Systemic Sclerosis Sine Scleroderma With Pulmonary Arterial Hypertension in a 3-Year-Old Girl

Systemic sclerosis sine scleroderma (ssSSc) is a rare variant of systemic sclerosis, with only one pediatric case reported in the medical literature to date. Pulmonary arterial hypertension as the presenting feature of ssSSc is extremely rare, even in adults, and so far has never been reported in ch...

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Bibliographic Details
Published in:Pediatrics (Evanston) 2020-05, Vol.145 (5), p.e20192504
Main Authors: Zloof, Yair, Schonfeld, Tommy, Dagan, Tamir, Amarilyo, Gil, Braun, Maya, Hashkes, Philip, Harel, Liora
Format: Article
Language:English
Subjects:
Health risk assessment
Hypertension
Inflammation
Lung diseases
Pediatrics
Pulmonary hypertension
Scleroderma
Systemic sclerosis
Vasodilators
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Summary:Systemic sclerosis sine scleroderma (ssSSc) is a rare variant of systemic sclerosis, with only one pediatric case reported in the medical literature to date. Pulmonary arterial hypertension as the presenting feature of ssSSc is extremely rare, even in adults, and so far has never been reported in children. We report, for the first time, a case of pediatric ssSSc in a 3-year-old girl, who presented with interstitial lung disease and pulmonary hypertension. The patient was prescribed early aggressive pulmonary vasodilators combined with anti-inflammatory medications. The clinical response was good, and her current condition at 12 years of age is remarkable, considering the high mortality rates reported in adults. We underscore the importance of early aggressive treatment in future cases of similar presentation.
ISSN:0031-4005
1098-4275
DOI:10.1542/PEDS.2019-2504