Serum IgA/C3 Ratio May Be a Useful Marker of Disease Activity in Severe Henoch-Schönlein Nephritis

Background/Aims: This study was designed to investigate whether the serum IgA/C3 ratio can be a serologic marker of disease activity in children with severe Henoch-Schönlein nephritis (HSN). Methods: Twelve HSN patients who were treated with steroids and cyclosporine were examined. The levels of serum IgA and C3 were measured using an international reference preparation (IFCC/CRM470) and a renal biopsy was performed in all patients before and after therapy. After therapy, patients were divided into 3 groups: complete remission (n = 6, group I), mild urinary abnormalities (n = 3, group II), and active renal disease (n = 3, group III). Results: The serum IgA/C3 ratio decreased significantly in groups I and II after therapy (2.62 ± 0.82 vs. 2.02 ± 0.52, p = 0.02), whereas the ratio in group III increased, although it was not statistically significant (2.13 ± 0.93 vs. 4.67 ± 1.71, p = 0.25). A follow-up renal biopsy revealed that the activity index was reduced in groups I and II (7.0 ± 2.4 vs. 3.6 ± 1.6, p = 0.016), and not changed in group III (7.3 ± 2.1 vs. 9.3 ± 2.5, p = 0.25). The activity index at a follow-up renal biopsy correlated positively with the changes of the serum IgA/C3 ratio: posttherapy activity index = 1.20 × ΔIgA/C3 + 4.78 (r = 0.635, p = 0.027); where ΔIgA/C3 is posttherapy IgA/C3 – pretherapy IgA/C3. Conclusion: These findings suggest that the serum IgA/C3 ratio may be a useful marker to predict disease activity and histologic severity in HSN.

[1]  J. Knight The rheumatic poison: a survey of some published investigations of the immunopathogenesis of Henoch-Schönlein purpura , 1990, Pediatric Nephrology.

[2]  S. Andreoli,et al.  Treatment of severe IgA nephropathy in children , 1989, Pediatric Nephrology.

[3]  M. Nuutinen,et al.  Cyclosporin A for the treatment of severe Henoch-Schönlein glomerulonephritis , 2003, Pediatric Nephrology.

[4]  T. Jahnukainen,et al.  Outcome of Henoch-Schoenlein nephritis with nephrotic-range proteinuria. , 2003, Clinical nephrology.

[5]  Y. Tomino,et al.  Clinical guidelines for immunoglobulin A (IgA) nephropathy in Japan, second version , 2003, Journal of Clinical and Experimental Nephrology.

[6]  Y. Tomino,et al.  Significance of serum IgA levels and serum IgA/C3 ratio in diagnostic analysis of patients with IgA nephropathy , 2003, Journal of clinical laboratory analysis.

[7]  C. Edelmann,et al.  Henoch-Schönlein purpura nephritis: course of disease and efficacy of cyclophosphamide , 2003, Pediatric Nephrology.

[8]  M. Nuutinen,et al.  The adult kidney 24 years after childhood Henoch-Schönlein purpura: a retrospective cohort study , 2002, The Lancet.

[9]  Y. Tomino,et al.  Serum IgA/C3 Ratio May Predict Diagnosis and Prognostic Grading in Patients with IgA Nephropathy , 2002, Nephron.

[10]  I. T. Ten Berge,et al.  What is the difference between IgA nephropathy and Henoch-Schönlein purpura nephritis? , 2001, Kidney international.

[11]  C. Bernard,et al.  Effective therapy for severe Henoch-Schonlein purpura nephritis with prednisone and azathioprine: a clinical and histopathologic study. , 2000, The Journal of pediatrics.

[12]  P. Rieu,et al.  Henoch-Schönlein nephritis in children and adults. Morphological features and clinicopathological correlations. , 1999, Annales de medecine interne.

[13]  P. Niaudet,et al.  Methylprednisolone pulse therapy in the treatment of severe forms of Schönlein-Henoch purpura nephritis , 1998, Pediatric Nephrology.

[14]  J. Feehally,et al.  Abnormal IgA glycosylation in Henoch-Schönlein purpura restricted to patients with clinical nephritis. , 1998, Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.

[15]  E. Holme,et al.  Complement activation in Henoch-Schönlein purpura , 1997, Pediatric Nephrology.

[16]  J. Floege,et al.  Activated complement C3: a potentially novel predictor of progressive IgA nephropathy. , 1997, Kidney international.

[17]  J. Davin Pathogenesis of IgA nephropathy. , 1995, Acta clinica Belgica.

[18]  G. Müller,et al.  The long-term prognosis of the primary glomerulonephritides. A morphological and clinical analysis of 1747 cases. , 1992, Pathology, Research and Practice.

[19]  R. White,et al.  Long-term follow-up of childhood Henoch-Schönlein nephritis , 1992, The Lancet.

[20]  A. Gressner,et al.  Measurement of proteins with the Behring Nephelometer. A multicentre evaluation. , 1989, Journal of clinical chemistry and clinical biochemistry. Zeitschrift fur klinische Chemie und klinische Biochemie.

[21]  J. Davin,et al.  Sequential Measurements of the Reticulo‐Endothelial System Function in Henoch‐Schönlein Disease of Childhood: Correlations with Various Immunological Parameters , 1985, Acta paediatrica Scandinavica.

[22]  J. Balow,et al.  Henoch-Schönlein nephritis: prognostic features and the challenge of therapy. , 1983, American journal of kidney diseases : the official journal of the National Kidney Foundation.

[23]  C. Chantler,et al.  Serum complement components in Henoch-Schönlein purpura. , 1978, Archives of disease in childhood.

[24]  R. Derham Prognosis of Henoch-Schönlein nephritis in children. , 1977, British medical journal.

[25]  S. R. Meadow,et al.  Schönlein-Henoch nephritis. , 1972, The Quarterly journal of medicine.