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Clinicopathological correlations of paediatric lupus nephritis

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Abstract

The International Society of Nephrology and Renal Pathology Society Working Group revised the histopathological classification of lupus nephritis (LN) in 2003. We studied the clinical outcome of 39 children (85% female) aged 3.3-18.0 (median 13.7) years who underwent 49 percutaneous renal biopsies at 0.1–7.8 (median 1.0) years from diagnosis of systemic lupus erythematosus (SLE) at our centre over 10 years. All renal biopsies were reviewed and reclassified according to the new criteria by one histopathologist: 2%, 13%, 15%, 51% and 20% of all cases were classes I–V, respectively (with no cases of class VI LN) and 12% overlap cases (4% classes III and V, 8% classes IV and V). Patients were followed up for 1.3–15.4 (median 5.5) years with renal and overall survival rates of 90% and 92%, respectively. Half of the children with LN have features of class IV LN, with diffuse global (class IV-G) LN associated with the worst clinical outcome and the three most severe cases of chronic renal failure with estimated glomerular filtration rates (GFRs) <25 ml/min per 1.73 m2 in patients with diffuse global sclerosing [class IV-G(C)] LN. The new classification allows expanded histopathological grading of LN with further delineation of classes III and IV with activity and chronicity indices.

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References

  1. Moss KE, Ioannou Y, Sultan SM, Haq I, Isenberg DA (2002) Outcome of a cohort of 300 patients with systemic lupus erythematosus attending a dedicated clinic for over two decades. Ann Rheum Dis 61:409–413

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Glidden RS, Mantzouranis EC, Borel Y (1983) Systemic lupus erythematosus in childhood: clinical manifestations and improved survival in fifty-five patients. Clin Immunol Immunopathol 29:196–210

    Article  CAS  PubMed  Google Scholar 

  3. Lehman TJ, McCurdy DK, Bernstein BH, King KK, Hanson V (1989) Systemic lupus erythematosus in the first decade of life. Pediatrics 83:235–239

    CAS  PubMed  Google Scholar 

  4. Marini R, Costallat LT (1999) Young age at onset, renal involvement, and arterial hypertension are of adverse prognostic significance in juvenile systemic lupus erythematosus. Rev Rhum Engl Ed 66:303–309

    CAS  PubMed  Google Scholar 

  5. Stichweh D, Arce E, Pascual V (2004) Update on pediatric systemic lupus erythematosus. Curr Opin Rheumatol 16:577–587

    Article  CAS  PubMed  Google Scholar 

  6. Walravens PA, Chase HP (1976) The prognosis of childhood systemic lupus erythematosus. Am J Dis Child 130:929–933

    CAS  PubMed  Google Scholar 

  7. Font J, Cervera R, Espinosa G, Pallares L, Ramos-Casals M, Jimenez S, Garcia-Carrasco M, Seisdedos L, Ingelmo M (1998) 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 57:456–459

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Tucker LB, Menon S, Schaller JG, Isenberg DA (1995) Adult- and childhood-onset systemic lupus erythematosus: a comparison of onset, clinical features, serology, and outcome. Br J Rheumatol 34:866–872

    Article  CAS  PubMed  Google Scholar 

  9. Cameron JS (1994) Lupus nephritis in childhood and adolescence. Pediatr Nephrol 8:230–249

    Article  CAS  PubMed  Google Scholar 

  10. Austin HA, III, Muenz LR, Joyce KM, Antonovych TA, Kullick ME, Klippel JH, Decker JL, Balow JE (1983) Prognostic factors in lupus nephritis. Contribution of renal histologic data. Am J Med 75:382–391

    Article  PubMed  Google Scholar 

  11. Baqi N, Moazami S, Singh A, Ahmad H, Balachandra S, Tejani A (1996) Lupus nephritis in children: a longitudinal study of prognostic factors and therapy. J Am Soc Nephrol 7:924–929

