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Pediatric Nephrology

, Volume 8, Issue 6, pp 752–753 | Cite as

Sequential occurrence of IgA nephropathy and Henoch-Schönlein purpura: support for common pathogenesis

  • Douglas M. Silverstein
  • Ira Greifer
  • Vaughn Folkert
  • Boyce Bennett
  • Howard E. Corey
  • Adrian Spitzer
Brief Report

Abstract

We report a patient who developed Henoch-Schönlein purpura (HSP) 13 years after he presented with IgA nephropathy (IgAN). In both HSP and IgAN renal biopsy most commonly reveals focal proliferative glomerulonephritis on light microscopy and immunofluorescence displays mesangial IgA deposits. In addition, patients with HSP or IgAN have elevated serum IgA levels, circulating IgA immune complexes, IgA-bearing lymphocytes, immunoglobulin-producing cells, and binding of IgG to glomerular components of similar molecular weight. The occurrence of both diseases in the same patient or the same families and the presence of immune abnormalities compatible with HSP or IgAN in relatives of patients with these diseases suggest a common pathogenesis.

Key words

Henoch-Schönlein purpura IgA nephropathy Familial Immunological Pathogenesis 

References

  1. 1.
    Henoch EH (1874) Veber eine eigenthumliche Form von Purpura. Berl Klin Wochenschr 11:641–643Google Scholar
  2. 2.
    Meadow SR (1972) Schönlein-Henoch purpura. Q J Med 163:241–258Google Scholar
  3. 3.
    Meadow SR (1992) Schönlein-Henoch purpura. In: Edelman CM Jr (ed) Pediatric kidney disease, 2nd edn. Little, Brown and Company, Burton/Toronto/London, pp 1525–1533Google Scholar
  4. 4.
    Koskimies O, Mir S, Rapola J, Vilksa J (1981) Henoch-Schönlein nephritis: long term prognosis of unselected patients. Arch Dis Child 56:482–484Google Scholar
  5. 5.
    Michalk D, Waldherr E, Seelig HP, Weber HP, Schoren K (1980) Idiopathic mesangial-IgA glomerulonephritis in childhood. Eur J Pediatr 134: 13–22Google Scholar
  6. 6.
    Southwest Pediatric Nephrology Study Group (1982) A multicenter study of IgA nephropathy in children. Kidney Int 22:643–652Google Scholar
  7. 7.
    Cream J (1970) Schönlein-Henoch purpura in the adult. Q J Med 156: 461–484Google Scholar
  8. 8.
    Nakomoto Y, Asano Y, Dohi K, Fujioka M, Iida H, Kida H, Kibe Y, Haltori N, Takeuchi J (1978) Primary IgA glomerulonephritis and Schönlein-Henoch purpura: clinicopathological and immunohistological characteristics. Q J Med 188:495–516Google Scholar
  9. 9.
    Coppo R, Basolo B, Martina G, Rollino C, DeMarchi M, Gracchino F, Mazzucco G, Messina M, Piccoli G (1981) Circulating immune complexes containing IgA, IgG and IgM in patients with primary IgA nephropathy and Henoch-Schönlein nephritis. Correlation with clinical and histological signs of activity. Clin Nephrol 18:230–239Google Scholar
  10. 10.
    Trygstad CW, Stiehm ER (1971) Elevated serum IgA globulin in anaphylactoid purpura. Pediatrics 47:1023–1028Google Scholar
  11. 11.
    Kuno-Sakai H, Sakai H, Nomoto Y, Takakura I, Kimura M (1979) Increase of IgA-bearing lymphocytes in children with Henoch-Schönlein purpura. Pediatrics 64:918–922Google Scholar
  12. 12.
    Casanueva B, Rodriguez-Valverde V, Merino J, Arias M, Garcia-Fuentes M (1983) Increased IgA-producing cells in the blood of patients with active Henoch-Schönlein purpura. Arthris Rheum 26:854–860Google Scholar
  13. 13.
    Nomoto Y, Sakai H, Arimori S (1979) Increase of IgA-bearing lymphocytes in peripheral blood from patients with IgA nephropathy. Am J Clin Pathol 79:158–160Google Scholar
  14. 14.
    Casanueva B, Rodriguez-Valverde V, Arias M, Vallo A, Garcia-Fuentes M, Rodriguez-Soriano J (1986) Immunoglobulin-producing cells in IgA nephropathy. Nephron 43:33–37Google Scholar
  15. 15.
    Jen-Jar Lin, Stewart CL, Kaskel FJ, Fine R (1993) IgG and IgA classes of anti-neutrophil cytoplasmic autoantibodies in a 13-year-old girl with recurrent Henoch-Schönlein purpura. Pediatr Nephrol 7:143–146Google Scholar
  16. 16.
    O'Donoghue DJ, Jewkes F, Prosthelwaite RJ, Ballardie FW (1992) Autoimmunity to glomerular antigens in Henoch-Schönlein nephritis. Clin Sci 83:281–287Google Scholar
  17. 17.
    Ballardie FW, Brenchley PEC, Williams S, O'Donoghue DJ (1988) Autoimmunity in IgA nephropathy. Lancet II:588–592Google Scholar
  18. 18.
    Wall Bake AWL van den, Daha MR, Woude FJ van de, Halma C, Schrama E, Es LA van (1989) IgA class anti-neutrophil cytoplasmic antibodies (IgA ANCA) in primary IgA nephropathy. APMIS [Suppl] 6:25–26Google Scholar
  19. 19.
    Meadow SR, Scott DG (1985) Berger disease: Henoch-Schönlein syndrome without the rash. J Pediatr 106:27–32Google Scholar
  20. 20.
    Miyagawa S, Dohi K, Hanatani M, Yamanaka F, Okuchi T, Sakamoto K, Isjikawa H (1989) Anaphylactoid purpura and familial IgA nephropathy. Am J Med 86:340–342Google Scholar

Copyright information

© IPNA 1994

Authors and Affiliations

  • Douglas M. Silverstein
    • 1
  • Ira Greifer
    • 1
  • Vaughn Folkert
    • 2
  • Boyce Bennett
    • 3
  • Howard E. Corey
    • 1
  • Adrian Spitzer
    • 1
  1. 1.Department of Pediatrics, Division of NephrologyAlbert Einstein College of MedicineBronxUSA
  2. 2.Department of Medicine, Division of NephrologyAlbert Einstein College of MedicineBronxUSA
  3. 3.Department of PathologyAlbert Einstein College of MedicineBronxUSA

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