Ciurtin, Coziana;
(2023)
Pulmonary hypertension in juvenile-onset systemic lupus erythematosus: a case series.
Clinical and Experimental Rheumatology
(In press).
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Abstract
OBJECTIVES: Systemic lupus erythematosus (SLE) is a rare multisystem autoimmune disorder with a variable clinical phenotype. Pulmonary hypertension (PHTN) is a recognised (and not uncommonly asymptomatic) complication of the condition with 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 and aged 8 to 27 years at time of diagnosis of PHTN from the United Kingdom. All cases were identified through the UK 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 having PHTN. 3/4 of the PHTN cases presented with cardiovascular symptoms and / or signs at presentation. 3/4 were treated with Rituximab and had a good long-term outcome. Shared clinical features include high baseline disease activity scores. CONCLUSION: JSLE has a high associated cardiovascular morbidity and mortality and early identification of treatable complications such as PHTN is vital. We suggest that children with high baseline disease activity scores and those presenting with cardiovascular symptoms and signs are most likely to have concurrent PHTN. Routine echocardiography is an effective screening tool and should be used as part of a standard diagnostic work-up.
| Type: | Article |
|---|---|
| Title: | Pulmonary hypertension in juvenile-onset systemic lupus erythematosus: a case series |
| Open access status: | An open access version is available from UCL Discovery |
| Publisher version: | https://www.clinexprheumatol.org/default.asp |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher's terms and conditions. |
| Keywords: | juvenile systemic lupus erythematosus, pulmonary hypertension, vasculitis, paediatrics |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10165342 |
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