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<https://discovery.ucl.ac.uk/id/eprint/10165342> <http://purl.org/dc/terms/title> "Pulmonary hypertension in juvenile-onset systemic  lupus erythematosus: a case series"^^<http://www.w3.org/2001/XMLSchema#string> .
<https://discovery.ucl.ac.uk/id/eprint/10165342> <http://purl.org/ontology/bibo/abstract> "OBJECTIVES: Systemic lupus erythematosus (SLE) is a rare multisystem autoimmune disorder with a variable clinical phenotype.\r\nPulmonary hypertension (PHTN) is a recognised (and not uncommonly asymptomatic) complication of the condition\r\nwith an associated poor prognosis in adults. It is relatively rare in juvenile-onset SLE (JSLE). METHODS: We present a retrospective descriptive case series of four female children aged 4 to 15 years at presentation of JSLE\r\n and aged 8 to 27 years at time of diagnosis of PHTN from the United Kingdom. All cases were identified through the\r\nUK JSLE Cohort Study. RESULTS: Of 665 children with JSLE in the UK cohort study to date (data from 2006–2020), four (0.6%) were identified as\r\nhaving PHTN. 3/4 of the PHTN cases presented with cardiovascular symptoms and / or signs at presentation.\r\n3/4 were treated with Rituximab and had a good long-term outcome. Shared clinical features include high baseline\r\ndisease activity scores. CONCLUSION: JSLE has a high associated cardiovascular morbidity and mortality and early identification of treatable complications\r\nsuch as PHTN is vital. We suggest that children with high baseline disease activity scores and those presenting with\r\ncardiovascular symptoms and signs are most likely to have concurrent PHTN. Routine echocardiography is an\r\neffective screening tool and should be used as part of a standard diagnostic work-up."^^<http://www.w3.org/2001/XMLSchema#string> .
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