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Published in: Clinical Rheumatology 2/2011

01-02-2011 | Case Based Review

Early onset systemic lupus erythematosus: differential diagnoses, clinical presentation, and treatment options

Authors: Christian Michael Hedrich, Hildegard Zappel, Simon Straub, Martin W. Laass, Kathrin Wieczorek, Gabriele Hahn, Georg Heubner, Manfred Gahr

Published in: Clinical Rheumatology | Issue 2/2011

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Abstract

Juvenile systemic lupus erythematosus is a rare multisystemic autoimmune disease with variable clinical manifestations, and disease onset before 16 years of age. Patients younger than 5 years are rarely affected and the age of onset may contribute to the course of disease in terms of clinical presentation, organ involvement, and serological findings. Here, we report two exemplary early-onset SLE patients, a 28-month-old patient with WHO class IIB kidney disease, arthritis, and a typical antibody constellation and an 11-month-old infant that presented with microcytic anemia, leukocytosis, arthritis, fasciitis, fatty liver disease, protein losing enteropathy, edema, and minimal change glomerulonephritis. Epidemiologic and clinical features of early-onset SLE compared to other forms of SLE are given and differential diagnoses and treatment options are discussed.
Literature
1.
go back to reference Zulian F, Pluchinotta F, Martini G, Da Dalt L, Zacchello G (2008) Severe clinical course of systemic lupus erythematosus in the first year of life. Lupus 17(9):780–786CrossRefPubMed Zulian F, Pluchinotta F, Martini G, Da Dalt L, Zacchello G (2008) Severe clinical course of systemic lupus erythematosus in the first year of life. Lupus 17(9):780–786CrossRefPubMed
2.
go back to reference Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40(9):1725CrossRefPubMed Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40(9):1725CrossRefPubMed
3.
go back to reference Jordan SC, Lemire JM, Border W, Sakai R, Ettenger RB, Fine RN (1984) False-negative anti-DNA antibody activity in infantile systemic lupus erythematosus (SLE). Clin Immunol 4(2):156–162CrossRef Jordan SC, Lemire JM, Border W, Sakai R, Ettenger RB, Fine RN (1984) False-negative anti-DNA antibody activity in infantile systemic lupus erythematosus (SLE). Clin Immunol 4(2):156–162CrossRef
4.
go back to reference Anderson JR (1981) Intracerebral calcification in a case of systemic lupus erythematosus with neurological manifestations. Neuropathol Appl Neurobiol 7(2):161–166CrossRefPubMed Anderson JR (1981) Intracerebral calcification in a case of systemic lupus erythematosus with neurological manifestations. Neuropathol Appl Neurobiol 7(2):161–166CrossRefPubMed
5.
go back to reference Cummings NP, Hansen J, Hollister JR (1985) Systemic lupus erythematosus in a premature infant. Arthritis Rheum 28(5):573–575CrossRefPubMed Cummings NP, Hansen J, Hollister JR (1985) Systemic lupus erythematosus in a premature infant. Arthritis Rheum 28(5):573–575CrossRefPubMed
7.
go back to reference Horn HC, Ottosen P, Junker P (2001) Renal crisis in asclerodermic scleroderma–lupus overlap syndrome. Lupus 10(12):886–888CrossRefPubMed Horn HC, Ottosen P, Junker P (2001) Renal crisis in asclerodermic scleroderma–lupus overlap syndrome. Lupus 10(12):886–888CrossRefPubMed
8.
go back to reference Cassidy JT, Petty RE, Laxer RM, Lindsley CB (2005) Textbook of pediatric rheumatology 5th edn. Elsevier, Amsterdam, pp 342–391 Cassidy JT, Petty RE, Laxer RM, Lindsley CB (2005) Textbook of pediatric rheumatology 5th edn. Elsevier, Amsterdam, pp 342–391
9.
go back to reference Hedrich CM, Fiebig B, Hauck FH, Sallmann S, Hahn G, Pfeiffer C, Heubner G, Lee-Kirsch MA, Gahr M (2008) Chilblain lupus erythematosus—a review of literature. Clin Rheumatol 27(8):949–954, Epub 2008 Jun 10CrossRefPubMed Hedrich CM, Fiebig B, Hauck FH, Sallmann S, Hahn G, Pfeiffer C, Heubner G, Lee-Kirsch MA, Gahr M (2008) Chilblain lupus erythematosus—a review of literature. Clin Rheumatol 27(8):949–954, Epub 2008 Jun 10CrossRefPubMed
10.
