Juvenile and juvenile-onset systemic lupus erythematosus patients: Clinical characteristics, disease activity and damage

Gheith, Rasha E.; El‐Gazzar, Iman I.; Fishawy, Hussein S. El; El-Din, Abeer M. Nour; Bahgat, Dina M. Rasheed; Gheita, Tamer A.

doi:10.1016/j.epag.2017.03.003

Cited by 6 publications

(9 citation statements)

References 27 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Our cohort demonstrated a high incidence of major organ involvement affecting the renal, haematological and CNS even at diagnosis, which is consistent with other pSLE studies (Table 2) but different from adult-onset SLE cohorts. 3,5,20,21,24–26 This is not surprising as younger patients have generally been known to have a more severe form of lupus. 3–6,26,27 Apart from higher rates of constitutional symptoms and vasculitic rash, the pattern of disease manifestations was similar to other paediatric cohorts (Table 2).…”

Section: Discussionmentioning

confidence: 99%

Clinical features, disease activity and outcomes of Malaysian children with paediatric systemic lupus erythematosus: A cohort from a tertiary centre

Lim

Chan

Tang

2020

Lupus

View full text Add to dashboard Cite

Background Paediatric systemic lupus erythematosus is a rare autoimmune disease with a wide spectrum of clinical presentation in different populations. We present a cohort of paediatric systemic lupus erythematosus in Malaysia where the disease features and outcomes are still largely unknown. Methods A retrospective review of all paediatric systemic lupus erythematosus patients with at least 6 months follow-up at Selayang Hospital from 2004 to 2016. Epidemiological, clinical and outcome data were collected and analysed. Results A total of 141 paediatric systemic lupus erythematosus patients, 87.9% females, were followed up for a median 6.3 years (interquartile range 3.6–9.0). The median age at diagnosis was 10.8 years (interquartile range 9.0–12.0 years), positive family history of systemic lupus erythematosus was present in 12.1% and the majority (61.7%) were of Malay ethnicity. Common presentations included fever (87.2%), vasculitic rash (72.3%) and lethargy (69.5%). At diagnosis, leukopenia (51.1%), thrombocytopenia (41.8%) and cutaneous lupus (56%) predominate with significant renal involvement (39.7%). Renal (45.4%), liver (26%) and the central nervous system (17%) were important major organs involved during the course of the disease. At diagnosis, almost all (99.3%) patients had high disease activity (mean Systemic Lupus Erythematosus Disease Activity Index score 20.1 ± 9.6). The majority (62.4%) achieved remission or low disease activity after 6 months, maintained over the next 10 years. Damage occurred early (39.1% at 1 year) and increased with time. Ocular damage was the most common side effect (29%) and was predominantly corticosteroid related (93%). Growth retardation was significant (38.2%) with no gonadal failure or secondary malignancies. End-stage renal disease occurred in 3.1% patients whereas 53.1% had sustained renal remission. Overall mortality was 1.4%. Conclusion Despite high disease activity at diagnosis, the majority had good sustained response to treatment with low overall mortality. However, there was progressive accrual of organ damage, highlighting the need for further research and refinements into therapies for paediatric systemic lupus erythematosus.

show abstract

Section: Discussionmentioning

confidence: 99%

Clinical features, disease activity and outcomes of Malaysian children with paediatric systemic lupus erythematosus: A cohort from a tertiary centre

Lim

Chan

Tang

2020

Lupus

View full text Add to dashboard Cite

show abstract

“…Childhood SLE usually has an onset at 11-12 years and is common in Asians [5]. However, it is rare to find a case under five years of age [8]. Similar cutaneous involvement was seen in another case of juvenile SLE, with erythematous, nonblanching, and a macular rash over the body [9].…”

Section: Discussionmentioning

confidence: 81%

“…Nevertheless, our ratio was higher than both, indicating a potential renal involvement, indicated by our patient's renal biopsy. Renal and neuropsychiatric involvement have been commonly associated with Juvenile SLE cases [8].…”

Section: Discussionmentioning

confidence: 99%

Study about Multisystem Autoimmune Disorder: Systemic Lupus Erythematosus, A Third World Prodigy

