of muscle enzyme levels are often normal; electromyography and muscle biopsy are invasive. Alternative tools are needed to help decide if more aggressive treatment is needed. Our objective is to determine the effectiveness of muscle Magnetic Resonance Imaging (MRI) in detecting JDM flares, and how an MRI affects physician’s decision-making regarding treatment.MethodsThis study was approved by the Institutional Review Board of Nationwide Children’s Hospital. JDM patients were consulted between 1/2005 and 6/2015. MRIs were performed on both lower extremities without contrast sequentially: axial T1, axial T2 fat saturation, axial and coronal inversion recovery, and axial diffusion weighted. The physician decision that a JDM patient was in a flare was considered the gold standard. MRI results were compared with physician’s decisions on whether a relapse had occurred, and if there was a concordance between the assessment methods.ResultsForty-five JDM patients were studied. Eighty percent had weakness at diagnosis, 100% typical rash, and 73% typical nail-fold capillary changes. At diagnosis, muscle enzymes were compatible with JDM generally (CK 52%, LDH 62%, aldolase 72%, AST 54% abnormal). EMG was abnormal in 3/8, muscle biopsy typical of JDM in 10/11, and MRI abnormal demonstrating myositis in 31/40. Thirteen patients had a repeat MRI for possible flares with differing indications. Three repeat MRI’s were abnormal, demonstrating myositis. There was moderate agreement about flares between MRI findings and physician’s treatment decisions (kappa = 0.59). In each abnormal MRI case the physician decided to increase treatment (100% probability for flares). MRI was negative for myositis in 10 patients, by which 7/10 the physicians chose to continue or to taper the medications (70% probability for non-flares).ConclusionA muscle MRI would facilitate objective assessments of JDM flares. When an MRI shows myositis, physicians tend to treat 100% of the time. When an MRI shows no myositis, physicians continued the same medications or tapered medications 70% of the time. Further studies would help confirm the utility and cost-effectiveness of MRI to determine JDM flares.Electronic supplementary materialThe online version of this article (doi:10.1186/s12969-017-0154-4) contains supplementary material, which is available to authorized users.

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“…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%

“…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. It should be noted that generalized subcutaneous edema might be the first presenting feature of JDM and may indicate a more rapid onset and more aggressive disease course.…”

Section: Continued From Page 471mentioning

confidence: 91%

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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Clinical features and disease course of patients with juvenile dermatomyositis

Cited by 62 publications

References 19 publications

Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study

Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study

Muscle MRI at the time of questionable disease flares in Juvenile Dermatomyositis (JDM)

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

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