Pulmonary hypertension in juvenile-onset systemic lupus erythematosus: a case series.

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.

Paediatric rhabdomyolysis: A UK centre's 10-year retrospective experience.

AIMS: To describe the aetiologies of paediatric rhabdomyolysis and explore the medium-term renal consequences. METHODS: Retrospective, single-centre review of children with rhabdomyolysis. RESULTS: Two hundred and thirty-two children met inclusion criteria for the analysis. Mean age at presentation was 8.4 (SD ± 5.5) years. The commonest aetiology was infection (28%), with viral myositis making up the clear majority (75%). Trauma was identified as a cause in 18% of children, seizures in 10% and immune-mediated mechanisms in 8%. Acute kidney injury (AKI) was present in 32% of the cases overall. Children with AKI tended to be younger, with higher peak creatine kinase (CK) and active urinary sediment on urinalysis at presentation. AKI and the need for renal replacement therapy (RRT) were associated with a prolonged hospital stay (15 (interquartile range, IQR 6.5-33) vs. 2 (IQR 0-7) days). A total of 18 children and young people required RRT, with a mean duration of 7.1 ± 4.3 days. Those who received RRT were more likely to have abnormalities on urinalysis at presentation (46% vs. 5%). Over the period of the study, 9% of children died and 2% met criteria for a diagnosis of chronic kidney disease. CONCLUSIONS: This large paediatric rhabdomyolysis case series provides new and unique insights into the condition. Our results highlight the common aetiologies and provide evidence of good renal recovery overall, even in the most severely affected cases. Abnormalities of urinalysis appear to be important in predicting the development of AKI and the need for RRT.