    CAS  PubMed  Google Scholar 

  12. Bogdanovic R, Nikolic V, Pasic S, Dimitrijevic J, Lipkovska-Markovic J, Eric-Marinkovic J, Ognjanovic M, Minic A, Stajic N (2004) Lupus nephritis in childhood: a review of 53 patients followed at a single center. Pediatr Nephrol 19:36–44

    Article  PubMed  Google Scholar 

  13. Emre S, Bilge I, Sirin A, Kilicaslan I, Nayir A, Oktem F, Uysal V (2001) Lupus nephritis in children: prognostic significance of clinicopathological findings. Nephron 87:118–126

    Article  CAS  PubMed  Google Scholar 

  14. Hagelberg S, Lee Y, Bargman J, Mah G, Schneider R, Laskin C, Eddy A, Gladman D, Urowitz M, Hebert D, Silverman E (2002) Longterm followup of childhood lupus nephritis. J Rheumatol 29:2635–2642

    PubMed  Google Scholar 

  15. Cameron JS (1999) Lupus nephritis. J Am Soc Nephrol 10:413–424

    CAS  PubMed  Google Scholar 

  16. Churg J, Sobin LH (1982) Glomerulonephritis of systemic disease: lupus nephritis. In: Churg J, Sobin LH (eds). Renal disease: classification and atlas of glomerular disease. Igaku-Shoin, Tokyo, pp 127–149

    Google Scholar 

  17. Churg J, Bernstein J, Glassock RJ (1995) Renal disease: classification and atlas of glomerular disease. Igaku-Shoin, New York

    Google Scholar 

  18. Appel GB, Cohen DJ, Pirani CL, Meltzer JI, Estes D (1987) Long-term follow-up of patients with lupus nephritis. A study based on the classification of the World Health Organization. Am J Med 83:877–885

    Article  CAS  PubMed  Google Scholar 

  19. Donadio JV Jr, Hart GM, Bergstralh EJ, Holley KE (1995) Prognostic determinants in lupus nephritis: a long-term clinicopathologic study. Lupus 4:109–115

    Article  PubMed  Google Scholar 

  20. Esdaile JM, Levinton C, Federgreen W, Hayslett JP, Kashgarian M (1989) The clinical and renal biopsy predictors of long-term outcome in lupus nephritis: a study of 87 patients and review of the literature. Q J Med 72:779–833

    CAS  PubMed  Google Scholar 

  21. Esdaile JM, Mackenzie T, Barre P, Danoff D, Osterland CK, Somerville P, Quintal H, Kashgarian M, Suissa S (1992) Can experienced clinicians predict the outcome of lupus nephritis? Lupus 1:205–214

    Article  CAS  PubMed  Google Scholar 

  22. Jacobsen S, Starklint H, Petersen J, Ullman S, Junker P, Voss A, Rasmussen JM, Tarp U, Poulsen LH, van Overeem Hansen G, Skaarup B, Hansen TM, Podenphant J, Halberg P (1999) Prognostic value of renal biopsy and clinical variables in patients with lupus nephritis and normal serum creatinine. Scand J Rheumatol 28:288–299

    Article  CAS  PubMed  Google Scholar 

  23. MacGowan JR, Ellis S, Griffiths M, Isenberg DA (2002) Retrospective analysis of outcome in a cohort of patients with lupus nephritis treated between 1977 and 1999. Rheumatology (Oxford) 41:981–987

    Article  CAS  Google Scholar 

  24. Celermajer DS, Thorner PS, Baumal R, Arbus GS (1984) Sex differences in childhood lupus nephritis. Am J Dis Child 138:586–588

    CAS  PubMed  Google Scholar 

  25. Khoo J, Pee S, Thevarajah B, Yap Y, Chin C (2005) Lupus nephritis in children in Malaysia. J Paediatr Child Health 41:31–35

