title: [Disease course, frequency of relapses and survival of patients with juvenile or adult dermatomyositis].
abstract: BACKGROUND
  The idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by chronic muscle inflammation resulting progressive weakness and frequent involvement of internal organs, mainly the pulmonary, gastrointestinal and cardiac systems.
  
  
  OBJECTIVE
  To present clinical characteristics, disease course, frequency of relapses and survival of 79 patients with juvenile or adult dermatomyositis.
  
  
  METHODS
  A national registry of patients with juvenile dermatomyositis was elaborated by the authors in Hungary. The authors summarize data of the register such as signs and symptoms, disease course, frequency of relapses and survival of patients with juvenile dermatomyositis. Analysis was performed using data of 44 patients diagnosed between 1976 and 2004 according to Bohan and Peter's criteria. Survival probability was calculated by Kaplan-Meier method. Data of patients with juvenile dermatomyositis were compared with data of 35 patients with adult dermatomyositis.
  
  
  RESULTS
  In view of the disease course, the authors found that more than the half of patients have monophasic disease, while one third of them suffered from polycyclic disease. The risk of the relapse was found to be higher during the first year after the remission. None of the juvenile patients died. Among adult patients, 4 disease-specific deaths occurred.
  
  
  CONCLUSIONS
  There was no correlation between relapse free survival and initial therapeutic regimen. Many of the patients had polycyclic or chronic disease. As relapses can occur after a prolonged disease-free interval, patients should be followed up for at least 2 years. Despite favourable survival probability, further investigations are needed to assess functional outcome.
authors.affiliation: Semmelweis Egyetem, Altalános Orvostudományi Kar, II. Gyermekgyógyászati Klinika, Budapest, Tuzoltó u. 7-9., 1094. ctamas@gyer2.sote.hu
authors.forename: Tamás
authors.initials: T
authors.lastname: Constantin
authors.forename: Andrea
authors.initials: A
authors.lastname: Ponyi
authors.forename: Tamás
authors.initials: T
authors.lastname: Kálovics
authors.forename: Ilonka
authors.initials: I
authors.lastname: Orbán
authors.forename: Katalin
authors.initials: K
authors.lastname: Molnár
authors.forename: Beáta
authors.initials: B
authors.lastname: Dérfalvy
authors.forename: Ferenc
authors.initials: F
authors.lastname: Dicso
authors.forename: Agnes
authors.initials: A
authors.lastname: Sallai
authors.forename: Miklós
authors.initials: M
authors.lastname: Garami
authors.forename: Zsolt
authors.initials: Z
authors.lastname: Balogh
authors.forename: Zsuzsanna
authors.initials: Z
authors.lastname: Szalai
authors.forename: György
authors.initials: G
authors.lastname: Fekete
authors.forename: Katalin
authors.initials: K
authors.lastname: Dankó
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D003882:Dermatomyositis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy* / Q000401:mortality; D018450:Disease Progression; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D006814:Hungary / Q000453:epidemiology; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D016019:Survival Analysis
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents