Clinical and Immunological Spectrum of Systemic Lupus Erythematosus in Children

Abstract

Introduction: Systemic Lupus Erythematosus (SLE) is an auto immune disorder affecting mainly adolescent females and young women of reproductive age. The disease is characterised by widespread inflammation of blood vessels and connective tissues due to the presence of anti-nuclear antibodies (ANA). There are limited number of studies from South India on paediatric lupus. Our objectives were to study the clinical and immunological features of childhood SLE along with treatment modalities and its outcome at the end of one year follow up. The correlation between various auto-antibodies and systemic involvement was also assessed.
 Methods: This was a retrospective observational study carried out in paediatric unit at a tertiary care centre in South India. Data was obtained through patient’s medical records. From April 2003 to April 2019, 32 children were diagnosed to have SLE as per the American college of Rheumatology 1997 criteria.
 Results: The study population included 32 children fulfilling the criteria. Female to male ratio was 4.3:1. The mean age at diagnosis was 11.52 years. The most common clinical manifestations were renal (87.5%) followed by haematological (81.3%), musculoskeletal (59.4%), mucocutaneous (53.1%) and nervous system (31.3%) involvement. All patients were positive for anti-nuclear antibodies. Anti-double stranded DNA (78.1%) was the most common auto-antibody profile followed by anti-ribosomal p protein (37.5%) and anti-nucleosome antibody (37.5%). During the follow up, 13 (40.6%) children attained complete remission, 10 (31.2%) went into partial remission and nine (28.1%) had persisting active disease. 
 Conclusion: The clinical spectrum and outcome of paediatric SLE depends upon the age of presentation and number of organ systems involved at the time of diagnosis. Our study throws light on various aspects of SLE in children from developing countries like India.

Autoantibody Profile of Egyptian Juvenile Systemic Lupus Erythematosus Patients and Its Association with Clinical Characteristics and Disease Activity

“…The mean value of the age, at diagnosis, was 12.91±1.94 years in our study. These results were not different from those demonstrated by Kini et al 18 and Gomes et al 19…”

“…The mean value of the age, at diagnosis, was 12.91±1.94 years in our study. These results were not different from those demonstrated by Kini et al 18 and Gomes et al 19 Musculoskeletal manifestation were the most prevalent clinical characteristics found in among those patients, in the form of polyarthralgia (71%), followed by haematological manifestations (65%), malar rash (54%), nephritis (51%), and neuropsychiatric manifestations (27%). Similar results were obtained by different studies by Torrente-Segarra et al and Kosaraju et al who found that musculoskeletal manifestations were the most frequent clinical characteristics (77.9%, 64.8%) respectively.…”

“…25 In the opposite of our study, Kini et al observed that the different anti-ENA antibodies and the characteristic organ involvement did not have any association. 18 This difference in results may be attributed to the different ethnic backgrounds, geographical locations, age, and number of the study population.…”

Presentation and outcome of pediatric lupus nephritis from a large single centre contemporary cohort in Eastern India

“…One needs to be cautious in comparing studies as definition of treatment response and proteinuria in various previous studies has been variable. [18][19][20][21][22][23][24][25][26] Similar to the prospective German registry by Suhlrie et al 17 we quantified proteinuria as per 24 h estimation normalized to body surface area (BSA) and used the KDIGO 2021 definition for cut-off. 36 Others used spot urine protein creatinine ratio 26 or even a fixed cut-off without adjusting for age or size.…”

“…9 Due to the scarcity of published pediatric cohorts, especially from diverse ethnic and healthcare settings, [10][11][12][13][14][15][16][17] the management of childhood LN have been primarily extrapolated from mainly adult studies from high resource Western countries. Childhood LN may affect South Asian children differently but a literature search found only few representative cohorts with most of the studies reported from a bygone era (Supplementary Table 1) [18][19][20][21][22][23][24][25][26] when mycophenolate mofetil (MMF) and biologics were not commonly used.…”