Caroline Platt, Catherine Longthorpe, Jacqueline Sit, Stephen D. Marks, Matthew Harmer, Cozianna Ciurtin, Athimalaipet V. Ramanan, Michael W. Beresford
Bristol Royal Hospital for Children. Evelina London Children’s Hospital. University College London and Great Ormond Street Hospital for Children NHS Foundation Trust. Southampton Children’s Hospital. University of Liverpool and Alder Hey Children’s NHS Foundation Trust
United Kingdom
Clinical and Experimental Rheumatology
Clin Exp Rheumatol 2023;
DOI: 10.55563/clinexprheumatol/pye2t3
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.
Conclusions: 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.
Category
Class I. Pulmonary Hypertension Associated with Connective Tissue Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
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