Qazi

Shah

Maher

et al. 2022

JPRI

View full text Add to dashboard Cite

Childhood-onset Systemic Lupus Erythematosus (SLE) has an incidence of 0.3-0.9/100,000 children. It is very rare to find a case of childhood-onset SLE before 5 years of age. We describe a case of a 4-year-old female child, from a low-socioeconomic background who was diagnosed with SLE based on a typical rash, oral ulcer, positive laboratory parameters such as ANA, anti-ds DNA, anti-nucleosome, anti-histone antibodies, and positive lupus anticoagulants. There was an involvement of each system with considerable signs and symptoms including lupus nephritis, a cardiological complication such as Libman-Sack endocarditis, and neurological manifestations showing diffuse cerebral atrophy. The child was also positive for parameters linking to the presence of Macrophage Activation Syndrome MAS). The hypercoagulable state of SLE has been linked to more central nervous system involvement, cardiovascular dysfunction, renal complication, and hyponatremia as indicated in our case making it more riveting.

show abstract

“…The main causes of death in our pediatric patients were infection and organ failure, consistent with the literature. [ 26 , 42 , 43 ] Since JSLE is associated with higher disease activity, damage indices, and comorbidities, it is unsurprising that JSLE has higher mortality rates with diverse causes of mortality.…”

Section: Discussionmentioning

confidence: 99%

Disease characteristics in patients with juvenile- and adult-onset systemic lupus erythematosus: A multi-center comparative study

et al. 2021

View full text Add to dashboard Cite

Objectives: This study aims to compare disease characteristics in patients with juvenile-onset systemic lupus erythematosus (JSLE) and adult-onset systemic lupus erythematosus (ASLE). Patients and methods: Between June 2010 and March 2020, a total of 186 patients with JSLE (23 males, 163 females; median age: 25 years; range, 20 to 30.3 years) and 236 patients with ASLE (23 males, 213 females; median age: 35 years; range, 29 to 40 years) were retrospectively analyzed. Clinical and laboratory data, treatment received, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating Clinics (SLICC)/ACR Damage Index (SDI) scores, comorbidities and deaths were compared between the groups. Results: The JSLE patients showed statistically significant higher constitutional manifestations, cardiac manifestations, serositis, nephritis, end-stage renal disease, neurological manifestations, gastrointestinal manifestations, secondary vasculitis, Raynaud’s, livedo-reticularis, dry mouth, dry eye, ocular manifestations, avascular necrosis, hematological manifestations, and hypocomplementemia (p<0.001, p=0.016, p=0.005, p=0.001, p=0.04, p<0.001, p<0.001, p<0.001, p=0.002, p=0.043, p=0.004, p=0.03, p<0.001, p=0.01, p<0.001, and p=0.001, respectively). Median SLEDAI scores were statistically significant higher in the JSLE group, both at onset (p<0.001) and in the final follow-up visit (p<0.001). Median SLICC scores were also higher in the JSLE group (p<0.001). Mycophenolate mofetil and intravenous pulse steroids were more frequently used in the juvenile group (p<0.001 and p=0.03, respectively). Hypertension, dyslipidemia, and avascular necrosis were found to be statistically significantly higher in the JSLE group (p<0.001, p=0.006, and p=0.01, respectively). The mortality rate was statistically significantly higher in the JSLE group than the ASLE group (p<0.001). Conclusion: The JSLE patients showed more serious manifestations, higher disease activity, higher damage index, and mortality rate compared to ASLE patients. These results suggest the need of a regular follow-up and close surveillance of JSLE patients.

show abstract

Juvenile and juvenile-onset systemic lupus erythematosus patients: Clinical characteristics, disease activity and damage

Cited by 6 publications

References 27 publications

Clinical features, disease activity and outcomes of Malaysian children with paediatric systemic lupus erythematosus: A cohort from a tertiary centre

Clinical features, disease activity and outcomes of Malaysian children with paediatric systemic lupus erythematosus: A cohort from a tertiary centre

Study about Multisystem Autoimmune Disorder: Systemic Lupus Erythematosus, A Third World Prodigy

Disease characteristics in patients with juvenile- and adult-onset systemic lupus erythematosus: A multi-center comparative study

Contact Info

Product

Resources

About