Skip to main content
Top
Published in: Pediatric Rheumatology 1/2012

Open Access 01-12-2012 | Research

Differential manifestations of prepubescent, pubescent and postpubescent pediatric patients with systemic lupus erythematosus: A retrospective study of 96 Chinese children and adolescents

Authors: Li-Lan Chiang, Yu-Tsan Lin, Hung-Yi Chan, Bor-Luen Chiang

Published in: Pediatric Rheumatology | Issue 1/2012

Login to get access

Abstract

Background

Children represent 10-20% of all systemic lupus erythematosus (SLE) patients. Their clinical manifestations and outcomes vary with age. We aim to clarify the relationship between pubescent status and the clinical manifestations of pediatric SLE.

Methods

In this study, pediatric SLE patients were divided into three groups, based on age at disease onset (≦8, 8–13 & 13–18 years), defined as prepubescent, pubescent and postpubescent, respectively. Initial clinical manifestations and laboratory characteristics at diagnosis were analyzed.

Results

Ninety-six patients were entered into the study: 8 had disease onset before age 8, while 49 were between 8–13 and 39 of them were 13–18. Female predominance was noted in all three groups (2.5-7.0:1). Postpubescents showed significantly more renal involvement and lymphopenia, along with lower levels of C3 and C4, when compared with prepubescents. They also showed significantly more lymphopenia when compared with pubescents. Pubescents showed significantly more renal involvement, leukopenia and lupus anticoagulant (LAC) positivity, along with lower C3 and C4 levels, when compared with prepubescents. Pubescents also showed significantly higher anti-Sm antibody positivity when compared with postpubescents. Prepubescents showed significantly more splenomegaly and anti-Jo-1 antibody positivity when compared with those of pubescents. The results showed that the disease activity (SLEDAI-2K score) correlated positively with age at disease onset and negatively with disease duration before diagnosis (p = 0.011).

