DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20201552

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

Shashidhara V. S., Anil Kumar H., Mallesh Kariyappa

Abstract


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.

Keywords


Auto antibodies, Lupus nephritis, Systemic lupus erythematosus

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References


Petty RE, Laxer RM. Systemic lupus erythematosus: In: Cassidy JT, Petty RE, Laxer ML, Lindsley CB, Eds. Textbook of Pediatric Rheumatology, 5th edn. Philadelphia: Elsevier Saunders; 2005:342-391.

Hiraki LT, Benseler SM, Tyrrell PN, Hebert D, Harvey E, Silverman ED. Clinical and laboratory characteristics and long-term outcome of pediatric systemic lupus erythematous: a longitudinal study. J Pediatr. 152:2008:550-6.

Schur PH. Clinical features of systemic lupus erythematosus. In: Textbook of Rheumatology, 4th edn. Eds. Kelly WN, Harris ED, Ruddy S, Sledge CB. Philadelphia, W.B. Saunders Company;1993:1017-1042.

Rebecca E. Sadun, stacy p.ardoin, and laura E. Schanberg. Systemic lupus erythematosus: Robert M Kleigmann. Nelson text book of pediatrics: first south asia ed; 2016:1176-1180.

Desai SC, Bhandarkar SD. A case report of acute disseminated lupus erythematosus. Indian J Dermatol Venereol. 1955;21:131-4.

Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus Erythematosus, Arthritis Rheum. 1997:40:1725.

WHO. Haemoglobin concentrations for the diagnosis of Anaemia and assessment of severity. Vitamin and Mineral Nutrition Information System. Geneva, World Health Organization, 2011. Available at: https://www.who.int/vmnis/indicators/haemoglobin/en/.

Singh S, Kumar L, Khetarpal R, Aggarwal P, Marwaha RK, Minz RW et al. Clinical and immunological profile of SLE: Some unusual features: Indian Pediatr. 1997:34.

Yadav V, Bhardwaj P. Clinical and immunological profile of systemic lupus Erythematosus in a pediatric population. North India Egyptian Rheumatol Rehabilit. 2014;41:148-51.

Youssef DM, Tawfek DM, Mohammed AM, Mohammed R, Khalifa NA. Pediatric systemic lupus erythematosus in a single nephrology unit. Saudi Journal of Kidney Diseases and Transplantation. 2015;26(2):314.

Carien B Gulay, Leonila F Dans. Clinical presentations and outcomes of Filipino juvenile systemic lupus erythematosus. Pediatr Rheumatol. 2011;9:7.

Uthman IW, Muffarij AA, Mudawar WA, Nasr FW, Masri AF. Lupus nephritis in Lebanon. Lupus. 2001;10:378-81.

Nazri SKSM, Wong KK. Hamid WZWA. Pediatric systemic lupus erythematosus. Retrospective analysis of clinico-laboratory Parameters and their association with Systemic Lupus Erythematosus Disease Activity Index score: Saudi Med J. 2018;39(6):627-31.

Perfumo F, Martini A. Lupus nephritis in children. Lupus. 2005;14:83-8.

Agarwal I, Kumar TS, Ranjini K, Kirubakaran C, Danda D. Clinical features and outcome of systemic lupus erythematosus. Indian Pediatr. 2009;46(8).

Ali US, Dalvi RB, Merchant RH, Mehta KP, Chablani AT, Badakere SS, et al. Systemic lupus erythematosus in Indian children. Indian Pediatr. 1989;26:868-73.

Ilias MI, Ali JM, Ismail NZ, Rostenberghe HV, Rahman AA. Pediatric Systemic Lupus Erythematosus (SLE) Manifestations and Outcomes in a Tertiary Hospital. Lupus. 2017;2:123.

Dubois EL, Tuffanelli DL. Clinical manifestations of SLE. Computer analysis of 520 cases. J Am Med Assoc. 1964;190:104-11.

Madhavan R. SLE - The Madras Experience. J Assoc Phys India 1988;36:481-4.

Paul BJ, Fassaludeen M, Nandakumar, Razia MV. Clinical Profile of Systemic Lupus Erythematosus In Northern Kerala. J Indian Rheumatol Assoc. 2003:11:94-7.

Vaidya S, Samant RS, Nadkar MY, Borges NE. Systemic lupus erythematosus-review of two hundred and twenty patients. JIRA. 1997;5:14-8.

Mondal R, Nandi M, Ganguli S, Ghosh A, Hazra A. Childhood lupus: experience from eastern India. Indian Pediatr. 2010;77:889-91.

Pradhan SV, Patwardhan M, Rajadhyaksha A, Ghosh K. Clinical and immunological profile of systemic lupus erythematosus. Indian Pediatr. 2013:50:405-7.

Chandrashekran AN, Rajendran CP, Ramakrishan S, Madhavan R, Pratibhan M. Childhood systemic lupus Erythematosus in South India. Indian J Pediatr. 1994;61:223-9.