Severe Cardiac Involvement in Children with Systemic Sclerosis and Myositis

PIERRE QUARTIER, DAMIEN BONNET, JEAN-CHRISTOPHE FOURNET, CHRISTINE BODEMER, PHILIPPE ACAR, MARIE OUACHÉE-CHARDIN, JÉROME LE BIDOIS, and ANNE-MARIE PRIEUR

ABSTRACT.

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. (J Rheumatol 2002:29:1767-73)

Key Indexing Terms:

SYSTEMIC SCLEROSIS
MYOSITIS
CHILD
CARDIOMYOPATHY
IMMUNOSUPPRESSION
HEART TRANSPLANTATION

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From the Unité d'immunologie-hématologie et rhumatologie pédiatrique, Service de cardiologie pédiatrique, Service d'anatomo-pathologie, and Service de dermatologie pédiatrique, Hôpital Necker-Enfants Malades, Paris, France.

P. Quartier, MD, Unité d'immunologie-hématologie et rhumatologie pédiatrique; D. Bonnet, MD, PhD, Service de cardiologie pédiatrique; J-C. Fournet, MD, PhD, Service d'anatomo-pathologie; C. Bodemer, MD, PhD, Service de dermatologie pédiatrique; P. Acar, MD, Service de cardiologie pédiatrique; M. Ouachée-Chardin, MD, Unité d'immunologie-hématologie et rhumatologie pédiatrique; J. Le Bidois, MD, Service de cardiologie pédiatrique; A-M. Prieur, MD, Unité d'immunologie-hématologie et rhumatologie pédiatrique.

Address reprint requests to Dr. P. Quartier, Unité d'immuno-hématologie et rhumatologie pédiatrique, Hôpital Necker-Enfants Malades, 149 rue de Sèvres, 75 743 Paris cedex 15, France.

Submitted June 4, 2001; revision accepted February 27, 2002.

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