go back to reference Bader-Meunier B, Armengaud JB, Haddad E, Salomon R, Deschênes G, Koné-Paut I, Leblanc T, Loirat C, Niaudet P, Piette JC, Prieur AM, Quartier P, Bouissou F, Foulard M, Leverger G, Lemelle I, Pilet P, Rodière M, Sirvent N, Cochat P (2005) Initial presentation of childhood-onset systemic lupus erythematosus: a French multicenter study. J Pediatr 146(5):648–653CrossRefPubMed Bader-Meunier B, Armengaud JB, Haddad E, Salomon R, Deschênes G, Koné-Paut I, Leblanc T, Loirat C, Niaudet P, Piette JC, Prieur AM, Quartier P, Bouissou F, Foulard M, Leverger G, Lemelle I, Pilet P, Rodière M, Sirvent N, Cochat P (2005) Initial presentation of childhood-onset systemic lupus erythematosus: a French multicenter study. J Pediatr 146(5):648–653CrossRefPubMed
11.
go back to reference Heidet L, Gubler MC (2009) The renal lesions of Alport syndrome. J Am Soc Nephrol 20(6):1210–1215, Epub 2009 May 21CrossRefPubMed Heidet L, Gubler MC (2009) The renal lesions of Alport syndrome. J Am Soc Nephrol 20(6):1210–1215, Epub 2009 May 21CrossRefPubMed
12.
go back to reference Maxon WT (1963) Benign proteinuria of childhood and adoslescence: a survey. Clin Pediatr (Phila) 2:662–668CrossRef Maxon WT (1963) Benign proteinuria of childhood and adoslescence: a survey. Clin Pediatr (Phila) 2:662–668CrossRef
13.
go back to reference Abaterusso C, De Biase V, Salviati A, Fabris A, Millardi D, Tomei P, Bernich P, Lupo A, Gambaro G (2009) Unusual renal presentation of Fabry disease in a female patient. Nat Rev Nephrol 5(6):349–354, Epub 2009 Apr 28CrossRefPubMed Abaterusso C, De Biase V, Salviati A, Fabris A, Millardi D, Tomei P, Bernich P, Lupo A, Gambaro G (2009) Unusual renal presentation of Fabry disease in a female patient. Nat Rev Nephrol 5(6):349–354, Epub 2009 Apr 28CrossRefPubMed
14.
go back to reference Strippoli GF, Di Paolo S, Cincione R, Di Palma AM, Teutonico A, Grandaliano G, Schena FP, Gesualdo L (2003) Clinical and therapeutic aspects of diabetic nephropathy. J Nephrol 16(4):487–499PubMed Strippoli GF, Di Paolo S, Cincione R, Di Palma AM, Teutonico A, Grandaliano G, Schena FP, Gesualdo L (2003) Clinical and therapeutic aspects of diabetic nephropathy. J Nephrol 16(4):487–499PubMed
15.
go back to reference Ritz E, Nowicki M, Fliser D, Hörner D, Klimm HP (1994) Proteinuria and hypertension. Kidney Int Suppl 47:S76–S80PubMed Ritz E, Nowicki M, Fliser D, Hörner D, Klimm HP (1994) Proteinuria and hypertension. Kidney Int Suppl 47:S76–S80PubMed
16.
go back to reference Tsimihodimos V, Psychogios N, Kakaidi V, Bairaktari E, Elisaf M (2007) Salicylate-induced proximal tubular dysfunction. Am J Kidney Dis 50(3):463–467CrossRefPubMed Tsimihodimos V, Psychogios N, Kakaidi V, Bairaktari E, Elisaf M (2007) Salicylate-induced proximal tubular dysfunction. Am J Kidney Dis 50(3):463–467CrossRefPubMed
17.
18.
go back to reference Gibson T, Myers AR (1981) Subclinical liver disease in systemic lupus erythematosus. J Rheumatol 8(5):752–759PubMed Gibson T, Myers AR (1981) Subclinical liver disease in systemic lupus erythematosus. J Rheumatol 8(5):752–759PubMed
19.
go back to reference Matsumoto T, Yoshimine T, Shimouchi K, Shiotu H, Kuwabara N, Fukuda Y, Hoshi T (1992) The liver in systemic lupus erythematosus: pathologic analysis of 52 cases and review of Japanese Autopsy Registry Data. Hum Pathol 23(10):1151–1158CrossRefPubMed Matsumoto T, Yoshimine T, Shimouchi K, Shiotu H, Kuwabara N, Fukuda Y, Hoshi T (1992) The liver in systemic lupus erythematosus: pathologic analysis of 52 cases and review of Japanese Autopsy Registry Data. Hum Pathol 23(10):1151–1158CrossRefPubMed
20.
go back to reference Jaeken J, Matthijs G (2007) Congenital disorders of glycosylation: a rapidly expanding disease family. Annu Rev Genomics Hum Genet 8:261–278CrossRefPubMed Jaeken J, Matthijs G (2007) Congenital disorders of glycosylation: a rapidly expanding disease family. Annu Rev Genomics Hum Genet 8:261–278CrossRefPubMed
21.
