Session Information
Date: Sunday, November 8, 2015
Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects Poster I: Lupus, Scleroderma, JDMS
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose: Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myositis in children. It is a heterogeneous disease and clinical manifestations range from a relatively mild disease, to that causing significant morbidity and mortality. Especially, JDM that associated interstitial lung disease (ILD) may vary from asymptomatic to severe, rapidly leading causes of fatal outcome. The aim of this study is to determine the clinical and laboratory features of JDM complicated with ILD.
Methods: During March 1984 and May 2015, we retrospectively reviewed the clinical and laboratory features of twenty nine patients who were diagnosed as definite and probable JDM.
Results: Eight cases (27.6%) were complicated with ILD. The mean age was 6.3 years (2-13 years), and 75% of the patients were female. Cutaneous manifestations including Gottron’s rash in 6, malar rash in 3, heliotrope rash in 1, skin ulcers in 2 each. The common systemic manifestations were myalgias in 5, arthralgia in 7, fever 6. Five cases had proximal muscle weakness. Anti-Jo-1 antibodies was positive in 2, anti-MDA-5 in 2. The testing for serum KL-6 was elevated in all cases. Respiratory symptoms were initially noticed in three cases. These two of three cases that were found to contain anti-MDA5 antibodies had rapid-progressive ILD (RPILD). One patient with anti-Jo-1 antibodies had ILD and polyarthritis was diagnosed as antisynthetase syndrome. All 8 patients were treated with glucocorticoids; 4 required pulse steroids therapy, 6 required immunosuppressive agents (methotrexate, Cyclosporin A, intravenous cyclophosphamide, mycophenolate mofetil (MMF),
intravenous immunoglobulin, Adalimumab. 3 patients with RPILD died of respiratory failure. Five (62.5%) patients showed no featured of myositis after a follow up of 3 years to 12 years. 5 patients had a normal CK levels.
Conclusion:
ILD is one of the most serious complications with JDM, especially positive for anti-MDA5 antibodies.
Table
|
Case1 |
Case2 |
Case3 |
Case4 |
Case5 |
Case6 |
Case7 |
Case8 |
|
|
Sex |
Female |
Male |
Female |
Female |
Female |
Female |
Female |
Male |
|
Age at onset of JDM (yr) |
3 |
8 |
10 |
5 |
2 |
2 |
13 |
7 |
|
CK(IU/l) |
916 |
148 |
1374 |
32 |
90 |
90 |
257 |
194 |
|
Aldolase (IU/l) |
50.4 |
18.1 |
82.6 |
9.9 |
16.4 |
15.7 |
17.6 |
16.1 |
|
Antinuclear antibody |
1:2560 |
negative |
1:1280 |
1:2560 |
1:40 |
1:40 |
negative |
1:80 |
|
Other Ab |
dsDNA |
Jo-1, RF |
non |
Jo-1 |
MDA5, RF |
MDA5, RF |
TPO |
non |
|
KL-6 |
1720 |
454 |
975 |
686 |
1290 |
904 |
376 |
2950 |
|
Treatment |
PSL, CyA, MMF |
PSL, CyA, MMF, anti-TNF |
PSL |
mPSL, PSL,CyA, MMF |
mPSL, PSL,CyA IVCY, IVIG |
mPSL, PSL,CyA IVCY, |
PSL,CyA MTX |
mPSL, PSL |
To cite this abstract in AMA style:
Sato S, Uejima Y, Suganuma E, Takano T, Kawano Y. Clinical Analysis and Outcome of Interstitial Lung Disease with Juvenile Dermatomyositis [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/clinical-analysis-and-outcome-of-interstitial-lung-disease-with-juvenile-dermatomyositis/. Accessed .« Back to 2015 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-analysis-and-outcome-of-interstitial-lung-disease-with-juvenile-dermatomyositis/