    Article  CAS  PubMed  Google Scholar 

  26. Massardo L, Martinez ME, Jacobelli S, Villarroel L, Rosenberg H, Rivero S (1994) Survival of Chilean patients with systemic lupus erythematosus. Semin Arthritis Rheum 24:1–11

    Article  CAS  PubMed  Google Scholar 

  27. McCurdy DK, Lehman TJ, Bernstein B, Hanson V, King KK, Nadorra R, Landing BH (1992) Lupus nephritis: prognostic factors in children. Pediatrics 89:240–246

    CAS  PubMed  Google Scholar 

  28. Rush PJ, Baumal R, Shore A, Balfe JW, Schreiber M (1986) Correlation of renal histology with outcome in children with lupus nephritis. Kidney Int 29:1066–1071

    Article  CAS  PubMed  Google Scholar 

  29. Vachvanichsanong P, Dissaneewate P, Winn T (2004) Intravenous cyclophosphamide for lupus nephritis in Thai children. Scand J Rheumatol 33:339–342

    Article  CAS  PubMed  Google Scholar 

  30. Yang LY, Chen WP, Lin CY (1994) Lupus nephritis in children—a review of 167 patients. Pediatrics 94:335–340

    CAS  PubMed  Google Scholar 

  31. Zappitelli M, Duffy C, Bernard C, Scuccimarri R, Watanabe Duffy K, Kagan R, Gupta IR (2004) Clinicopathological study of the WHO classification in childhood lupus nephritis. Pediatr Nephrol 19:503–510

    Article  PubMed  Google Scholar 

  32. Weening JJ, D’Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F, Fogo AB, Ginzler EM, Hebert L, Hill G, Hill P, Jennette JC, Kong NC, Lesavre P, Lockshin M, Looi LM, Makino H, Moura LA, Nagata M; International Society of Nephrology Working Group on the Classification of Lupus Nephritis; Renal Pathology Society Working Group on the Classification of Lupus Nephritis (2004) The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney Int 65:521–530

    Article  PubMed  Google Scholar 

  33. Weening JJ, D’Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F, Fogo AB, Ginzler EM, Hebert L, Hill G, Hill P, Jennette JC, Kong NC, Lesavre P, Lockshin M, Looi LM, Makino H, Moura LA, Nagata M (2004) The classification of glomerulonephritis in systemic lupus erythematosus revisited. J Am Soc Nephrol 15:241–250

    Article  PubMed  Google Scholar 

  34. Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40:1725

    Article  CAS  PubMed  Google Scholar 

  35. Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, Schaller JG, Talal N, Winchester RJ (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271–1277

    Article  CAS  PubMed  Google Scholar 

  36. Morris MC, Allanby CW, Toseland P, Haycock GB, Chantler C (1982) Evaluation of a height/plasma creatinine formula in the measurement of glomerular filtration rate. Arch Dis Child 57:611–615

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  37. Wang LC, Yang YH, Lu MY, Chiang BL (2003) Retrospective analysis of mortality and morbidity of pediatric systemic lupus erythematosus in the past two decades. J Microbiol Immunol Infect 36:203–208

    PubMed  Google Scholar 

  38. Yokoyama H, Wada T, Hara A, Yamahana J, Nakaya I, Kobayashi M, Kitagawa K, Kokubo S, Iwata Y, Yoshimoto K, Shimizu K, Sakai N, Furuichi K, Kanazawa; Study Group for Renal Diseases and Hypertension (2004) The outcome and a new ISN/RPS 2003 classification of lupus nephritis in Japanese. Kidney Int 66:2382–2388

    Article  PubMed  Google Scholar 

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Correspondence to Stephen D. Marks.

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Marks, S.D., Sebire, N.J., Pilkington, C. et al. Clinicopathological correlations of paediatric lupus nephritis. Pediatr Nephrol 22, 77–83 (2007). https://doi.org/10.1007/s00467-006-0296-y

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  • DOI: https://doi.org/10.1007/s00467-006-0296-y

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