22.
go back to reference Baerlocher K, Gitzelmann R, Steinmann B, Gitzelmann-Cumarasamy N (1978) Hereditary fructose intolerance in early childhood: a major diagnostic challenge. Survey of 20 symptomatic cases. Helv Paediatr Acta 33(6):465–487PubMed Baerlocher K, Gitzelmann R, Steinmann B, Gitzelmann-Cumarasamy N (1978) Hereditary fructose intolerance in early childhood: a major diagnostic challenge. Survey of 20 symptomatic cases. Helv Paediatr Acta 33(6):465–487PubMed
23.
go back to reference Locher S (1987) Acute liver and kidney failure following sorbitol infusion in a 28-year-old patient with undiagnosed fructose intolerance. Anasth Intensivther Notfallmed 22(4):194–197CrossRefPubMed Locher S (1987) Acute liver and kidney failure following sorbitol infusion in a 28-year-old patient with undiagnosed fructose intolerance. Anasth Intensivther Notfallmed 22(4):194–197CrossRefPubMed
24.
go back to reference Risser A, Donovan D, Heintzman J, Page T (2009) NSAID prescribing precautions. Am Fam Phys 80(12):1371–1378 Risser A, Donovan D, Heintzman J, Page T (2009) NSAID prescribing precautions. Am Fam Phys 80(12):1371–1378
25.
go back to reference Pérez-Gutthann S, García-Rodríguez LA, Duque-Oliart A, Varas-Lorenzo C (1999) Low-dose diclofenac, naproxen, and ibuprofen cohort study. Pharmacotherapy 19(7):854–859CrossRefPubMed Pérez-Gutthann S, García-Rodríguez LA, Duque-Oliart A, Varas-Lorenzo C (1999) Low-dose diclofenac, naproxen, and ibuprofen cohort study. Pharmacotherapy 19(7):854–859CrossRefPubMed
26.
go back to reference Yoshida M, Miyata M, Saka M, Sasajima T, Sato H, Takiguchi J, Shishido H, Saito A, Kokubun M, Nishimaki T, Kasukawa R (2001) Protein-losing enteropathy exacerbated with the appearance of symptoms of systemic lupus erythematosus. Intern Med 40(5):449–453CrossRefPubMed Yoshida M, Miyata M, Saka M, Sasajima T, Sato H, Takiguchi J, Shishido H, Saito A, Kokubun M, Nishimaki T, Kasukawa R (2001) Protein-losing enteropathy exacerbated with the appearance of symptoms of systemic lupus erythematosus. Intern Med 40(5):449–453CrossRefPubMed
27.
go back to reference Trentham DE, Masi AT (1976) Systemic lupus erythematosus with a protein-losing enteropathy. JAMA 236(3):287–288CrossRefPubMed Trentham DE, Masi AT (1976) Systemic lupus erythematosus with a protein-losing enteropathy. JAMA 236(3):287–288CrossRefPubMed
28.
go back to reference Viallard JF, Fach J, Mercie P, Leng B, Pellegrin JL, Longy-Boursier M (1998) Exudative enteropathy in disseminated lupus erythematosus. Report of 3 cases and review of the literature. Ann Med Interne (Paris) 149(8):485–491 Viallard JF, Fach J, Mercie P, Leng B, Pellegrin JL, Longy-Boursier M (1998) Exudative enteropathy in disseminated lupus erythematosus. Report of 3 cases and review of the literature. Ann Med Interne (Paris) 149(8):485–491
29.
go back to reference Benner KG, Montanaro A (1989) Protein-losing enteropathy in systemic lupus erythematosus. Diagnosis and monitoring immunosuppressive therapy by alpha-1-antitrypsin clearance in stool. Dig Dis Sci 34(1):132–135CrossRefPubMed Benner KG, Montanaro A (1989) Protein-losing enteropathy in systemic lupus erythematosus. Diagnosis and monitoring immunosuppressive therapy by alpha-1-antitrypsin clearance in stool. Dig Dis Sci 34(1):132–135CrossRefPubMed
30.
