Juvenile Systemic Sclerosis Presenting With Severe Pulmonary Vascular Disease: ECMO Stabilization During Aggressive Therapy Resulting in a Favorable Outcome.

Abstract

Juvenile systemic sclerosis (jSSc) associated pulmonary hypertension (PH) is rare, but, the leading cause of morbidity and mortality in jSSc. This is a case of a 10-year-old girl whose initial presentation of positive U3-RNP antibody jSSc included diffuse skin findings, severe pulmonary arterial hypertension, and right ventricular failure. Veno-arterial extracorporeal membranous oxygenation (VA-ECMO) and atrial stent placement facilitated treatment with pulse-dose steroids, mycophenolate mofetil, and B-cell depleting therapy to treat the underlying autoimmune inflammation and triple therapy with treprostinil, ambrisentan, and tadalafil for her pulmonary hypertension. At 9-month follow-up, her jSSc is well-controlled with complete resolution of her PH. This case demonstrates that multi-disciplinary treatment, including upfront multi-drug therapy for jSSC and PAH, that included VA-ECMO, may improve outcomes, particularly when treatment for underlying causes (in this case, jSSc) is just being initiated.