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Primary pulmonary hypertension in a patient with systemic-onset juvenile arthritis

✍ Scribed by Shai Padeh; Ronald M. Laxer; Meredith M. Silver; Earl D. Silverman

Publisher: John Wiley and Sons
Year: 2010
Tongue: English
Weight: 582 KB
Volume: 34
Category: Article
ISSN: 0004-3591
DOI: 10.1002/art.1780341216

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✦ Synopsis

We describe a 16-year-old girl with systemic-onset juvenile arthritis who presented with pulmonary hypertension, without evidence of pleural or parenchymal involvement of the lung, pulmonary vasculitis, or immune deposition in the pulmonary vasculature. Pleuropulmonary involvement occurs occasionally in juvenile arthritis, but primary pulmonary hypertension has not, to our knowledge, been previously reported. Histocompatibility typing showed positivity for HLA-DR3 and DRw52, both of which are associated with idiopathic pulmonary hypertension in children, and with pulmonary hypertension among patients with systemic sclerosis. Treatment with cyclosporine and corticosteroids resulted in a marked improvement in the clinical findings and pulmonary function in our patient.

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