Am. Huber et al., Medium- and long-term functional outcomes in a multicenter cohort of children with juvenile dermatomyositis, ARTH RHEUM, 43(3), 2000, pp. 541-549
Objective. To evaluate functional outcomes in a cohort of patients with juv
enile dermatomyositis (DM),
Methods. A retrospective inception cohort of patients diagnosed as having j
uvenile DM between January 1, 1984 and January 1, 1995 was established at 4
Canadian tertiary care pediatric centers. Informed consent was obtained, E
ach subject was interviewed by telephone or in person. The primary outcome
was physical function, as measured by the Childhood Health Assessment Quest
ionnaire (CHAQ), Additional outcomes were educational and vocational achiev
ement, growth, development of calcinosis, patient satisfaction with outcome
, and development of other illnesses. Data regarding illness presentation,
treatment, and disease course were obtained through chart review.
Results. Sixty-five of 80 patients (81%; 46 females and 19 males) could be
contacted. The median followup time was 7.2 years (range 3.2-13.9 years), w
ith a median age at followup of 13 years (range 7-26 years). Twenty-four pa
tients (37%) had a monocyclic course, while the remaining 41 (63%) had a ch
ronic continuous or polycyclic course. Sixty-two patients (95%) were treate
d with corticosteroids, while 41 (63%) received a second-line agent. Physic
al function was excellent, with a median CHAQ score of 0 (range 0-2.50), Ei
ghteen patients had scores >0, and only 5 had moderate-to-severe disability
, as defined by a CHAQ score >1.0, Females had higher CHAQ scores, and all
but 1 of the patients with scores >0 were female (range 0-2.50; P = 0.015),
Patients with a chronic continuous course also had higher CHAQ scores. Six
teen patients in the chronic continuous group had CHAQ scores >0 (range 0-2
.50; P = 0.0009), Calcinosis developed in 22 patients (34%) and persisted t
o followup in 14, Development of calcinosis was not related to initial ther
apy, sex, or disease course, but was significantly associated with higher C
HAQ scores (range 0-1.0 versus 0-2.5; P = 0.01). At the time of followup, 2
6 patients (40%) still had rash, 15 (23%) still reported weakness, and 23 (
35%) continued taking medications, despite the fact that all were at least
3 years postdiagnosis. There was 1 death,
Conclusion. In general, patients in this cohort had favorable outcomes. Mos
t had CHAQ scores of 0, and only 8% met our definition of moderate-to-sever
e disability. However, many patients continued to have chronic disease, per
sistent rash, and continued taking medications >3 years after diagnosis. Fu
rther research is needed to improve outcomes for patients with juvenile DM.