eprintid: 10165342
rev_number: 7
eprint_status: archive
userid: 699
dir: disk0/10/16/53/42
datestamp: 2023-02-23 11:14:50
lastmod: 2023-02-23 11:14:50
status_changed: 2023-02-23 11:14:50
type: article
metadata_visibility: show
sword_depositor: 699
creators_name: Ciurtin, Coziana
title: Pulmonary hypertension in juvenile-onset systemic  lupus erythematosus: a case series
ispublished: inpress
divisions: UCL
divisions: B02
divisions: C10
divisions: D17
divisions: G90
keywords: juvenile systemic lupus erythematosus, pulmonary hypertension, vasculitis, paediatrics
note: This version is the author accepted manuscript. For information on re-use, please refer to the publisher's terms and conditions.
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.
date: 2023-05-08
date_type: published
publisher: Clinical and Experimental Rheumatology Sas
official_url: https://www.clinexprheumatol.org/default.asp
oa_status: green
full_text_type: other
language: eng
primo: open
primo_central: open_green
verified: verified_manual
elements_id: 2006426
lyricists_name: Ciurtin, Coziana
lyricists_id: CCIUR23
actors_name: Ciurtin, Coziana
actors_id: CCIUR23
actors_role: owner
full_text_status: public
publication: Clinical and Experimental Rheumatology
citation:        Ciurtin, Coziana;      (2023)    Pulmonary hypertension in juvenile-onset systemic lupus erythematosus: a case series.                   Clinical and Experimental Rheumatology           (In press).    Green open access   
 
document_url: https://discovery.ucl.ac.uk/id/eprint/10165342/1/CER%2016248%20Platt.pdf