Pediatric systemic lupus erythematosus

Nazri, Siti Khadijah S.M.; Wong, Kah Keng; Hamid, Wan Zuraida Wan Abdul

doi:10.15537/smj.2018.6.22112

Cited by 8 publications

(9 citation statements)

References 13 publications

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“…[8][9][10] The female to male ratio in this series was much lower than other studies but concordance with study by Siti Khadhija et, al. [11][12][13] The most common clinical manifestation was haematological with anaemia in 92% cases, haemolytic anaemia was found in 33% of cases similar to other studies. [8][9][10] The second most common features was renal manifestation seen in 88% of the cases similar to observations made by Perfumo F et al, Indira Agarwal et.al, 77.1%.…”

Section: Discussionsupporting

confidence: 77%

Study of paediatric systemic lupus erythematosus in tertiary care center in South India

Shashidhara

Kumar

Kariyappa

2020

Int J Contemp Pediatr

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Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem inflammation and the presence of circulating autoantibodies directed against self-antigens. The reported prevalence of SLE in children and adolescents (1-6/100,000) is lower than that in adults (20-70/100,000). The study among pediatric population were few and hence this study from south India was undertaken. Objective of this study was to study the clinical and immunological profile of children with systemic lupus erythematosus (SLE). To study the Distribution of Renal Lesions according to ISN/RPS Classification of Lupus Nephritis Methods: Retrospective hospital based observational study in tertiary level centre in Bengaluru (BMCRI). Medical records of children with SLE admitted in Pediatric department from the period of 2010-2019 through the hospital information system were analyzed. Clinico pathological features and immunological profile were compared with other studies.Results: Among 25 patients studied male to female ratio was1:2.5. The mean patient’s age at the time of presentation was13.2 year, the youngest child being 7 year. The mean duration of disease before diagnosis was 1year, most common systems involved were haematological (92%), followed by kidney (88%), GIT (72%), mucocutaneous (68%) cases. 19 (91%) cases were ANA positive and two ANA negatives. Anti-ds DNA was positive in 18(85%) patients, 5 were anti smith antibody positive. Diffuse proliferative glomerulonephritis (ISN/RPS class IV) was the most commonly seen histological pattern, seen in 9(56%) patients, 4(25%) patient had focal and segmental proliferative glomerulonephritis (ISN/RPS class III) and 2(12%) had membranous glomerulonephritis (grade ISN/RPS class V).Conclusions: SLE can present with diverse, unpredictable clinical manifestations, the primary diagnosis can often be missed if the index of suspicion is not high, since childhood SLE does not present with classical manifestations.

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Section: Discussionsupporting

confidence: 77%

Study of paediatric systemic lupus erythematosus in tertiary care center in South India

Shashidhara

Kumar

Kariyappa

2020

Int J Contemp Pediatr

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“…Tuy nhiên kết quả của chúng tôi cao hơn với nghiên cứu củaPusongchai [11] trên trẻ em mắc Lupus tại Thái lan ( 14,9+ 2). Trong khi đó nghiên cứu của Shamim [5] tại Mỹ số trẻ điểm SLEDAI > 10 chiếm 87%, cao hơn nhiều so với Nazri [7] tại Malaysia điểm SLEDAI > 6 chỉ chiếm 57%. Sự khác biệt này có thể do quần thể cỡ mẫu nghiên cứu khác nhau.…”

Section: Bàn Luậnunclassified

Assessment of SLEDAI Score in Children with Lupus Nephritis Class III-IV in Vietnam National Children’s Hospital

