Clinico-immunological profile and outcome of childhood systemic lupus erythematosus

Ramya Kadiyala, Pushpa Gurudas Kini, Shrikiran Aroor, Sandeep Kumar, Karen Moras

Research output: Contribution to journalArticle

Abstract

Introduction: Paediatric lupus is an autoimmune disorder most commonly affecting adolescent females. Various studies regarding paediatric lupus have been reported across the world. Objective: To study the clinical and immunological features of systemic lupus erythematosus (SLE) along with treatment modalities and the response at the end of one year follow up. Relationship between the types of autoantibodies and probability of systemic involvement is also assessed. Method: A prospective and retrospective observational study was carried out in the Paediatric Department of a tertiary care hospital from January 2010 to July 2016. Subjects included children from 1 month to 18 years of age fulfilling the 1997 American College of Rheumatology criteria for SLE. Results: Study population included 44 children fulfilling the criteria. Among them 10 were in the prospective group and 34 in the retrospective group. Female: male ratio was 4.5:1. Median age at diagnosis was 13.2 years (interquartile range 11.2- 14.6). Presenting features were constitutional in 86.4%, musculoskeletal in 72.7%, renal in 65.9%, haematological in 63.6%, muco-cutaneous in 61.3%, central nervous system in 36.3% and serositis in 25% children. Anaemia was the commonest haematological abnormality and was found in 75% of children. Hypocomplementaemia was seen in 100% of children. All subjects were positive for antinuclear antibodies. Anti-double stranded DNA (77.2%) was the most commonly observed autoantibody profile followed by antiribosomal P protein (47.7%) and antiribonucleoprotein (43.1%). During follow up of 36 children, 19 (52.7%) children attained complete remission, 8 (22.3%) went into partial remission and 9 (25%) had persisting active disease. During the study period 13 (29.5%) of 44 children succumbed either to the active disease process or to complication of SLE. Conclusions: The clinical presentation and course of progression of the disease varies depending on the age of onset and the organ system involved. Low complement levels indicate activation of the disease especially lupus nephritis.

Original languageEnglish
Pages (from-to)201-207
Number of pages7
JournalSri Lanka Journal of Child Health
Volume48
Issue number3
DOIs
Publication statusPublished - 01-01-2019

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Systemic Lupus Erythematosus
Pediatrics
Autoantibodies
Serositis
Lupus Nephritis
Antinuclear Antibodies
Tertiary Healthcare
Age of Onset
Tertiary Care Centers
Observational Studies
Disease Progression
Anemia
Central Nervous System
Retrospective Studies
Kidney
Skin
DNA
Population
Proteins

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health

Cite this

@article{6b981b75c46f44f280fad49badde4172,
title = "Clinico-immunological profile and outcome of childhood systemic lupus erythematosus",
abstract = "Introduction: Paediatric lupus is an autoimmune disorder most commonly affecting adolescent females. Various studies regarding paediatric lupus have been reported across the world. Objective: To study the clinical and immunological features of systemic lupus erythematosus (SLE) along with treatment modalities and the response at the end of one year follow up. Relationship between the types of autoantibodies and probability of systemic involvement is also assessed. Method: A prospective and retrospective observational study was carried out in the Paediatric Department of a tertiary care hospital from January 2010 to July 2016. Subjects included children from 1 month to 18 years of age fulfilling the 1997 American College of Rheumatology criteria for SLE. Results: Study population included 44 children fulfilling the criteria. Among them 10 were in the prospective group and 34 in the retrospective group. Female: male ratio was 4.5:1. Median age at diagnosis was 13.2 years (interquartile range 11.2- 14.6). Presenting features were constitutional in 86.4{\%}, musculoskeletal in 72.7{\%}, renal in 65.9{\%}, haematological in 63.6{\%}, muco-cutaneous in 61.3{\%}, central nervous system in 36.3{\%} and serositis in 25{\%} children. Anaemia was the commonest haematological abnormality and was found in 75{\%} of children. Hypocomplementaemia was seen in 100{\%} of children. All subjects were positive for antinuclear antibodies. Anti-double stranded DNA (77.2{\%}) was the most commonly observed autoantibody profile followed by antiribosomal P protein (47.7{\%}) and antiribonucleoprotein (43.1{\%}). During follow up of 36 children, 19 (52.7{\%}) children attained complete remission, 8 (22.3{\%}) went into partial remission and 9 (25{\%}) had persisting active disease. During the study period 13 (29.5{\%}) of 44 children succumbed either to the active disease process or to complication of SLE. Conclusions: The clinical presentation and course of progression of the disease varies depending on the age of onset and the organ system involved. Low complement levels indicate activation of the disease especially lupus nephritis.",
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Clinico-immunological profile and outcome of childhood systemic lupus erythematosus. / Kadiyala, Ramya; Kini, Pushpa Gurudas; Aroor, Shrikiran; Kumar, Sandeep; Moras, Karen.

