Juvenile Dermatomyositis: New Clues to Diagnosis and Therapy

Pachman, Lauren M; Nolan, Brian E.; DeRanieri, Deidre; Khojah, Amer

doi:10.1007/s40674-020-00168-5

Cited by 23 publications

(27 citation statements)

References 155 publications

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“…Beim dermatopulmonalen Syndrom handelt es sich um eine seltene Form einer Dermatomyositis. Die bisher in der Literatur beschriebenen Fälle liegen größtenteils von adulten Patienten vor, doch gibt es Fallberichte auch bei Kindern und Jugendlichen 1 . Das dermatopulmonale Syndrom wird auch „clinically amyopathic dermatomyositis“ (CADM) genannt und wurde 1979 erstmals von Pearson beschrieben 2 .…”

Section: Diskussionunclassified

“…Charakteristisch ist der positive Nachweis von anti-MDA5 (Melanom-assoziiertes Differenzierungsgen-5)-Antikörpern. Die Häufigkeit des Nachweises liegt bei 13–35 % bei der adulten Dermatomyositis sowie 7 % bei der juvenilen Dermatomyositis 1 . Das Krankheitsbild zeichnet sich durch eine milde Myositis, Arthritis, mukokutane Ulzerationen sowie eine interstitielle Lungenerkrankung aus, die vor allem bei Patienten ostasiatischen Ursprungs einen rapid progressiven Verlauf nehmen kann 4 .…”

Section: Diskussionunclassified

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Seronegative polyartikuläre juvenile idiopathische Arthritis mit Lungenbeteiligung – oder wohl doch nicht?

Dressler¹,

Price²,

Meyer‐Bahlburg³

2021

Arthritis und Rheuma

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ZusammenfassungEs wird ein Patient vorgestellt, der im Alter von 9 Jahren eine polyartikuläre Arthritis entwickelte, die auf eine Therapie mit Methotrexat zunächst ansprach. Aufgrund deutlicher Transaminasenerhöhungen wurde die Methotrexat-Therapie nach 4 Monaten beendet und Etanercept gegeben. Darunter entwickelte der Patient eine Pneumonitis und es kam es zur Entwicklung eines systemischen Lupus erythematodes, der nach Absetzen des Etanercept wieder verschwand. Wir vermuteten dann eine Mischkollagenose und therapierten mit Steroiden und Mycophenolat-Mofetil sowie später mit Tocilizumab. Im Verlauf kam es zur Entwicklung einer Kalzinose und erst auswärts nach Nachweis von MDA-5-Antikörpern zur Diagnose eines dermatopulmonalen Syndroms und einer weitgehend amyopathischen juvenilen Dermatomyositis. Unter einer Therapie mit Cyclosporin A persistieren Bewegungseinschränkungen an der Schulter, ansonsten ist der Patient weitgehend beschwerdefrei, treibt Sport und wird von Erwachsenenrheumatologen weiter betreut.

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Section: Diskussionunclassified

Seronegative polyartikuläre juvenile idiopathische Arthritis mit Lungenbeteiligung – oder wohl doch nicht?

Dressler¹,

Price²,

Meyer‐Bahlburg³

2021

Arthritis und Rheuma

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“…However, in 2017, EULAR and ACR proposed new diagnostic criteria which refined the scoring for each criterion and added esophageal dysmotility and anti-histidyl-tRNA synthetase antibody, leading to improvement in the specificity over the original Bohan and Peter criteria [ 12 ]. Others had suggested including MRI findings, nailfold capillary changes, and other myositis-specific antibodies (MSA) to the criteria to further improve the diagnostic yield and minimize the need for muscle biopsy in the often-young child [ 13 ].…”

Section: Introductionmentioning

confidence: 99%

Clues to Disease Activity in Juvenile Dermatomyositis: Neopterin and Other Biomarkers

