Childhood lupus nephritis: 12 years of experience from a developing country’s perspective

Abstract

Objective: To assess the long-term outcome of lupus nephritis in children with systemic lupus erythematosus followed up over 12 years at a tertiary care teaching hospital in Eastern India. Material and Methods:This is a retrospective observational study of the clinicopathological presentation, management, and outcome in 46 children with lupus nephritis over a period of 12 years at a tertiary teaching hospital in Eastern India. Mortality was compared between different lupus classes and therapy groups with Kaplan-Meier analysis and log-rank testResults: The incidence of lupus nephritis was 58.97% [95% confidence interval (CI) 48.06%-59.89%] with the mean age at presentation being 10.2±2.43 years (range 5.5-14.5) years. Majority belonged to class IV (30.43%), followed by class II (26.91%), class III (23.91), and class V (8.70%). Outcome analysis of children with lupus nephritis over 12 years revealed that 24 (52.17%) achieved complete remission of disease activity, 5 attained partial remission, 4 continued to have active disease, 5 developed end-stage renal disease (ESRD), and 8 died. Overall mortality thus observed was 17.39% with septicemia in the background of ESRD being the commonest cause. No significant difference in mortality was observed between different lupus nephritis classes or therapy arm groups. Conclusion:The study throws light on various aspects of lupus nephritis and their long-term outcome patterns in children from developing countries such as India.

Improving musculoskeletal health for children and young people – A ‘call to action’

“…Juvenile systemic lupus erythematosus (JSLE) is the most common connective disease in children and is life-threatening with no cure. Prevalence and incidence rates of JSLE (3.3–24 per 100,000 children and 0.3–0.9 per 100, 000 children per year, respectively) vary around the world [ 72 ] and JSLE is reported to be more common and more severe in Asia, SEA, Latin America and Africa [ [73] , [74] , [75] , [76] , [77] ] compared with the USA and Europe [ 72 , 78 ]. Such variation may be attributed to genetic background, ethnicity as well as ascertainment and selection bias.…”

“…Diagnosis can be challenging where infections (such as TB) are often mimics of rheumatic diseases and diagnostic capabilities (including availability of autoantibody tests) are often limited in low resource settings [ 5 , 95 ]. Delayed diagnosis and access to specialist care in JSLE with worse clinical outcomes is widely reported across the world [ 79 , 88 , [96] , [97] , [98] ], often associated with more severe disease activity and organ damage at presentation [ 75 , 99 , 100 ]. Many of the innovations that have improved survival in HRIC remain unavailable in most of the world [ 77 ] and access to renal replacement therapy may be severely limited or non-existent [ 101 ].…”

Clinical and Immunological Spectrum of Systemic Lupus Erythematosus in Children

“…The presentation is highly variable marked by periods of flare and remission with significant morbidity and mortality. 1 The peak incidence is around 10-14 years with a male to female ratio of 3:4 before puberty and 1:4 after puberty. SLE is rare in children under five years and the disease has a higher risk of mortality and morbidity compared to adults.…”

Pattern of Indirect Immunofluorescence Assay Antinuclear Antibody in Pediatric Lupus Nephritis

“…ANA is autoantibodies which formed by the immune system that can attack self-antigen and cause inflammation in many tissues and organs. 1,2,3,4,5 ANA test is a primary test to determines the presence of ANA. Despite this, ACR 1997 recommends Immunoflourecence Assay (IFA) method as a gold standard of ANA test, because it can help the clinician to diagnose the patient more specifically and reduce risk of false positive or errorneous based on the results of the test.…”

“…The autoantibody also known as Anti-Nuclear Antibody (ANA) which have many subtypes that can attack self-antigens and cause inflammation in several organs, such as skin, joints, kidneys, blood-forming cells, blood vessels, and central nervous system. 1,2,3,4 ANA test is one of tests that used for helping diagnose autoimmune three major categories, namely nuclear (such as homogeneous, speckled, centromere, nucleardots and nucleolar), cytoplasmic and mitotic patterns. The results of the titer and ANA pattern will be varied in each patients.…”