go back to reference Mader R, Adawi M, Schonfeld S (1997) Malabsorption in systemic lupus erythematosus. Clin Exp Rheumatol 15(6):659–661PubMed Mader R, Adawi M, Schonfeld S (1997) Malabsorption in systemic lupus erythematosus. Clin Exp Rheumatol 15(6):659–661PubMed
31.
go back to reference Grossman J, Schwartz RH, Callerame ML, Condemi JJ (1975) Systemic lupus erythematosus in a 1-year-old child. Am J Dis Child 129(1):123–125PubMed Grossman J, Schwartz RH, Callerame ML, Condemi JJ (1975) Systemic lupus erythematosus in a 1-year-old child. Am J Dis Child 129(1):123–125PubMed
32.
go back to reference Dudley J, Fenton T, Unsworth J, Chambers T, MacIver A, Tizard J (1996) Systemic lupus erythematosus presenting as congenital nephrotic syndrome. J Pediatr Nephrol 10(6):752–755CrossRef Dudley J, Fenton T, Unsworth J, Chambers T, MacIver A, Tizard J (1996) Systemic lupus erythematosus presenting as congenital nephrotic syndrome. J Pediatr Nephrol 10(6):752–755CrossRef
33.
go back to reference Shetty VB, Rao S, Krishnamurthy PN, Shenoy VU (2001) Peripheral gangrene during infancy: a rare presentation of systemic lupus erythematosus. Arch Dis Child 85(4):335–336CrossRefPubMed Shetty VB, Rao S, Krishnamurthy PN, Shenoy VU (2001) Peripheral gangrene during infancy: a rare presentation of systemic lupus erythematosus. Arch Dis Child 85(4):335–336CrossRefPubMed
34.
go back to reference Kreindler J, Ellis D, Vats A, Kurland G, Ranganathan S, Moritz ML (2005) Infantile systemic lupus erythematosus presenting with pulmonary hemorrhage. Pediatr Nephrol 20(4):522–525CrossRefPubMed Kreindler J, Ellis D, Vats A, Kurland G, Ranganathan S, Moritz ML (2005) Infantile systemic lupus erythematosus presenting with pulmonary hemorrhage. Pediatr Nephrol 20(4):522–525CrossRefPubMed
35.
go back to reference Duzova A, Bakkaloglu A (2008) Central nervous system involvement in pediatric rheumatic diseases: current concepts in treatment. Curr Pharm Des 14(13):1295–1301PubMed Duzova A, Bakkaloglu A (2008) Central nervous system involvement in pediatric rheumatic diseases: current concepts in treatment. Curr Pharm Des 14(13):1295–1301PubMed
36.
go back to reference Hedrich CM, Fiebig B, Sallmann S, Bruck N, Hahn G, Roesler J, Roesen-Wolff A, Heubner G, Gahr M (2008) Good response to IL-1beta blockade by anakinra in a 23-year-old CINCA/NOMID patient without mutations in the CIAS1 gene. Cytokine profiles and functional studies. Scand J Rheumatol 37(5):385–389CrossRefPubMed Hedrich CM, Fiebig B, Sallmann S, Bruck N, Hahn G, Roesler J, Roesen-Wolff A, Heubner G, Gahr M (2008) Good response to IL-1beta blockade by anakinra in a 23-year-old CINCA/NOMID patient without mutations in the CIAS1 gene. Cytokine profiles and functional studies. Scand J Rheumatol 37(5):385–389CrossRefPubMed
37.
go back to reference Jordan JM, Valenstein P, Kredich DW (1989) Systemic lupus erythematosus with Libman-Sachs endocarditis in a 9-month-old infant with neonatal lupus erythematosus and congenital heart block. Pediatrics 84(3):574–578PubMed Jordan JM, Valenstein P, Kredich DW (1989) Systemic lupus erythematosus with Libman-Sachs endocarditis in a 9-month-old infant with neonatal lupus erythematosus and congenital heart block. Pediatrics 84(3):574–578PubMed
38.
go back to reference Massengill SF, Richard GA, Donnelly WH (1994) Infantile systemic lupus erythematosus with onset simulating congenital nephrotic syndrome. J Pediatr 124(1):27–31CrossRefPubMed Massengill SF, Richard GA, Donnelly WH (1994) Infantile systemic lupus erythematosus with onset simulating congenital nephrotic syndrome. J Pediatr 124(1):27–31CrossRefPubMed
39.
go back to reference Saberi MS, Jones BA (1998) Remission of infantile systemic lupus erythematosus with intravenous cyclophosphamide. Pediatr Nephrol 12(2):136–138CrossRefPubMed Saberi MS, Jones BA (1998) Remission of infantile systemic lupus erythematosus with intravenous cyclophosphamide. Pediatr Nephrol 12(2):136–138CrossRefPubMed
40.
