Beguin, V

Bodemer, C

de Blic, J

Fabi, M

Le Bourgeois, M

Prieur, AM

Quartier, P

English

PubMed

M Fabi

M Le Bourgeois

C Bodemer

V Beguin

AM Prieur

P Quartier

J de Blic

Springer - Publisher Connector

BioMed Central
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Pediatric Rheumatology
Open AccessPoster presentation
Respiratory involvement in juvenile dermatomyositis
M Fabi*1, M Le Bourgeois2, C Bodemer3, V Beguin2, AM Prieur4, P Quartier4 
and J de Blic2
Address: 1Pediatric Unit, Ospedale S.Orsola-Malpighi, Bologna, Italy, 2Service de Pneumologie et Allergologie Pédiatriques, Hôpital Necker- 
Enfants Malades, Paris, France, 3Service de Dermatologie Pédiatrique, Hôpital Necker- Enfants Malades, Paris, France and 4Service de 
Rheumatologie Pédiatriques, Hôpital Necker- Enfants Malades, Paris, France
* Corresponding author    
Background
Juvenile dermatomyositis (JDM) is a rare chronic idio-
pathic inflammatory disorder primarily affecting the stri-
ated muscle and the skin. Pulmonary involvement is a
common complication and cause of morbidity and mor-
tality, but few data are available concerning pulmonary
function impairment in childhood.
The aim of this prospective study was to assess pulmonary
function impairment in JDM.
Materials and methods
16 patients (9 girls) with diagnosis of JDM (age 3–16,2
yrs) performed pulmonary function tests (PFT). 14 were
receiving treatment; all had muscular testing. 13 had
weakness as major symptom. Respiratory symptoms: dry
cough in 2/16; reduced exercise tolerance: 3/16. Chest x-
ray: normal in 13/13. Echocardiogram and ECG: normal
in all. 9/16 had positive ANA-Ab; antiJO1 Ab negative in
all.
Results
7/15 pt (46%): restrictive syndrome measured on TLC
and VC; 9/16 (56%): reduced FRC; 9/13 pt: reduced RV.
DLCO: impaired in 2 with restrictive pattern. No patient
had bronchial obstruction. We didn't find a relation
between PFT and steroid treatment/impregnation, nor
with relapses, nor with muscle enzymes nor with ANA/
FAN. Muscular testing didn't always correlate with respira-
tory impairment.
Conclusion
Lung involvement is frequent in JDM and is better
detected by PFT than by chest x-ray or symptoms. The
most frequent respiratory pattern is restrictive syndrome
secondary to respiratory muscular deficit and evidenced
by a reduction of lung volumes with normal DLCO and
chest x-ray. We couldn't detect any risk factor predicting a
major lung involvement, but the longer is the follow-up,
the more frequent a functional impairment is present.
from 15th Paediatric Rheumatology European Society (PreS) Congress
London, UK. 14–17 September 2008
Published: 15 September 2008
Pediatric Rheumatology 2008, 6(Suppl 1):P219 doi:10.1186/1546-0096-6-S1-P219
<supplement> <title> <p>15<sup>th </sup>Paediatric Rheumatology European Society (PreS) Congress</p> </title> <editor>Wietse Kuis, Patricia Woo, Angelo Ravelli, Hermann Girschick, Michaël Hofer, Johannes Roth, Rotraud K Saurenmann, Alberto Martini, Pavla Dolezova, Janjaap van der Net, Pierre Quartier, Lucy Wedderburn and Jan Scott</editor> <note>Meeting abstracts – A single PDF containing all abstracts in this Supplement is available <a href="http://www.biomedcentral.com/content/files/PDF/1546-0096-6-S1-full.pdf">here</a>.</note> </supplement>
This abstract is available from: http://www.ped-rheum.com/content/6/S1/P219
© 2008 Fabi et al; licensee BioMed Central Ltd. 


Respiratory involvement in juvenile dermatomyositis

Crossref

Fabi M

Le Bourgeois M

Bodemer C

Beguin V

Prieur AM

Quartier P

de Blic J

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Pediatric Rheumatology

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Respiratory involvement in juvenile dermatomyositis

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