Abstract
Immune complex and complement systems play an important role in membranoproliferative glomerulonephritis (MPGN). X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by severe hypogammaglobulinemia. We report the case of an XLA patient who developed MPGN during an intravenous immunoglobulin (IVIG) treatment. In this patient, the serum IgG level was maintained at more than 400 mg/dl of regular IVIG administration (2.5 g/dose/month). The patient presented with microscopic hematuria, proteinuria (U-pro/Cr: 4.0–4.2) and low serum complement levels (C3: 57.8 mg/dl) 3 years after IVIG treatment and was diagnosed histopathologically as having MPGN type III. Both hematuria and proteinuria significantly improved, and the serum complement level returned to a normal level following methylprednisolone pulse therapy. To our knowledge, this is the first case report of MPGN associated with XLA. Although it is unclear how MPGN occurred in this XLA patient, we suggest that residual humoral immunity in the patient could be associated with the development of MPGN.
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References
Johnson RJ, Alpers CE, Schen P (2000) Membranoproliferative glomerulonephritis and cryoglobulinemic glomerulonephritis. In: Johnson RJ, Feehally J (eds) Comprehensive clinical nephrology, 1st edn. Mosby Company, St Louis, pp1–10
Holley KE, Donadio JV (1994) Membranoproliferative glomerulonephritis. In: Tisher CC, Brenner BM (eds). Renal pathology, with clinical and functional correlations, vol.1, 2nd edn. JB Lippincott, Philadelphia, pp 284–329
Nakopoulou L (2001) Membranoproliferative glomerulonephritis. Nephrol Dial Transplant 16:71–73
Meyers KEC, Finn L, Kaplan BS (1998) Membranoproliferative glomerulonephritis type III. Pediatr Nephrol 12:512–522
Vetrie D, Vorechovsky I, Sideras P, Holland J, Davies A, Flinter F, Hammarstrom L, Kinnon C, Levinsky R, Bobrow M, et al (1993) The gene involved in X-linked agammaglobulinaemia is a member of the src family of protein-tyrosine kinases. Nature 361:226–233
Tsukada S, Saffran DC, Rawlings DJ, Parolini O, Allen RC, Klisak I, Sparkes RS, Kubagawa H, Mohandas T, Quan S, et al (1993) Deficient expression of a B cell cytoplasmic tyrosine kinase in human X-linked agammaglobulinemia. Cell 72:279–290
Nonoyama S, Tsukada S, Yamadori T, Miyawaki T, Jin YZ, Watanabe C, Morio T, Yata J, Ochs HD (1998) Functional analysis of peripheral blood B cells in patients with X-linked agammaglobulinemia. J Immunol 161:3925–3929
Wegmüller E (1998) Effect of intravenous immunoglobulin therapy on plasma complement. Transfus Sci 19:307–318
Acknowledgements
This study was supported by grants from the Ministry of Education, Culture, Sports, Science and Technology of Japan, and the Ministry of Health, Labor and Welfare of Japan.
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Yoshino, A., Honda, M., Kanegane, H. et al. Membranoproliferative glomerulonephritis in a patient with X-linked agammaglobulinemia. Pediatr Nephrol 21, 36–38 (2006). https://doi.org/10.1007/s00467-005-2029-z
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DOI: https://doi.org/10.1007/s00467-005-2029-z