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 art...
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| Format: | Article |
| Language: | English |
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Wiley
2025-04-01
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| Series: | Pulmonary Circulation |
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| Online Access: | https://doi.org/10.1002/pul2.70106 |
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| author | Stephanie M. Tsoi Shannon Cheung Roberto Alejandro Valdovinos Elena Amin Hythem Nawaytou Elizabeth Colglazier Claire Parker April Edwell Susan Kim Jeffrey R. Fineman |
| author_facet | Stephanie M. Tsoi Shannon Cheung Roberto Alejandro Valdovinos Elena Amin Hythem Nawaytou Elizabeth Colglazier Claire Parker April Edwell Susan Kim Jeffrey R. Fineman |
| author_sort | Stephanie M. Tsoi |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-dd254a80d0e7458486b2a63cef730ad8 |
| institution | Kabale University |
| issn | 2045-8940 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | Wiley |
| record_format | Article |
| series | Pulmonary Circulation |
| spelling | doaj-art-dd254a80d0e7458486b2a63cef730ad82025-08-20T03:27:37ZengWileyPulmonary Circulation2045-89402025-04-01152n/an/a10.1002/pul2.70106Juvenile Systemic Sclerosis Presenting With Severe Pulmonary Vascular Disease: ECMO Stabilization During Aggressive Therapy Resulting in a Favorable OutcomeStephanie M. Tsoi0Shannon Cheung1Roberto Alejandro Valdovinos2Elena Amin3Hythem Nawaytou4Elizabeth Colglazier5Claire Parker6April Edwell7Susan Kim8Jeffrey R. Fineman9Division of Critical Care Medicine, Department of Pediatrics University of California San Francisco San Francisco California USADivision of Critical Care Medicine, Department of Pediatrics University of California San Francisco San Francisco California USADivision of Rheumatology, Department of Pediatrics University of California, San Francisco San Francisco California USADivision of Cardiology, Department of Pediatrics University of California San Francisco San Francisco California USADivision of Cardiology, Department of Pediatrics University of California San Francisco San Francisco California USADivision of Critical Care Medicine, Department of Pediatrics University of California San Francisco San Francisco California USADivision of Critical Care Medicine, Department of Pediatrics University of California San Francisco San Francisco California USADivision of Critical Care Medicine, Department of Pediatrics University of California San Francisco San Francisco California USADivision of Rheumatology, Department of Pediatrics University of California, San Francisco San Francisco California USADivision of Critical Care Medicine, Department of Pediatrics University of California San Francisco San Francisco California USAABSTRACT 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.https://doi.org/10.1002/pul2.70106extracorporeal membranous oxygenationjuvenile systemic sclerosispediatric critical carepulmonary hypertensionright ventricular failure |
| spellingShingle | Stephanie M. Tsoi Shannon Cheung Roberto Alejandro Valdovinos Elena Amin Hythem Nawaytou Elizabeth Colglazier Claire Parker April Edwell Susan Kim Jeffrey R. Fineman Juvenile Systemic Sclerosis Presenting With Severe Pulmonary Vascular Disease: ECMO Stabilization During Aggressive Therapy Resulting in a Favorable Outcome Pulmonary Circulation extracorporeal membranous oxygenation juvenile systemic sclerosis pediatric critical care pulmonary hypertension right ventricular failure |
| title | Juvenile Systemic Sclerosis Presenting With Severe Pulmonary Vascular Disease: ECMO Stabilization During Aggressive Therapy Resulting in a Favorable Outcome |
| title_full | Juvenile Systemic Sclerosis Presenting With Severe Pulmonary Vascular Disease: ECMO Stabilization During Aggressive Therapy Resulting in a Favorable Outcome |
| title_fullStr | Juvenile Systemic Sclerosis Presenting With Severe Pulmonary Vascular Disease: ECMO Stabilization During Aggressive Therapy Resulting in a Favorable Outcome |
| title_full_unstemmed | Juvenile Systemic Sclerosis Presenting With Severe Pulmonary Vascular Disease: ECMO Stabilization During Aggressive Therapy Resulting in a Favorable Outcome |
| title_short | Juvenile Systemic Sclerosis Presenting With Severe Pulmonary Vascular Disease: ECMO Stabilization During Aggressive Therapy Resulting in a Favorable Outcome |
| title_sort | juvenile systemic sclerosis presenting with severe pulmonary vascular disease ecmo stabilization during aggressive therapy resulting in a favorable outcome |
| topic | extracorporeal membranous oxygenation juvenile systemic sclerosis pediatric critical care pulmonary hypertension right ventricular failure |
| url | https://doi.org/10.1002/pul2.70106 |
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