Binh¹,

Hương²,

Kien³

et al. 2020

MPS

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This study describes clinical, paraclinical characteristics and treatment response in children with nephritis class II-IV caused by systemic lupus erythematosus and validates SLEDAI for the evaluation of disease activity and the appropriate treatment strategy. A cross-sectional descriptive study was carried out on 40 children, 37 girls (92%) and 3 boys (8%), with an average age of 11.7 years with lupus nephritis class III- IV in Vietnam National Children’s Hospital in 2019. The study results show that the average score of SLEDAI in the children with pericardial and pleural effusions was 20.94 ± 4.09; high blood pressure, 20.89 ± 4.23; and gross hematuria, 20.29 ± 5.03, which were higher than those in children without these manifestations with p< 0.05. The most common kidney manifestations were nephrotic-range nephritis with renal failure (40%) and Glomerulonephritis (35%), corresponding to an average SLEDAI score of 24.25 ± 5.52 and 24.33 ± 3.2, respectively (p = 0.001). SLEDAI had an inverse correlation with the C3 complement value (r -0.315, p <0.05). The average SLEDAI score decreased gradually from 18.75 ± 4.22 to 3.38 ± 3.95 points (p <0.001) after 12 months of treatment. The study concludes that SLEDAI score was higher in patients with pleural and/or pericardial effusions, hypertension and gross hematuria, nephrotic-range nephritis with kidney failure or glomerulonephritis. SLEDAI score corresponded with the C3 complement value and the average SLEDAI score decreased gradually with treatment. Keywords: Lupus Nephritis class III- IV, SLEDAI. References [1] George Bertsias, Ricard Cervera và Dimitrios T Boumpas, Systemic Lupus Erythematosus: Pathogenesis and Clinical Features<sample chapter 20_mod 17_Systemic Lupus nephritis 2012.pdf> (2012), EULAR Textbook on Rheumatic Diseases, EULAR, 476-505.[2] D.M. Levy and S. Kamphuis, Systemic lupus erythematosus in children and adolescents. Pediatr Clin North Am59(2) (2012)345-64.[3] Thai Thien Nam, 2018, Lupus in National Children,s Hospital, [4] C.Bombardier, M.B. Hurwitz et al, Derivation of the SLEDAI: A disease activity index for lupus patients. The committee on prognosis studies in SLE, Arthritis Rheum 35(6) (1992) 630-640.[5] R. Shamim, S. Farman, S. Batool et al, Association of systemic lupus erythematosus disease activity index score with clinical and laboratory parameters in pediatric onset systemic lupus erythematosus. Pak J Med Sci. 36(3) (2020) 467-472.[6] Le Thuy Hang, Assesment of SLEDAI score and panthology in children with lupus nephritis, 2016, Pediatrician thesis, Hanoi Medical University.[7] S.K.S.M. Nazri, K.K. Wong and W.Z.W.A. Hamid, Pediatric systemic lupus erythematosus. Retrospective analysis of clinico-laboratory parameters and their association with Systemic Lupus Erythematosus Disease Activity Index score, Saudi Med J. 39(6) (2018) 627-631. [8] Nguyen Thuy Duong, clinical, paraclinical and pathology characteristics in children with nephritis caused by systemic lupus erythematosus, 2011, Master thesis, Hanoi Medical University.[9] S.N. Wong, W.K. Chan, J.Hui et al, Membranous lupus nephritis in Chinese children--a case series and review of the literature. Pediatr Nephrol, 24(10)(2009) 1989-1996.[10] N.T.N. Dung, H.T. Loan, S. Nielsen et al, Juvenile systemic lupus erythematosus onset patterns in Vietnamese children: a descriptive study of 45 children. Pediatric Rheumatology Online Journal, 10 (2010) 38-48.[11] T. Pusongchai, J. Jungthirapanich, S. Khositseth, Pediatric Systemic Lupus Erythematosus in Thammasat University Hospital, J Med Assoc Thai. 93(12) (2010) 283-290.

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“…6,7 Long-term monitoring was recommended to evaluate the disease activity and adverse effect therapy, with 3-6 month assessment for mild disease and more frequent in patients with severe active disease . [7][8][9][10] Disease activity cannot be determined by a single clinical sign or laboratory. Different measures have been developed to assess SLE disease activity including the systemic lupus activity measure (SLAM), SLE disease activity index (SLEDAI), the European consensus lupus activity measurement (ECLAM), and the British isles lupus assessment group (BILAG).…”

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confidence: 99%

Efficacy of high-dose methylprednisolone and cyclophosphamide in childhood-onset systemic lupus erythematosus

Trisnia

Wati²,

Witarini³

et al. 2020

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Background Systemic lupus erythematosus (SLE) is a chronic, multisystem, autoimmune disease. Untreated SLE often become progressive and lead to increased risk of mortality. Corticosteroid and cyclophosphamide remain the treatment of choice for severe SLE. Disease activity assessed with SLE Daily Activity Index (SLEDAI). Objective To compare the disease activity of childhood-onset severe SLE at the time of diagnosis, after completion of high dose methylprednisolone, and after three month of cyclophosphamide by using SLEDAI. Methods This study was conducted in the Division of Pediatric Allergy and Immunology, Department of Child Health, Udayana University/Sanglah Hospital, Denpasar, Bali. Subjects were SLE patient aged 0-18 years who had severe clinical manifestations. Subject received therapy combination of high dose methylprednisolone and cyclophosphamide every 2 weeks for six doses. SLEDAI score was assessed at the time of diagnosis, after completion of high dose methylprednisolone, and after three month of cyclophosphamide. Results During the study period, 51 children were diagnosed as SLE. Twenty-one subjects were included for analysis. Median SLEDAI score at the time of diagnosis was 23 (range 13-39). SLEDAI score after three months of cyclophosphamide was decreased to 2 (range 0-14). Post hoc analysis with Wilcoxon signed-rank test showed the improvement of SLEDAI score at the time of diagnosis and after three months of cyclophosphamide was statistically significant (Z=-4.016, P<0.0001). Conclusion SLEDAI score reduced after completion of high-dose methylprednisolone and three month of cyclophosphamide therapy.

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Pediatric systemic lupus erythematosus

Cited by 8 publications

References 13 publications

Study of paediatric systemic lupus erythematosus in tertiary care center in South India

Study of paediatric systemic lupus erythematosus in tertiary care center in South India

Assessment of SLEDAI Score in Children with Lupus Nephritis Class III-IV in Vietnam National Children’s Hospital

Efficacy of high-dose methylprednisolone and cyclophosphamide in childhood-onset systemic lupus erythematosus

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