Conclusions

Age at disease onset is related to initial manifestations in pediatric SLE patients at our center. Certain parameters such as renal involvement, splenomegaly, low C3 level, low C4 level, lymphopenia, leukopenia, and anti-Sm & anti-Jo-1 antibody were found to be significantly different among the age groups. Renal involvement might be the key symptom that varies with age.
Appendix
Available only for authorised users
Literature
1.
go back to reference Carreno L, Lopez-Longo FJ, Monteagudo I, Rodriguez-Mahou M, Bascones M, Gonzalez CM, Saint-Cyr C, Lapointe N: Immunological and clinical differences between juvenile and adult onset of systemic lupus erythematosus. Lupus. 1999, 8: 287-292.CrossRefPubMed Carreno L, Lopez-Longo FJ, Monteagudo I, Rodriguez-Mahou M, Bascones M, Gonzalez CM, Saint-Cyr C, Lapointe N: Immunological and clinical differences between juvenile and adult onset of systemic lupus erythematosus. Lupus. 1999, 8: 287-292.CrossRefPubMed
2.
go back to reference Cook CD, Wedgwood RJ, Craig JM, Hartmann JR, Janeway CA: Systemic lupus erythematosus. Description of 37 cases in children and a discussion of endocrine therapy in 32 of the cases. Pediatrics. 1960, 26: 570-585.PubMed Cook CD, Wedgwood RJ, Craig JM, Hartmann JR, Janeway CA: Systemic lupus erythematosus. Description of 37 cases in children and a discussion of endocrine therapy in 32 of the cases. Pediatrics. 1960, 26: 570-585.PubMed
3.
go back to reference Costallat LT, Coimbra AM: Systemic lupus erythematosus: clinical and laboratory aspects related to age at disease onset. Clin Exp Rheumatol. 1994, 12: 603-607.PubMed Costallat LT, Coimbra AM: Systemic lupus erythematosus: clinical and laboratory aspects related to age at disease onset. Clin Exp Rheumatol. 1994, 12: 603-607.PubMed
4.
go back to reference Harvey AM, Shulman LE, Tumulty PA, Conley CL, Schoenrich EH: Systemic lupus erythematosus: review of the literature and clinical analysis of 138 cases. Medicine (Baltimore). 1954, 33: 291-437.CrossRef Harvey AM, Shulman LE, Tumulty PA, Conley CL, Schoenrich EH: Systemic lupus erythematosus: review of the literature and clinical analysis of 138 cases. Medicine (Baltimore). 1954, 33: 291-437.CrossRef
5.
go back to reference Macdermott EJ, Adams A, Lehman TJ: Systemic lupus erythematosus in children: current and emerging therapies. Lupus. 2007, 16: 677-683.CrossRefPubMed Macdermott EJ, Adams A, Lehman TJ: Systemic lupus erythematosus in children: current and emerging therapies. Lupus. 2007, 16: 677-683.CrossRefPubMed
6.
go back to reference Stichweh D, Arce E, Pascual V: Update on pediatric systemic lupus erythematosus. Curr Opin Rheumatol. 2004, 16: 577-587.CrossRefPubMed Stichweh D, Arce E, Pascual V: Update on pediatric systemic lupus erythematosus. Curr Opin Rheumatol. 2004, 16: 577-587.CrossRefPubMed
7.
go back to reference Tucker LB, Menon S, Schaller JG, Isenberg DA: Adult- and childhood-onset systemic lupus erythematosus: a comparison of onset, clinical features, serology, and outcome. Br J Rheumatol. 1995, 34: 866-872.CrossRefPubMed Tucker LB, Menon S, Schaller JG, Isenberg DA: Adult- and childhood-onset systemic lupus erythematosus: a comparison of onset, clinical features, serology, and outcome. Br J Rheumatol. 1995, 34: 866-872.CrossRefPubMed
8.
go back to reference Lacks S, White P: Morbidity associated with childhood systemic lupus erythematosus. J Rheumatol. 1990, 17: 941-945.PubMed Lacks S, White P: Morbidity associated with childhood systemic lupus erythematosus. J Rheumatol. 1990, 17: 941-945.PubMed
9.
go back to reference Lehman TJ, McCurdy DK, Bernstein BH, King KK, Hanson V: Systemic lupus erythematosus in the first decade of life. Pediatrics. 1989, 83: 235-239.PubMed Lehman TJ, McCurdy DK, Bernstein BH, King KK, Hanson V: Systemic lupus erythematosus in the first decade of life. Pediatrics. 1989, 83: 235-239.PubMed
10.
go back to reference Mok CC, Mak A, Chu WP, To CH, Wong SN: Long-term survival of southern Chinese patients with systemic lupus erythematosus: a prospective study of all age-groups. Medicine (Baltimore). 2005, 84: 218-224.CrossRef Mok CC, Mak A, Chu WP, To CH, Wong SN: Long-term survival of southern Chinese patients with systemic lupus erythematosus: a prospective study of all age-groups. Medicine (Baltimore). 2005, 84: 218-224.CrossRef
11.
go back to reference Rood MJ, ten Cate R, van Suijlekom-Smit LW, den Ouden EJ, Ouwerkerk FE, Breedveld FC, Huizinga TW: Childhood-onset Systemic Lupus Erythematosus: clinical presentation and prognosis in 31 patients. Scand J Rheumatol. 1999, 28: 222-226.CrossRefPubMed Rood MJ, ten Cate R, van Suijlekom-Smit LW, den Ouden EJ, Ouwerkerk FE, Breedveld FC, Huizinga TW: Childhood-onset Systemic Lupus Erythematosus: clinical presentation and prognosis in 31 patients. Scand J Rheumatol. 1999, 28: 222-226.CrossRefPubMed
