Juvenile dermato/polymyositis: a retrospective analysis of 33 cases with special focus on initial CPK levels.
Retrospective analysis of 33 patients with juvenile dermato/polymyositis showed 45% complete recovery, 26% remission (steroid dependent), 6% wheelchair-dependency, 3% deaths and 10% development of other connective tissue diseases after a mean follow up of 4 years. Patients who received 2 mg/kg of prednisone had the worst prognosis but since they apparently represent a subgroup it is questionable whether high dose prednisone was the cause of the poor prognosis. This subgroup is characterized by high CPK serum levels (a 10-fold increase or more) and an acute type of onset. An initial high ANA titer was found to predict the later development of other connective tissue disorders.
Van Rossum
M A
MA
University Hospital for Children and Youth, Het Wilhelmina Kinderziekenhuis, Department of Rheumatology and Immunology, Utrecht, The Netherlands.
Hiemstra
I
I
Prieur
A M
AM
Rijkers
G T
GT
Kuis
W
W
D000293:Adolescent; D000974:Antibodies, Antinuclear / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D003402:Creatine Kinase / Q000097:blood*; D003882:Dermatomyositis / Q000097:blood / Q000201:enzymology* / Q000453:epidemiology*; D004305:Dose-Response Relationship, Drug; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D017285:Polymyositis / Q000188:drug therapy / Q000201:enzymology* / Q000453:epidemiology*; D011241:Prednisone / Q000627:therapeutic use; D012189:Retrospective Studies
D000974:Antibodies, Antinuclear; D003402:Creatine Kinase; D011241:Prednisone