Final Diagnosis -- Henoch-Schöenlein Purpura


FINAL DIAGNOSIS: HENOCH-SCHOENLEIN PURPURA, SEVERE, WITH GLOMERULAR CRESCENT FORMATION

Contributor's Note:

Henoch-Schöenlein Purpura (HSP) is a disease which occurs in childhood; greater than 80% of the patients affected are children between the ages of 5 and 15. HSP can have multiorgan involvement upon presentation. The classic constellation of symptoms and findings includes cutaneous purpura and edema, migratory arthralgias, and spasmodic gastrointestinal pain which can progress to hematochezia, hematemesis, and which may mimic surgical emergencies such as bowel infarction or intussusception; most important in terms of prognosis are the renal manifestations of the disease, which include gross or microscopic hematuria, mild to nephrotic range proteinuria, and possible later progression to chronic renal failure in up to 25% of cases. HSP accounts for up to 3% of terminal renal failure in Europe, but progression to this stage of disease is usually slow, requiring 5 to 10 years for the patient to develop chronic renal insufficiency after a long period of persistent microhematuria and microproteinuria. However, over 50% of affected individuals enjoy complete clinical remission with return of normal renal function indices after an average of 2 years.

Grossly, affected kidneys usually appear normal. Microscopically, HSP affecting the kidney is characterized primarily by glomerular disease, with mesangial and focal and segmental endocapillary proliferation, similar to that seen in classical IgA nephropathy. However, in contrast to this entity, extracapillary proliferation is more commonly seen in HSP nephritis. The phenomenon of mesangial cell "interposition" between the GBM and the endothelial cells, demonstrated on electron microscopy, is a common finding in HSP, and can simulate similar findings in membranoproliferative glomerulonephritis type I. Segmental fibrinoid necrosis can be seen in biopsies of early lesions, and formation of glomerular crescents can be seen in the later stages of more severe disease. These crescents can later contribute to glomerulosclerosis and incite renal failure in some patients.

Immunofluorescence is an important adjunct in the diagnosis of HSP. Examination commonly reveals diffuse mesangial and occasional focal glomerular capillary staining with IgA. Concomitant staining with C3 component of complement is noted in 75-85% of cases, as opposed to IgA nephropathy, in which up to 95% stain for C3.

Electron microscopic examination of these cases reveals mesangial matrix expansion, with electron dense, often "scalloped" or "fluffy" deposits commonly seen in the mesangium. Patients with endocapillary proliferation can show interposition of mesangial cells between the layers of the basement membrane and the endothelial cells of the glomerular tuft.

REFERENCES

  1. Belgiojoso B, Tarantino A, Bazzi C, Colasanti G, Guerra L, Durante A. Immunofluorescence patterns in chronic membranoproliferative glomerulonephritis (MPGN). Clin Nephrol 1976; 6: 303.
  2. Boyce NW, Holdsworth SR, Thomson NM, Atkins RC. Clinicopathological associations in mesangial IgA nephropathy. Am J Nephrol 1986; 6:246.
  3. Fauci AS. The vasculitis syndromes. in Harrison's Principles of Internal Medicine, 13th Ed., Mc Graw Hill, New York, 1994.
  4. Faull RJ, Aarons I, Woodroffe AJ, Clarkson AR. Adult Henoch-Schöenlein nephritis. Aust NZ J Med 1987; 47: 396.
  5. Hall RP, Lawley TJ, Heck JA, Katz SI. IgA-containing immune complexes in dermatitis herpetiformis, Henoch-Schöenlein purpura, systemic lupus
  6. erythematosus and other diseases. Clin Exp Immunol 1980; 40:431.
  7. Glassock RJ, Brenner BM. Glomerulopathies associated with multisystem diseases. in Harrison's Principles of Internal Medicine, 13th Ed., McGraw Hill, New York, 1994.
  8. Levy M, Gubler MC, Sich M, Beziau A, Habib R. New concepts in membranoproliferative glomerulonephritis. In Progress in Glomerulonephritis, Kincaid-Smith P, d'Apice AJ, Atkins RC (eds.), Wiley, New York 1979.
  9. Linne T, Aperia A, Broberger O, Bergstrand A, Bohman SO, Wasserman J. Renal function and biopsy changes during the course of Henoch-Schöenlein glomerulonephritis. Acta Pediatr Scand 1983; 72:104.
  10. Nossent HC et al. Systemic lupus erythematosus after renal transplantation: Patient and graft survival and disease activity. Ann Intern Med 1991; 114: 183.
  11. Shichiri M, Tsutsumi K, Yamamoto I, Ida T, Iwamoto H. Diffuse intrapulmonary hemorrhage and renal failure in adult Henoch-Schöenlein purpura. Am J Nephrol 1987; 7: 140.
  12. Silva FG. Acute postinfectious glomerulonephritis and glomerulonephritis complicating persistent bacterial infection. in Pathology of the Kidney, 4th ed., Heptinstall, editor. Little, Brown, and Co., Boston, 1992.
  13. Silva FG. Membranoproliferative glomerulonephritis. in Pathology of the Kidney, 4th ed., Heptinstall, editor. Little, Brown, and Co., Boston, 1992.

Contributed by Kevin D. Horn, MD. and Paul S. Dickman, MD.

Case IndexCME Case StudiesFeedbackHome