{"doi":"10.1002/pul2.70106","title":"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.","journal":"Pulmonary Circulation","year":2025,"id":567464,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":0,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9604,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2025-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1231263,"name":"Shannon Cheung","orcid":"0000-0002-5806-642X","position":1,"is_corresponding":false},{"id":1471822,"name":"Roberto Alejandro Valdovinos","orcid":null,"position":2,"is_corresponding":false},{"id":909425,"name":"Elena K. Amin","orcid":"0000-0002-7698-278X","position":3,"is_corresponding":false},{"id":929931,"name":"Hythem Nawaytou","orcid":"0000-0002-9155-2665","position":4,"is_corresponding":false},{"id":1294248,"name":"Elizabeth Colglazier","orcid":"0000-0001-7517-0299","position":5,"is_corresponding":false},{"id":1322595,"name":"Claire Parker","orcid":"0000-0002-8431-7130","position":6,"is_corresponding":false},{"id":1471516,"name":"April Edwell","orcid":"0000-0001-7185-9566","position":7,"is_corresponding":false},{"id":260525,"name":"Susan Kim","orcid":"0000-0003-2159-8059","position":8,"is_corresponding":false},{"id":976529,"name":"Jeffrey R. Fineman","orcid":"0000-0001-9740-405X","position":9,"is_corresponding":false},{"id":1131883,"name":"Stephanie M. Tsoi","orcid":"0000-0002-0115-5843","position":0,"is_corresponding":true}],"reference_count":10,"raw_metadata":null,"created_at":"2026-07-19T02:56:44.340853Z","pmid":"40463491","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}