go back to reference Gaubitz M, Schotte H (2005) Early diagnosis in patients with systemic lupus erythematosus (SLE). Z Rheumatol 64(8):547–552CrossRefPubMed Gaubitz M, Schotte H (2005) Early diagnosis in patients with systemic lupus erythematosus (SLE). Z Rheumatol 64(8):547–552CrossRefPubMed
41.
go back to reference Houssiau FA, Vasconcelos C, D’Cruz D, Sebastiani GD, Garrido Ed Ede R, Danieli MG, Abramovicz D, Blockmans D, Mathieu A, Direskeneli H, Galeazzi M, Gül A, Levy Y, Petera P, Popovic R, Petrovic R, Sinico RA, Cattaneo R, Font J, Depresseux G, Cosyns JP, Cervera R (2002) Immunosuppressive therapy in lupus nephritis: the Euro-Lupus Nephritis Trial, a randomized trial of low-dose versus high-dose intravenous cyclophosphamide. Arthritis Rheum 46(8):2121–2131CrossRefPubMed Houssiau FA, Vasconcelos C, D’Cruz D, Sebastiani GD, Garrido Ed Ede R, Danieli MG, Abramovicz D, Blockmans D, Mathieu A, Direskeneli H, Galeazzi M, Gül A, Levy Y, Petera P, Popovic R, Petrovic R, Sinico RA, Cattaneo R, Font J, Depresseux G, Cosyns JP, Cervera R (2002) Immunosuppressive therapy in lupus nephritis: the Euro-Lupus Nephritis Trial, a randomized trial of low-dose versus high-dose intravenous cyclophosphamide. Arthritis Rheum 46(8):2121–2131CrossRefPubMed
42.
go back to reference Houssiau FA, D’Cruz DP, Sangle SR, Remy P, Vasconcelos C, de Ramon Garrido E, Gilboe IM Sr, Ravelingien I, Tektonidou M, Depresseux G, Guillevin LP, Cervera R (2009) Azathioprine versus mycophenolate mofetil for maintenance immunosuppression of proliferative lupus nephritis: results of a randomized trial (MAINTAIN). Arthritis Rheum 60(10):S429–S430 Houssiau FA, D’Cruz DP, Sangle SR, Remy P, Vasconcelos C, de Ramon Garrido E, Gilboe IM Sr, Ravelingien I, Tektonidou M, Depresseux G, Guillevin LP, Cervera R (2009) Azathioprine versus mycophenolate mofetil for maintenance immunosuppression of proliferative lupus nephritis: results of a randomized trial (MAINTAIN). Arthritis Rheum 60(10):S429–S430
43.
go back to reference Silverman E (1996) What’s new in the treatment of pediatric SLE. J Rheumatol 23(9):1657–1660PubMed Silverman E (1996) What’s new in the treatment of pediatric SLE. J Rheumatol 23(9):1657–1660PubMed
44.
go back to reference Austin HA, Balow JE (2000) Treatment of lupus nephritis. Semin Nephrol 20(3):265–276PubMed Austin HA, Balow JE (2000) Treatment of lupus nephritis. Semin Nephrol 20(3):265–276PubMed
45.
go back to reference Valesini G, Priori R, Francia A, Balestrieri G, Tincani A, Airo P, Cattaneo R, Zambruni A, Troianello B, Chofflon M et al (1994) Central nervous system involvement in systemic lupus erythematosus: a new therapeutic approach with intrathecal dexamethasone and methotrexate. Springer Semin Immunopathol 16(2–3):313–321PubMed Valesini G, Priori R, Francia A, Balestrieri G, Tincani A, Airo P, Cattaneo R, Zambruni A, Troianello B, Chofflon M et al (1994) Central nervous system involvement in systemic lupus erythematosus: a new therapeutic approach with intrathecal dexamethasone and methotrexate. Springer Semin Immunopathol 16(2–3):313–321PubMed
Metadata
Title
Early onset systemic lupus erythematosus: differential diagnoses, clinical presentation, and treatment options
Authors
Christian Michael Hedrich
Hildegard Zappel
Simon Straub
Martin W. Laass
Kathrin Wieczorek
Gabriele Hahn
Georg Heubner
Manfred Gahr
Publication date
01-02-2011
Publisher
Springer-Verlag
Published in
Clinical Rheumatology / Issue 2/2011
Print ISSN: 0770-3198
Electronic ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-010-1576-2

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