In: Sri Lanka Journal of Child Health, Vol. 48, No. 3, 01.01.2019, p. 201-207.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Clinico-immunological profile and outcome of childhood systemic lupus erythematosus

AU - Kadiyala, Ramya

AU - Kini, Pushpa Gurudas

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AU - Kumar, Sandeep

AU - Moras, Karen

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N2 - Introduction: Paediatric lupus is an autoimmune disorder most commonly affecting adolescent females. Various studies regarding paediatric lupus have been reported across the world. Objective: To study the clinical and immunological features of systemic lupus erythematosus (SLE) along with treatment modalities and the response at the end of one year follow up. Relationship between the types of autoantibodies and probability of systemic involvement is also assessed. Method: A prospective and retrospective observational study was carried out in the Paediatric Department of a tertiary care hospital from January 2010 to July 2016. Subjects included children from 1 month to 18 years of age fulfilling the 1997 American College of Rheumatology criteria for SLE. Results: Study population included 44 children fulfilling the criteria. Among them 10 were in the prospective group and 34 in the retrospective group. Female: male ratio was 4.5:1. Median age at diagnosis was 13.2 years (interquartile range 11.2- 14.6). Presenting features were constitutional in 86.4%, musculoskeletal in 72.7%, renal in 65.9%, haematological in 63.6%, muco-cutaneous in 61.3%, central nervous system in 36.3% and serositis in 25% children. Anaemia was the commonest haematological abnormality and was found in 75% of children. Hypocomplementaemia was seen in 100% of children. All subjects were positive for antinuclear antibodies. Anti-double stranded DNA (77.2%) was the most commonly observed autoantibody profile followed by antiribosomal P protein (47.7%) and antiribonucleoprotein (43.1%). During follow up of 36 children, 19 (52.7%) children attained complete remission, 8 (22.3%) went into partial remission and 9 (25%) had persisting active disease. During the study period 13 (29.5%) of 44 children succumbed either to the active disease process or to complication of SLE. Conclusions: The clinical presentation and course of progression of the disease varies depending on the age of onset and the organ system involved. Low complement levels indicate activation of the disease especially lupus nephritis.

AB - Introduction: Paediatric lupus is an autoimmune disorder most commonly affecting adolescent females. Various studies regarding paediatric lupus have been reported across the world. Objective: To study the clinical and immunological features of systemic lupus erythematosus (SLE) along with treatment modalities and the response at the end of one year follow up. Relationship between the types of autoantibodies and probability of systemic involvement is also assessed. Method: A prospective and retrospective observational study was carried out in the Paediatric Department of a tertiary care hospital from January 2010 to July 2016. Subjects included children from 1 month to 18 years of age fulfilling the 1997 American College of Rheumatology criteria for SLE. Results: Study population included 44 children fulfilling the criteria. Among them 10 were in the prospective group and 34 in the retrospective group. Female: male ratio was 4.5:1. Median age at diagnosis was 13.2 years (interquartile range 11.2- 14.6). Presenting features were constitutional in 86.4%, musculoskeletal in 72.7%, renal in 65.9%, haematological in 63.6%, muco-cutaneous in 61.3%, central nervous system in 36.3% and serositis in 25% children. Anaemia was the commonest haematological abnormality and was found in 75% of children. Hypocomplementaemia was seen in 100% of children. All subjects were positive for antinuclear antibodies. Anti-double stranded DNA (77.2%) was the most commonly observed autoantibody profile followed by antiribosomal P protein (47.7%) and antiribonucleoprotein (43.1%). During follow up of 36 children, 19 (52.7%) children attained complete remission, 8 (22.3%) went into partial remission and 9 (25%) had persisting active disease. During the study period 13 (29.5%) of 44 children succumbed either to the active disease process or to complication of SLE. Conclusions: The clinical presentation and course of progression of the disease varies depending on the age of onset and the organ system involved. Low complement levels indicate activation of the disease especially lupus nephritis.

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