Abstract
We report a case of Henoch-Schönlein purpura nephritis (HSPN) with acquired IgA deficiency due to parvovirus B19 infection. The patient was diagnosed as having Henoch-Schönlein purpura (HSP) at 6 years old, and subsequently developed macrohematuria and massive proteinuria of 7.4 g/day with decreased creatinine clearance of 70.2 ml/min/1.73 m2 and significantly elevated serum IgA level of 449 mg/dl. The first kidney biopsy yielded the diagnosis of severe HSPN. After the initiation of the immunosuppressive therapy, the patient was infected with parvovirus B19 and developed virus-associated hemophagocytic syndrome (VAHS). Thereafter, the serum level of IgA selectively decreased and remained undetectable until the present time. Repeated kidney biopsies performed over a period of 14 years revealed a remarkable histological improvement in association with stabilization of the patient's kidney function. Considering the severity of initial kidney injury, persistent acquired IgA deficiency was likely to add favorable effects to the immunosuppressive therapy in this patient with HSPN.
References
Schärer K, Krmar R, Querfeld U, Ruder H, Waldherr R, Schaefer F (1999) Clinical outcome of Schönlein-Henoch purpura nephritis in children. Pediatr Nephrol 13:816–823
International Study of Kidney Disease in Children (1978) Nephrotic syndrome in children: prediction of histopathology from clinical and laboratory characteristics at time of diagnosis. Kidney Int 13:159–165
Créput C, Galicier L, Buyse S, Azoulay E (2008) Understanding organ dysfunction in hemophagocytic lymphohistiocytosis. Intensive Care Med 34:1177–1187
Gleeson M, Clancy RL, Cripps AW, Henry RL, Hensley MJ, Wlodarczyk JH (1994) Acquired IgA deficiency. Pediatr Allergy Immunol 5:157–161
Iijima K, Ito-Kariya S, Nakamura H, Yoshikawa N (1998) Multiple combined therapy for severe Henoch-Schönlein nephritis in children. Pediatr Nephrol 12:244–248
Yoshikawa N, Ito H, Sakai T, Takekoshi Y, Honda M, Awazu M, Ito K, Iitaka K, Koitabashi Y, Yamaoka K, Nakagawa K, Nakamura H, Matsuyama S, Seino Y, Takeda N, Hattori S, Ninomiya M (1999) A controlled trial of combined therapy for newly diagnosed severe childhood IgA nephropathy. The Japanese Pediatric IgA Nephropathy Treatment Study Group. J Am Soc Nephrol 10:101–109
Yoshikawa N, Iijima K, Matsuyama S, Suzuki J, Kameda A, Okada S, Nakamura H (1990) Repeat renal biopsy in children with IgA nephropathy. Clin Nephrol 33:160–167
Lamm ME, Emancipator SN, Robinson JK, Yamashita M, Fujioka H, Qiu J, Plaut AG (2008) Microbial IgA protease removes IgA immune complexes from mouse glomeruli in vivo: potential therapy for IgA nephropathy. Am J Pathol 172:31–36
Author information
Authors and Affiliations
Corresponding authors
Rights and permissions
About this article
Cite this article
Tanaka, M., Seki, G., Ishizawa, K. et al. Resolution of Henoch-Schönlein purpura nephritis after acquired IgA deficiency. Pediatr Nephrol 25, 2355–2358 (2010). https://doi.org/10.1007/s00467-010-1568-0
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00467-010-1568-0