PS3:46 Relationship between damage clustering and mortality in juvenile systemic lupus erythematosus: cluster analyses in a large cohort from the spanish society of rheumatology lupus registry

Torrente-Segarra, Vicente; Salman-Monte, TC; Rúa-Figueroa, Íñigo; Calvo‐Alén, Jaime; Lόpez-Longo, FJ; Galindo, M.; Olivé, Alejandro; Pego-Reigosa, J.M.

doi:10.1136/lupus-2018-abstract.94

Cited by 5 publications

(11 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Similar to our results, hypocomplementemia was detected between 62% and 94% of the patients with juvenile onset SLE and low levels of complement C3 was shown to be more prevalent than low complement C4. 13–17 In addition hypocomplementemia were found to be more prevalent in children with SLE than in adults and complement levels were found to be higher in prepubertal children. 16–19…”

Section: Discussionmentioning

confidence: 99%

Significance of the weighted complement domain of the EULAR/ACR classification criteria in juvenile onset systemic lupus erythematosus

Kısaoğlu

Baba

Kalyoncu

2023

Lupus

View full text Add to dashboard Cite

Objective We aimed to compare the clinical and laboratory characteristics of patients with SLE according to the weighted complement status of the EULAR/ACR criteria and investigate whether different weighting of the complement status at disease onset is associated with outcomes. Methods Patients diagnosed with juvenile onset SLE who fulfilled the 2019 EULAR/ACR classification criteria were retrospectively analyzed. Results Among 43 patients included, hypocomplementemia was observed in 37 (86%), mostly with a low level of both complement C3 (C3) and complement C4 (C4) (53.5%). In patients with low levels of both C3 and C4, more common cutaneous (65.2% vs 28.6%, p: 0.045), musculoskeletal involvement (78.3% vs 42.9%, p: 0.039), autoimmune hemolytic anemia (52.2% vs 14.3%, p: 0.035), positive anti-dsDNA (65.2% vs 21.4%, p: 0.017) and anti-Sm antibodies (60.9% vs 21.4%, p: 0.04) were observed. In addition these patients had higher scores from the 2019 EULAR/ACR classification criteria (26 vs 15.5, p: < 0.0001). Remission and flare rates, and SLE associated damage were not differed according to the complement status in patients with hypocomplementemia. Conclusion Observation of more frequent clinical and serological activity with higher total scores from the EULAR/ACR classification criteria supported the higher scoring of patients with low C3 and C4 in the weighted criteria. However, since significant number of patients did not exhibit low complement C4, and the frequency of kidney involvement did not differ according to the weighted complement status, complement C3 might be suggested as a more important diagnostic tool in patients with juvenile onset SLE. Also, weighted complement status at onset did not seem to affect the disease outcomes.

show abstract

Section: Discussionmentioning

confidence: 99%

Significance of the weighted complement domain of the EULAR/ACR classification criteria in juvenile onset systemic lupus erythematosus

Kısaoğlu

Baba

Kalyoncu

2023

Lupus

View full text Add to dashboard Cite

show abstract

Section: Introductionmentioning

confidence: 99%

“…10,14–16 Thus, timely diagnosis, and treatment of NP-SLE is critical to improve patients’ quality of life and prevent damage. 14…”

Section: Introductionmentioning

confidence: 99%

“…Reports underscore that neurologic manifestations are not uncommon in JSLE, contributing to the increased morbidity and mortality demonstrated in the pediatric age group. 10,[14][15][16] Thus, timely diagnosis, and treatment of NP-SLE is critical to improve patients' quality of life and prevent damage. 14 This study aimed to investigate the prevalence, demographic characteristics, and clinical features of NP disease in JSLE patients enrolled in the UK JSLE Cohort Study.…”

Section: Introductionmentioning

confidence: 99%

“…10,[14][15][16] Thus, timely diagnosis, and treatment of NP-SLE is critical to improve patients' quality of life and prevent damage. 14 This study aimed to investigate the prevalence, demographic characteristics, and clinical features of NP disease in JSLE patients enrolled in the UK JSLE Cohort Study. Furthermore, associations between NP involvement and other clinical and/or laboratory features, as well correlation with disease severity and outcomes were investigated.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus: Data from the UK Juvenile-onset systemic lupus erythematosus cohort study

Giani

Smith

Al‐Abadi

et al. 2021

Lupus

View full text Add to dashboard Cite

Introduction Juvenile-onset systemic lupus erythematosus (JSLE) is a rare autoimmune/inflammatory disease with significant morbidity and mortality. Neuropsychiatric (NP) involvement is a severe complication, encompassing a heterogeneous range of neurological and psychiatric manifestations. Methods Demographic, clinical, and laboratory features of NP-SLE were assessed in participants of the UK JSLE Cohort Study, and compared to patients in the same cohort without NP manifestations. Results A total of 428 JSLE patients were included in this study, 25% of which exhibited NP features, half of them at first visit. Most common neurological symptoms among NP-JSLE patients included headaches (78.5%), mood disorders (48.6%), cognitive impairment (42%), anxiety (23.3%), seizures (19.6%), movement disorders (17.7%), and cerebrovascular disease (14.9%). Peripheral nervous system involvement was recorded in 7% of NP-SLE patients. NP-JSLE patients more frequently exhibited thrombocytopenia (<100 × 109/L) ( p = 0.04), higher C-reactive protein levels ( p = 0.01), higher global pBILAG score at first visit ( p < 0.001), and higher SLICC damage index score at first ( p = 0.02) and last ( p < 0.001) visit when compared to JSLE patients without NP involvement. Conclusions A significant proportion of JSLE patients experience NP involvement (25%). Juvenile-onset NP-SLE most commonly affects the CNS and is associated with increased overall disease activity and damage.

show abstract

Systemic Lupus Erythematosus-related Lung Disease

Bendstrup,

Lynn,

Troldborg

2024

Semin Respir Crit Care Med

View full text Add to dashboard Cite

Systemic Lupus Erythematosus (SLE) is a multifaceted, multisystem autoimmune disorder with diverse clinical expressions. While prevalence reports vary widely, pulmonary involvement accounts for significant morbidity and mortality in SLE. This comprehensive review explores the spectrum of pulmonary disease in SLE, including upper airway manifestations (e.g., laryngeal affection), lower airway conditions (e.g., bronchitis, bronchiolitis, bronchiectasis), parenchymal diseases (e.g., interstitial lung disease, acute lupus pneumonitis, diffuse alveolar hemorrhage), pleural diseases (e.g., serositis, shrinking lung syndrome), and vascular diseases (e.g., pulmonary arterial hypertension, pulmonary embolism, acute reversible hypoxemia syndrome). We discuss diagnostic modalities, treatment strategies, and prognosis for each pulmonary manifestation. With diagnostics remaining a challenge and with the absence of standardized treatment guidelines, we emphasize the need for evidence-based guidelines to optimize patient care and improve outcomes in this complex disease.

show abstract

PS3:46 Relationship between damage clustering and mortality in juvenile systemic lupus erythematosus: cluster analyses in a large cohort from the spanish society of rheumatology lupus registry

Cited by 5 publications

References 0 publications

Significance of the weighted complement domain of the EULAR/ACR classification criteria in juvenile onset systemic lupus erythematosus

Significance of the weighted complement domain of the EULAR/ACR classification criteria in juvenile onset systemic lupus erythematosus

Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus: Data from the UK Juvenile-onset systemic lupus erythematosus cohort study

Systemic Lupus Erythematosus-related Lung Disease

Contact Info

Product

Resources

About