Long-term outcome and prognostic factors of juvenile dermatomyositis: a multinational, multicenter study of 490 patients.
OBJECTIVE
To investigate the long-term outcome and prognostic factors of juvenile dermatomyositis (DM) through a multinational, multicenter study.


METHODS
Patients consisted of inception cohorts seen between 1980 and 2004 in 27 centers in Europe and Latin America. Predictor variables were sex, continent, ethnicity, onset year, onset age, onset type, onset manifestations, course type, disease duration, and active disease duration. Outcomes were muscle strength/endurance, continued disease activity, cumulative damage, muscle damage, cutaneous damage, calcinosis, lipodystrophy, physical function, and health-related quality of life (HRQOL).


RESULTS
A total of 490 patients with a mean disease duration of 7.7 years were included. At the cross-sectional visit, 41.2-52.8% of patients, depending on the instrument used, had reduced muscle strength/endurance, but less than 10% had severe impairment. Persistently active disease was recorded in 41.2-60.5% of the patients, depending on the activity measure used. Sixty-nine percent of the patients had cumulative damage. The frequency of calcinosis and lipodystrophy was 23.6% and 9.7%, respectively. A total of 40.7% of the patients had decreased functional ability, but only 6.5% had major impairment. Only a small fraction had decreased HRQOL. A chronic course, either polycyclic or continuous, consistently predicted a poorer outcome. Mortality rate was 3.1%.


CONCLUSIONS
This study confirms the marked improvement in functional outcome of juvenile DM when compared with earlier literature. However, many patients had continued disease activity and cumulative damage at followup. A chronic course was the strongest predictor of poor prognosis. These findings highlight the need for treatment strategies that enable a better control of disease activity over time and the reduction of nonreversible damage.
Ravelli
Angelo
A
Istituto di Ricovero e Cura a Carattere Scientifico G. Gaslini and Università degli Studi di Genoa, Genoa, Italy. angeloravelli@ospedale-gaslini.ge.it
Trail
Lucia
L
Ferrari
Cristina
C
Ruperto
Nicolino
N
Pistorio
Angela
A
Pilkington
Clarissa
C
Maillard
Susan
S
Oliveira
Sheila K
SK
Sztajnbok
Flavio
F
Cuttica
Ruben
R
Beltramelli
Matilde
M
Corona
Fabrizia
F
Katsicas
Maria Martha
MM
Russo
Ricardo
R
Ferriani
Virginia
V
Burgos-Vargas
Ruben
R
Magni-Manzoni
Silvia
S
Solis-Valleoj
Eunice
E
Bandeira
Marcia
M
Zulian
Francesco
F
Baca
Vicente
V
Cortis
Elisabetta
E
Falcini
Fernanda
F
Alessio
Maria
M
Alpigiani
Maria Giannina
MG
Gerloni
Valeria
V
Saad-Magalhaes
Claudia
C
Podda
Rosanna
R
Silva
Clovis A
CA
Lepore
Loredana
L
Felici
Enrico
E
Rossi
Federica
F
Sala
Elena
E
Martini
Alberto
A
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003882:Dermatomyositis / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D005260:Female; D006801:Humans; D007223:Infant; D038622:Internationality; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome