Kivonat:
Idiopathic inflammatory myopathies (IIMs) are systemic
autoimmune diseases characterized by chronic muscle inflammation
resulting in progressive weakness and frequent involvement of
internal organs, mainly the pulmonary, gastrointestinal and
cardiac systems which considerably contribute to the morbidity
and mortality of the IIMs. Aim of this study was to present
clinical characteristics, disease course, frequency of relapses
and survival in patients with juvenile dermatomyositis (DM). A
national registry of patients with juvenile IIMs was elaborated
by the authors in Hungary. We have summarized data of the
register according to signs and symptoms, disease course,
frequency of relapses and survival of patients with juvenile
IIM. Analysis was performed using data of 44 patients with
juvenile DM 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 DM were
compared with data of 66 patients with adult DM. The most
frequent cutaneous features were facial erythema and heliotrope
rash. Extramuscular and extraskeletal manifestations of the
disease were more frequent in adult patients. The most common
extramuscular feature was arthralgia in both groups of patients
with juvenile or adult DM. Cardiac manifestation of the disease
was not observed in juvenile patients. Respiratory muscle
involvement and interstitial lung disease (ILD) were more
frequent among adult DM patients than cardiac manifestation of
the myositis. In view of the disease course, the authors found
that frequency of polycyclic and monophasic subtypes of the
disease were mainly similar. The hazard of relapse was found
higher during the first year after the remission. None of the
juvenile patients died. Among adult patients four disease-
specific deaths occurred. There was no correlation between
relapse free survival and initial therapeutic regimen. Many of
our 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. Although we found
favourable survival probability, further investigations are
needed to assess functional outcome.
