TY  - INPR
N2  - 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.
ID  - discovery10165342
PB  - Clinical and Experimental Rheumatology Sas
UR  - https://www.clinexprheumatol.org/default.asp
JF  - Clinical and Experimental Rheumatology
A1  - Ciurtin, Coziana
KW  - juvenile systemic lupus erythematosus
KW  -  pulmonary hypertension
KW  -  vasculitis
KW  -  paediatrics
TI  - Pulmonary hypertension in juvenile-onset systemic  lupus erythematosus: a case series
Y1  - 2023/05/08/
AV  - public
N1  - This version is the author accepted manuscript. For information on re-use, please refer to the publisher's terms and conditions.
ER  -