RT Journal Article
SR Electronic
T1 Severe cardiac involvement in children with systemic sclerosis and myositis.
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 1767
OP 1773
VO 29
IS 8
A1 Pierre Quartier
A1 Damien Bonnet
A1 Jean-Christophe Fournet
A1 Christine Bodemer
A1 Philippe Acar
A1 Marie Ouachée-Chardin
A1 Jérome Le Bidois
A1 Anne-Marie Prieur
YR 2002
UL http://www.jrheum.org/content/29/8/1767.abstract
AB OBJECTIVE: To assess the outcome of children with systemic sclerosis (SSc) and features of polymyositis. METHODS: The charts of 4 children who met the American College of Rheumatology criteria for SSc and had features of polymyositis, as defined by the presence of proximal muscle weakness and elevated serum creatine phosphokinase or aldolase level, were retrospectively reviewed. RESULTS: All children had multivisceral involvement including (1) myocardial perfusion defects in all cases, with mild to severe dilated cardiomyopathy in 3; (2) lung restrictive syndrome in 3; (3) mild to severe esophageal involvement in all cases; and (4) severe intestinal dysfunction in one child. Combination therapy of corticosteroids, methotrexate (MTX), and cyclosporine resulted in improved skin thickness and muscle strength scores in all cases, as well as in lung restrictive syndrome in 2, but was not effective regarding the progression of intestinal malabsorption in one patient, esophageal dysmotility in 3 patients, and dilated cardiomyopathy in 3. Endstage cardiac failure caused 2 deaths. In one child, heart transplantation was performed for the first time in this indication. CONCLUSION: Children with diffuse cutaneous SSc and features of polymyositis are prone to develop severe cardiomyopathy. Combination therapy of corticosteroids, MTX, and cyclosporine seems to be active on muscle, skin, and lung involvement but does not impair progression of esophageal or myocardial dysfunction. Heart transplantation might be considered, as an experimental treatment, in young patients with severe cardiomyopathy and no other irreversible organ damage.