Abstract | OBJECTIVE: METHODS: 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:
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Authors | Pierre Quartier, Damien Bonnet, Jean-Christophe Fournet, Christine Bodemer, Philippe Acar, Marie Ouachée-Chardin, Jérome Le Bidois, Anne-Marie Prieur |
Journal | The Journal of rheumatology
(J Rheumatol)
Vol. 29
Issue 8
Pg. 1767-73
(Aug 2002)
ISSN: 0315-162X [Print] Canada |
PMID | 12180742
(Publication Type: Journal Article)
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Chemical References |
- Immunosuppressive Agents
- Cyclosporine
- Prednisone
- Methotrexate
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Topics |
- Adolescent
- Cardiomyopathies
(etiology, mortality, pathology)
- Child
- Cyclosporine
(therapeutic use)
- Drug Therapy, Combination
- Humans
- Immunosuppressive Agents
(therapeutic use)
- Male
- Methotrexate
(therapeutic use)
- Polymyositis
(complications, drug therapy, pathology)
- Prednisone
(therapeutic use)
- Scleroderma, Systemic
(complications, drug therapy, pathology)
- Survival Rate
- Treatment Outcome
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