Long‐term outcome and prognostic factors of juvenile dermatomyositis: A multinational, multicenter study of 490 patients

Arthritis Care and Research - Tập 62 Số 1 - Trang 63-72 - 2010
Angelo Ravelli1, Lucia Trail2, Cristina Ferrari2, Nicolino Ruperto2, Angela Pistorio2, Clarissa Pilkington3, Susan Maillard4, Sheila Oliveira5, Flávio Sztajnbok6, Rubén Cuttica7, M. Beltramelli8, Fabrizia Corona8, María Martha Katsicas9, Ricardo Russo9, Virgínia Paes Leme Ferriani10, Rubén Burgos‐Vargas11, Silvia Magni‐Manzoni12, Eunice Solis-Valleoj13, Márcia Bandeira14, Francesco Zulian15, Vicente Baca16, Elisabetta Cortis17, Fernanda Falcini18, Maria Alessio19, M. G. Alpigiani2, V. Gerloni20, Claudia Saad‐Magalhães21, Rosanna Podda22, Clóvis A. Silva23, Loredana Lepore24, Enrico Felici2, Federica Rossi2, Elena Sala2, Alberto Martini1
1Istituto di Ricovero e Cura a Carattere Scientifico G. Gaslini and Università degli Studi di Genoa, Genoa, Italy.
2Istituto di Ricovero e Cura a Carattere Scientifico G. Gaslini, Genoa, Italy
3Great Ormond Street Hospital for Children and University College London Institute of Child Health, London, UK
4University College London Institute of Child Health, London, UK
5Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil
6Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil
7Hospital General de Niños “Pedro de Elizalde”, Buenos Aires, Argentina
8Fondazione IRCCS Policlinico, Mangiagalli e Regina Elena, Milan, Italy
9Hospital de Pediatria Juan P. Garrahan, Buenos Aires, Argentina
10Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo, Ribeirão Preto, Brazil
11Hospital General de Mexico, Mexico City, Mexico
12Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. Matteo, Pavia, Italy
13Centro Medical National La Raza, Mexico City, Mexico
14Hospital Pequeno Principe, Curitiba Parana, Brazil
15Clinica Pediatrica I, Padua, Italy
16Centro Medico Nacional Siglo XXI Mexico City, Mexico
17Ospedale Pediatrico Bambino Gesù, Rome, Italy
18Ospedale Villa Monna Tessa, Florence, Italy
19Università di Napoli Federico II, Naples, Italy
20Istituto Ortopedico Gaetano Pini, Milan, Italy
21Universitade Estadual Paulista, Botucatu, Brazil
22II Clinica Pediatrica, Cagliari, Italy
23Universidade de São Paulo, São Paulo, Brazil
24Istituto di Ricovero e Cura a Carattere Scientifico Burlo Garofalo, Trieste, Italy

Tóm tắt

AbstractObjectiveTo investigate the long‐term outcome and prognostic factors of juvenile dermatomyositis (DM) through a multinational, multicenter study.MethodsPatients 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).ResultsA 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%.ConclusionThis 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.

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Arthritis Care and Research

Tập 62 Số 1

63-72

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