DOI QR코드

DOI QR Code

Two pediatric cases with hematuria, normal renal function and positive antineutrophil cytoplasmic antibodies

  • Ji Hyeon, Lim (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Ji Won, Jung (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Heoun Jeong, Go (Department of Pathology, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Joo Hoon, Lee (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Young Seo, Park (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine)
  • Received : 2022.07.04
  • Accepted : 2022.10.18
  • Published : 2022.12.31

Abstract

Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis requires prompt diagnosis and treatment, since renal function at the time of diagnosis is significantly associated with renal outcomes. Here, we report two pediatric patients with ANCA-positive glomerulonephritis initially presenting with hematuria, mild proteinuria, and normal renal function. The first patient with a high myeloperoxidase-ANCA titer (>134 IU/mL) was diagnosed with rapidly progressive glomerulonephritis based on renal biopsy and treated with immunosuppressive therapy after 10 months of follow-up. The second patient with a low myeloperoxidase-ANCA titer (11 IU/mL) maintained normal kidney function without medication. Two cases showed different clinical course according to ANCA titer.

Keywords

References

  1. Jennette JC. Rapidly progressive crescentic glomerulonephritis. Kidney Int 2003;63:1164-77. https://doi.org/10.1046/j.1523-1755.2003.00843.x
  2. Hauer HA, Bajema IM, Van Houwelingen HC, Ferrario F, Noel LH, Waldherr R, et al. Determinants of outcome in ANCA-associated glomerulonephritis: a prospective clinico-histopathological analysis of 96 patients. Kidney Int 2002;62:1732-42. https://doi.org/10.1046/j.1523-1755.2002.00605.x
  3. Vergunst CE, van Gurp E, Hagen EC, van Houwelingen HC, Hauer HA, Noel LH, et al. An index for renal outcome in ANCA-associated glomerulonephritis. Am J Kidney Dis 2003;41:532-8. https://doi.org/10.1053/ajkd.2003.50115
  4. Booth AD, Almond MK, Burns A, Ellis P, Gaskin G, Neild GH, et al. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis 2003;41:776-84. https://doi.org/10.1016/S0272-6386(03)00025-8
  5. Fussner LA, Hummel AM, Schroeder DR, Silva F, Cartin-Ceba R, Snyder MR, et al. Factors determining the clinical utility of serial measurements of antineutrophil cytoplasmic antibodies targeting proteinase 3. Arthritis Rheumatol 2016;68:1700-10. https://doi.org/10.1002/art.39637
  6. Cohen RA, Brown RS. Microscopic hematuria. N Engl J Med 2003;348:2330-8. https://doi.org/10.1056/NEJMcp012694
  7. Guchelaar NAD, Waling MM, Adhin AA, van Daele PLA, Schreurs MWJ, Rombach SM. The value of anti-neutrophil cytoplasmic antibodies (ANCA) testing for the diagnosis of ANCA-associated vasculitis, a systematic review and meta-analysis. Autoimmun Rev 2021;20:102716.
  8. Gaffo AL. Diagnostic approach to ANCA-associated vasculitides. Rheum Dis Clin North Am 2010;36:491-506. https://doi.org/10.1016/j.rdc.2010.05.009
  9. Hagen EC, Daha MR, Hermans J, Andrassy K, Csernok E, Gaskin G, et al. Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. Kidney Int 1998;53:743-53.
  10. HoubenE,BaxWA, vanDamB,SliekerWAT,VerhaveG, FrerichsFCP, et al. Diagnosing ANCA-associated vasculitis in ANCA positive patients: a retrospective analysis on the role of clinical symptoms and the ANCA titre. Medicine (Baltimore) 2016;95:e5096.
  11. Kerr GS, Fleisher TA, Hallahan CW, Leavitt RY, Fauci AS, Hoffman GS. Limited prognostic value of changes in antineutrophil cytoplasmic antibody titer in patients with Wegener's granulomatosis. Arthritis Rheum 1993;36:365-71. https://doi.org/10.1002/art.1780360312
  12. Baskin E, Bakkaloglu A, Besbas N, Hascelik G, Saatci U, Gok F, et al. Ceruloplasmin levels in antineutrophil cytoplasmic antibody-positive patients. Pediatr Nephrol 2002;17:917-9. https://doi.org/10.1007/s00467-002-0969-0
  13. Jennette JC, Nachman PH. ANCA glomerulonephritis and vasculitis. Clin J Am Soc Nephrol 2017;12:1680-91. https://doi.org/10.2215/cjn.02500317
  14. Jayne DR, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol 2007;18:2180-8. https://doi.org/10.1681/ASN.2007010090
  15. Smith RM, Jones RB, Jayne DR. Progress in treatment of ANCA-associated vasculitis. Arthritis Res Ther 2012;14:210.