12.
go back to reference Wang LC, Yang YH, Lu MY, Chiang BL: Retrospective analysis of the renal outcome of pediatric lupus nephritis. Clin Rheumatol. 2004, 23: 318-323.CrossRefPubMed Wang LC, Yang YH, Lu MY, Chiang BL: Retrospective analysis of the renal outcome of pediatric lupus nephritis. Clin Rheumatol. 2004, 23: 318-323.CrossRefPubMed
13.
go back to reference Parsa A, Peden E, Lum RF, Seligman VA, Olson JL, Li H, Seldin MF, Criswell LA: Association of angiotensin-converting enzyme polymorphisms with systemic lupus erythematosus and nephritis: analysis of 644 SLE families. Genes Immun. 2002, 3 (Suppl 1): S42-46.CrossRefPubMed Parsa A, Peden E, Lum RF, Seligman VA, Olson JL, Li H, Seldin MF, Criswell LA: Association of angiotensin-converting enzyme polymorphisms with systemic lupus erythematosus and nephritis: analysis of 644 SLE families. Genes Immun. 2002, 3 (Suppl 1): S42-46.CrossRefPubMed
14.
15.
16.
go back to reference Wandstrat A, Wakeland E: The genetics of complex autoimmune diseases: non-MHC susceptibility genes. Nat Immunol. 2001, 2: 802-809.CrossRefPubMed Wandstrat A, Wakeland E: The genetics of complex autoimmune diseases: non-MHC susceptibility genes. Nat Immunol. 2001, 2: 802-809.CrossRefPubMed
17.
go back to reference Zulian F, Pluchinotta F, Martini G, Da Dalt L, Zacchello G: Severe clinical course of systemic lupus erythematosus in the first year of life. Lupus. 2008, 17: 780-786.CrossRefPubMed Zulian F, Pluchinotta F, Martini G, Da Dalt L, Zacchello G: Severe clinical course of systemic lupus erythematosus in the first year of life. Lupus. 2008, 17: 780-786.CrossRefPubMed
18.
go back to reference Pluchinotta FR, Schiavo B, Vittadello F, Martini G, Perilongo G, Zulian F: Distinctive clinical features of pediatric systemic lupus erythematosus in three different age classes. Lupus. 2007, 16: 550-555.CrossRefPubMed Pluchinotta FR, Schiavo B, Vittadello F, Martini G, Perilongo G, Zulian F: Distinctive clinical features of pediatric systemic lupus erythematosus in three different age classes. Lupus. 2007, 16: 550-555.CrossRefPubMed
19.
go back to reference Mondal R, Nandi M, Ganguli S, Ghosh A, Hazra A: Childhood lupus: experience from Eastern India. Indian J Pediatr. 2010 Mondal R, Nandi M, Ganguli S, Ghosh A, Hazra A: Childhood lupus: experience from Eastern India. Indian J Pediatr. 2010
20.
go back to reference Al-Mayouf SM, Al Sonbul A: Influence of gender and age of onset on the outcome in children with systemic lupus erythematosus. Clin Rheumatol. 2008, 27: 1159-1162.CrossRefPubMed Al-Mayouf SM, Al Sonbul A: Influence of gender and age of onset on the outcome in children with systemic lupus erythematosus. Clin Rheumatol. 2008, 27: 1159-1162.CrossRefPubMed
21.
go back to reference Hochberg MC: Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997, 40: 1725-CrossRefPubMed Hochberg MC: Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997, 40: 1725-CrossRefPubMed
22.
go back to reference Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, Schaller JG, Talal N, Winchester RJ: The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982, 25: 1271-1277.CrossRefPubMed Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, Schaller JG, Talal N, Winchester RJ: The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982, 25: 1271-1277.CrossRefPubMed
23.
go back to reference Lai EH, Chang JZ, Jane Yao CC, Tsai SJ, Liu JP, Chen YJ, Lin CP: Relationship between age at menarche and skeletal maturation stages in Taiwanese female orthodontic patients. J Formos Med Assoc. 2008, 107: 527-532.CrossRefPubMed Lai EH, Chang JZ, Jane Yao CC, Tsai SJ, Liu JP, Chen YJ, Lin CP: Relationship between age at menarche and skeletal maturation stages in Taiwanese female orthodontic patients. J Formos Med Assoc. 2008, 107: 527-532.CrossRefPubMed
24.
go back to reference Xin X, Luan X, Xiao J, Wei D, Wang J, Lu D, Yang S: Association study of four activity SNPs of CYP3A4 with the precocious puberty in Chinese girls. Neurosci Lett. 2005, 381: 284-288.CrossRefPubMed Xin X, Luan X, Xiao J, Wei D, Wang J, Lu D, Yang S: Association study of four activity SNPs of CYP3A4 with the precocious puberty in Chinese girls. Neurosci Lett. 2005, 381: 284-288.CrossRefPubMed
25.
go back to reference Gladman DD, Ibanez D, Urowitz MB: Systemic lupus erythematosus disease activity index 2000. J Rheumatol. 2002, 29: 288-291.PubMed Gladman DD, Ibanez D, Urowitz MB: Systemic lupus erythematosus disease activity index 2000. J Rheumatol. 2002, 29: 288-291.PubMed
26.
go back to reference Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F: The classification of glomerulonephritis in systemic lupus erythematosus revisited. J Am Soc Nephrol. 2004, 15: 241-250.CrossRefPubMed Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F: The classification of glomerulonephritis in systemic lupus erythematosus revisited. J Am Soc Nephrol. 2004, 15: 241-250.CrossRefPubMed
