Clinical characteristics and prognostic factor in juvenile dermatomyositis : data of the Spanish registry
Carriquí-Arenas, Sonia (Institut de Recerca Sant Joan de Déu)
Mosquera, Juan Manuel (Institut de Recerca Sant Joan de Déu)
Quesada Masachs, Estefania (Hospital Universitari Vall d'Hebron)
López, Mireia (Hospital Universitari Vall d'Hebron)
Clemente, Daniel (Hospital Infantil Universitario Niño Jesús (Madrid))
Boteanu, Alina (Hospital Universitario Ramón y Cajal (Madrid))
Udaondo, Clara (Hospital Universitario infantil La Paz (Madrid))
de Inocencio, Jaime (Hospital Universitario 12 de Octubre (Madrid))
Nieto, Juan Carlos (Hospital General Universitario Gregorio Marañón)
Riancho, Leyre (Hospital Universitario Marqués de Valdecilla (Santander, Cantabria))
Núñez, Esmeralda (Hospital Universitario Regional de Málaga (Andalusia))
Sánchez Manubens, Judith (Parc Taulí Hospital Universitari. Institut d'Investigació i Innovació Parc Taulí (I3PT))
Lirola, María José (Hospital Universitario Virgen Macarena (Sevilla, Andalusia))
Roldán, Rosa (Hospital Universitario Reina Sofía (Còrdova, Espanya))
Camacho, Marisol (Hospital Universitario Virgen del Rocío (Sevilla, Andalusia))
Martínez, Melania (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Medrano, Marta (Hospital Universitario Miguel Servet (Saragossa))
Alcañiz, Paula (Hospital Universitario Virgen de la Arrixaca (Múrcia))
Antón, Jordi (Institut de Recerca Sant Joan de Déu)
Iglesias, Estíbaliz (Institut de Recerca Sant Joan de Déu)
Universitat Autònoma de Barcelona. Departament de Pediatria, Obstetrícia i Ginecologia i de Medicina Preventiva i Salut Pública

Fecha:	2024
Resumen:	Juvenile Dermatomyositis (JDM) is the most common chronic idiopathic inflammatory myopathy in children. The diagnosis is clinical. Baseline laboratory and complementary studies trace the phenotype of these patients. The objective of this study was to describe epidemiological, clinical and laboratory characteristics at diagnosis of JDM patients included in the Spanish JDM registry, as well as to identify prognostic factors on these patients. We retrospectively reviewed clinical features, laboratory tests, and complementary studies at diagnosis of JDM patients included on the Spanish JDM registry. These data were analyzed to assess whether there was a relationship with the development of complications and time to disease inactivity. One hundred and sixteen patients from 17 Spanish paediatric rheumatology centres were included, 76 girls (65%). Median age at diagnosis was 7. 3 years (Interquartile range (IQR) 4. 5-10. 2). All patients had pathognomonic skin lesions at the beginning of the disease. Muscle weakness was present in 86. 2%. Median Childhood Muscle Assessment Scale was 34 (IQR 22-47). Twelve patients (34%) had dysphagia and 3,5% dysphonia. Anti-p155 was the most frequently detected myositis specific antibody, followed by anti-MDA5. Twenty-nine patients developed calcinosis and 4 presented with macrophage activation syndrome. 70% reached inactivity in a median time of 8. 9 months (IQR 4. 5-34. 8). 41% relapsed after a median time of 14. 4 months (IQR 8. 6-22. 8) of inactivity. Shorter time to treatment was associated with better prognosis (Hazard ratio (HR) = 0. 95 per month of evolution, p = 0. 02). Heliotrope rash at diagnosis correlates with higher risk of development complications. We describe heliotrope rash as a risk factor for developing complications in our cohort of JDM patients, an easy-to-evaluate clinical sign that could help us to identify the group of patients we should monitor closely for this complication.
Derechos:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Lengua:	Anglès
Documento:	Article ; recerca ; Versió publicada
Materia:	Juvenile dermatomyositis ; Clinical features ; Medical tests ; Prognostic factors
Publicado en:	Pediatric rheumatology online journal, Vol. 22 Num. 66 (July 2024) , ISSN 1546-0096

DOI: 10.1186/s12969-024-00999-9
PMID: 39039532

8 p, 974.4 KB

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