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Long‐term outcome and prognostic factors of juvenile dermatomyositis: A multinational, multicenter study of 490 patients
Author(s) -
Ravelli Angelo,
Trail Lucia,
Ferrari Cristina,
Ruperto Nicolino,
Pistorio Angela,
Pilkington Clarissa,
Maillard Susan,
Oliveira Sheila K.,
Sztajnbok Flavio,
Cuttica Ruben,
Beltramelli Matilde,
Corona Fabrizia,
Katsicas Maria Martha,
Russo Ricardo,
Ferriani Virginia,
BurgosVargas Ruben,
MagniManzoni Silvia,
SolisValleoj Eunice,
Bandeira Marcia,
Zulian Francesco,
Baca Vicente,
Cortis Elisabetta,
Falcini Fernanda,
Alessio Maria,
Alpigiani Maria Giannina,
Gerloni Valeria,
SaadMagalhaes Claudia,
Podda Rosanna,
Silva Clovis A.,
Lepore Loredana,
Felici Enrico,
Rossi Federica,
Sala Elena,
Martini Alberto
Publication year - 2009
Publication title -
arthritis care and research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.032
H-Index - 163
eISSN - 2151-4658
pISSN - 2151-464X
DOI - 10.1002/acr.20015
Subject(s) - medicine , juvenile dermatomyositis , quality of life (healthcare) , disease , pediatrics , nursing
Abstract 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%. Conclusion 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.