2021

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Easily accessible biomarkers are urgently needed to evaluate immune activation in Juvenile Dermatomyositis (JDM). The goal of this retrospective study is to define immunological and clinical differences between untreated JDM patients with either normal or elevated (>10 mmol/L) levels of neopterin, a biomarker of macrophage activation. We included all JDM with neopterin data obtained before initiating medical therapy. We assessed T, B, NK cell populations, muscle enzymes, and disease activity scores for skin (sDAS), muscle (mDAS), total (tDAS), the duration of untreated disease, disease course, and myositis-specific antibody (MSA). Seventy-nine percent of 139 untreated JDM patients had elevated serum neopterin. The group with elevated neopterin had significantly more active disease: tDAS 11.9 vs. 8.1 (p < 0.0001), mDAS 5.8 vs. 3.1 (p < 0.0001), sDAS 6.1 vs. 4.9 (p = 0.0002), aldolase 24.0 vs. 7.6 U/L (p < 0.0001), von Willebrand factor antigen (p < 0.0001), and ESR 19.8 vs. 11.5 mm/hr (p = 0.01). The flow cytometry documented both reduced T cells (1494 vs. 2278/mm3, p = 0.008) and NK cells (145 vs. 240/mm3, p = 0.003). TNFα-308AA/AG polymorphism was more common in children with elevated neopterin than TNFα-308GG (p 0.05). We conclude that the availability of neopterin data will contribute to the rapid assessment of untreated JDM disease activity.

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“…Juvenile Dermatomyositis (JDM) is a multisystem pediatric disease characterized by chronic in ammation of muscle and skin (1). Despite the recent advances in the treatment of JDM, high dose corticosteroid remains the primary therapy to induce remission (2).…”

Section: Introductionmentioning

confidence: 99%

“…Despite the recent advances in the treatment of JDM, high dose corticosteroid remains the primary therapy to induce remission (2). Typically, JDM patients need either intravenous or oral corticosteroid therapy for around two years (1). Although previous studies showed that untreated JDM subjects have lower weight and height than age and gender-matched controls, (3) weight gain and cushingoid features are among the most common side effects observed in children after glucocorticoid initiation in various clinical trials (4).…”

Section: Introductionmentioning

confidence: 99%

Changes in Total Body Fat and Body Mass Index among Children with Juvenile Dermatomyositis Treated with High-dose Glucocorticoids

Khojah

Liu

Morgan

et al. 2021

Preprint

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Objective High-dose glucocorticoids (GC) remain the primary therapy to induce remission in Juvenile Dermatomyositis (JDM). Studies of the natural history of GC associated weight gain in children are very limited, especially in the JDM population. This study aims to measure BMI changes in a cohort of JDM subjects over 60 months and to examine the changes in body composition by DXA. Methods We included all subjects with JDM who had five years of follow up data and serial DXA. BMI and total body fat (TBF) percentiles were calculated based on the CDC published percentile charts. To study the natural history of weight gain and TBF, we assessed the data at four-time points (T0 = baseline, T1 > 1.5 years, T2= 1.51-3.49 years, T3 = 3.5-5 years). Results 96 subjects (78% female, 70% white) were included. Paired T-test showed a significant increase in the mean BMI percentile by 17.5 points (P=0.004) after the initiation of medical treatment, followed by a gradual decrease over the study period. However, the TBF percentile did not change over the study period. TBF in the last visit had a strong correlation with T1 BMI (P <0.001) and muscle weakness Disease Activity Score (P=0.002) Conclusions Although the BMI percentile decreased throughout the study, the TBF percentile remained high until the end of the study (60 months). This finding raises the concern that some of the reduction in the BMI percentile could reflect a drop in the lean body mass from muscle wasting rather than actual fat loss.

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Juvenile Dermatomyositis: New Clues to Diagnosis and Therapy

Cited by 23 publications

References 155 publications

Seronegative polyartikuläre juvenile idiopathische Arthritis mit Lungenbeteiligung – oder wohl doch nicht?

Seronegative polyartikuläre juvenile idiopathische Arthritis mit Lungenbeteiligung – oder wohl doch nicht?

Clues to Disease Activity in Juvenile Dermatomyositis: Neopterin and Other Biomarkers

Changes in Total Body Fat and Body Mass Index among Children with Juvenile Dermatomyositis Treated with High-dose Glucocorticoids

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