27.
go back to reference Cassidy JT, Sullivan DB, Petty RE, Ragsdale C: Lupus nephritis and encephalopathy. Prognosis in 58 children. Arthritis Rheum. 1977, 20: 315-322.PubMed Cassidy JT, Sullivan DB, Petty RE, Ragsdale C: Lupus nephritis and encephalopathy. Prognosis in 58 children. Arthritis Rheum. 1977, 20: 315-322.PubMed
28.
go back to reference Celermajer DS, Thorner PS, Baumal R, Arbus GS: Sex differences in childhood lupus nephritis. Am J Dis Child. 1984, 138: 586-588.PubMed Celermajer DS, Thorner PS, Baumal R, Arbus GS: Sex differences in childhood lupus nephritis. Am J Dis Child. 1984, 138: 586-588.PubMed
29.
go back to reference Hagge WW, Burke EC, Stickler GB: Treatment of systemic lupus erythematosus complicated by nephritis in children. Pediatrics. 1967, 40: 822-827.PubMed Hagge WW, Burke EC, Stickler GB: Treatment of systemic lupus erythematosus complicated by nephritis in children. Pediatrics. 1967, 40: 822-827.PubMed
30.
go back to reference King KK, Kornreich HK, Bernstein BH, Singsen BH, Hanson V: The clinical spectrum of systemic lupus erythematosus in childhood. Arthritis Rheum. 1977, 20: 287-294.PubMed King KK, Kornreich HK, Bernstein BH, Singsen BH, Hanson V: The clinical spectrum of systemic lupus erythematosus in childhood. Arthritis Rheum. 1977, 20: 287-294.PubMed
31.
go back to reference Norris DG, Colon AR, Stickler GB: Systemic lupus erythematosus in children: the complex problems of diagnosis and treatment encountered in 101 such patients at the Mayo Clinic. Clin Pediatr (Phila). 1977, 16: 774-778.CrossRef Norris DG, Colon AR, Stickler GB: Systemic lupus erythematosus in children: the complex problems of diagnosis and treatment encountered in 101 such patients at the Mayo Clinic. Clin Pediatr (Phila). 1977, 16: 774-778.CrossRef
32.
go back to reference Font J, Cervera R, Espinosa G, Pallares L, Ramos-Casals M, Jimenez S, Garcia-Carrasco M, Seisdedos L, Ingelmo M: Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults. Ann Rheum Dis. 1998, 57: 456-459.PubMedCentralCrossRefPubMed Font J, Cervera R, Espinosa G, Pallares L, Ramos-Casals M, Jimenez S, Garcia-Carrasco M, Seisdedos L, Ingelmo M: Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults. Ann Rheum Dis. 1998, 57: 456-459.PubMedCentralCrossRefPubMed
33.
go back to reference Hashimoto H, Tsuda H, Hirano T, Takasaki Y, Matsumoto T, Hirose S: Differences in clinical and immunological findings of systemic lupus erythematosus related to age. J Rheumatol. 1987, 14: 497-501.PubMed Hashimoto H, Tsuda H, Hirano T, Takasaki Y, Matsumoto T, Hirose S: Differences in clinical and immunological findings of systemic lupus erythematosus related to age. J Rheumatol. 1987, 14: 497-501.PubMed
34.
go back to reference Rosenberg AM: Systemic lupus erythematosus in children. Springer Semin Immunopathol. 1994, 16: 261-279.CrossRefPubMed Rosenberg AM: Systemic lupus erythematosus in children. Springer Semin Immunopathol. 1994, 16: 261-279.CrossRefPubMed
36.
go back to reference Yu HH, Wang LC, Lee JH, Lee CC, Yang YH, Chiang BL: Lymphopenia is associated with neuropsychiatric manifestations and disease activity in paediatric systemic lupus erythematosus patients. Rheumatology (Oxford). 2007, 46: 1492-1494.CrossRef Yu HH, Wang LC, Lee JH, Lee CC, Yang YH, Chiang BL: Lymphopenia is associated with neuropsychiatric manifestations and disease activity in paediatric systemic lupus erythematosus patients. Rheumatology (Oxford). 2007, 46: 1492-1494.CrossRef
37.
38.
go back to reference Lo JT, Tsai MJ, Wang LH, Huang MT, Yang YH, Lin YT, Liu J, Chiang BL: Sex differences in pediatric systemic lupus erythematosus: a retrospective analysis of 135 cases. J Microbiol Immunol Infect. 1999, 32: 173-178.PubMed Lo JT, Tsai MJ, Wang LH, Huang MT, Yang YH, Lin YT, Liu J, Chiang BL: Sex differences in pediatric systemic lupus erythematosus: a retrospective analysis of 135 cases. J Microbiol Immunol Infect. 1999, 32: 173-178.PubMed
39.
go back to reference Kumar K, Chambers S, Gordon C: Challenges of ethnicity in SLE. Best Pract Res Clin Rheumatol. 2009, 23: 549-561.CrossRefPubMed Kumar K, Chambers S, Gordon C: Challenges of ethnicity in SLE. Best Pract Res Clin Rheumatol. 2009, 23: 549-561.CrossRefPubMed
Metadata
Title
Differential manifestations of prepubescent, pubescent and postpubescent pediatric patients with systemic lupus erythematosus: A retrospective study of 96 Chinese children and adolescents
Authors
Li-Lan Chiang
Yu-Tsan Lin
Hung-Yi Chan
Bor-Luen Chiang
Publication date
01-12-2012
Publisher
BioMed Central
Published in
Pediatric Rheumatology / Issue 1/2012
Electronic ISSN: 1546-0096
DOI
https://doi.org/10.1186/1546-0096-10-12

Other articles of this Issue 1/2012

Pediatric Rheumatology 1/2012 Go to the issue