A case of immunotactoid glomerulopathy exhibiting nephrotic syndrome successfully treated with corticosteroids and antihypertensive therapy

  • Masaru Kinomura
  • Yohei Maeshima
  • Ryo Kodera
  • Hiroshi Morinaga
  • Daisuke Saito
  • Kazushi Nakao
  • Hiroyuki Yanai
  • Kenei Sada
  • Hitoshi Sugiyama
  • Hirofumi Makino
Case Report

Abstract

We report a case of immunotactoid glomerulopathy (ITG) with cerebral hemorrhage and hypocomplementemia, with successful therapeutic outcome following the corticosteroids and antihypertensive treatment. A 70-year-old man presented with facial edema in October 2006. One day prior to his referral, he experienced speech disturbance, headache, and vomiting, and on the next day he was referred to our hospital. The laboratory examination revealed massive proteinuria (11.3 g/day) and hematuria. The total serum hemolytic complement (CH50) was decreased to 23 U/ml and C4 component was decreased to 7.5 mg/dl, whereas C3 component remained within normal limits (82 mg/dl). Brain computed tomography scan showed high-density lesions in the left parieto-occipital area suggesting subcortical cerebral hemorrhage. Renal biopsy revealed diffuse subendothelial PAS-positive depositions. Immunofluorescence studies revealed intensive deposition of IgG, IgA, C3, C1q, Fibrinogen, and kappa light chain with granular pattern in the capillary and mesangial area. Electron microscopic examination revealed regularly arranged microtubular deposits, appearing as 21–33 nm in diameter. Based on these findings, this patient was diagnosed as ITG complicated with cerebral hemorrhage and hypocomplementemia. He received oral prednisolone (30 mg/day), resulting in reduction of proteinuria, improvement of hypocomplementemia, and prevention of renal functional deterioration. This case demonstrates that accurate diagnosis of ITG may result in successful therapeutic outcome.

Keywords

Immunotactoid glomerulopathy (ITG) Cerebral hemorrhage Hypocomplementemia 

References

  1. 1.
    Mukai K, Kitazawa K, Totsuka D, Saito K, Sugisaki T. A case of immunotactoid glomerulopathy with unusual microtubular deposits. Clin Nephrol. 1998;49:321–4.PubMedGoogle Scholar
  2. 2.
    Minami J, Ishimitsu T, Inenaga T, Ishibashi-Ueda H, Kawano Y, Takishita S. Immunotactoid glomerulopathy: report of a case. Am J Kidney Dis. 1997;30:160–3.CrossRefPubMedGoogle Scholar
  3. 3.
    Kurosu M, Ando Y, Takeda S, Kusano E, Sakurai T, Kuriki K, et al. Immunotactoid glomerulopathy characterized by steroid-responsive massive subendothelial deposition. Am J Kidney Dis. 2001;37:E21.CrossRefPubMedGoogle Scholar
  4. 4.
    Kurihara I, Saito T, Sato H, Chiba J, Saito J, Soma J, et al. Successful treatment with steroid pulse therapy in a case of immunotactoid glomerulopathy with hypocomplementemia. Am J Kidney Dis. 1998;32:E4.CrossRefPubMedGoogle Scholar
  5. 5.
    Kawashima M, Horita S, Nakayama H, Honda K, Uchida K, Nitta K, et al. Immunoelectron microscopic analysis of intraglomerular deposits in IgA-dominant immunotactoid glomerulopathy. Rinsho Byori. 2002;50:1085–9.PubMedGoogle Scholar
  6. 6.
    Fukuda M, Morozumi K, Oikawa T, Motokawa M, Usami T, Yoshida A, et al. Immunotactoid glomerulopathy with microtubular deposits, with reference to the characteristics of Japanese cases. Clin Nephrol. 2005;63:368–74.PubMedGoogle Scholar
  7. 7.
    Abe F, Sano K, Utsunomiya Y, Tokutome G, Kawamura T, Kawaguchi Y, et al. Case of immunotactoid glomerulopathy (ITG) associated with acute active glomerular lesion. Nippon Naika Gakkai Zasshi. 1999;88:1527–9.PubMedGoogle Scholar
  8. 8.
    Rosenstock JL, Markowitz GS, Valeri AM, Sacchi G, Appel GB, D’Agati VD. Fibrillary and immunotactoid glomerulonephritis: distinct entities with different clinical and pathologic features. Kidney Int. 2003;63:1450–61.CrossRefPubMedGoogle Scholar
  9. 9.
    Korbet SM, Schwartz MM, Rosenberg BF, Sibley RK, Lewis EJ. Immunotactoid glomerulopathy. Medicine (Baltimore). 1985;64:228–43.Google Scholar
  10. 10.
    Alpers CE. Immunotactoid (microtubular) glomerulopathy: an entity distinct from fibrillary glomerulonephritis? Am J Kidney Dis. 1992;19:185–91.PubMedGoogle Scholar
  11. 11.
    Ivanyi B, Degrell P. Fibrillary glomerulonephritis and immunotactoid glomerulopathy. Nephrol Dial Transplant. 2004;19:2166–70.CrossRefPubMedGoogle Scholar
  12. 12.
    Schwartz MM, Korbet SM, Lewis EJ. Immunotactoid glomerulopathy. J Am Soc Nephrol. 2002;13:1390–7.CrossRefPubMedGoogle Scholar
  13. 13.
    Bridoux F, Hugue V, Coldefy O, Goujon JM, Bauwens M, Sechet A, et al. Fibrillary glomerulonephritis and immunotactoid (microtubular) glomerulopathy are associated with distinct immunologic features. Kidney Int. 2002;62:1764–75.CrossRefPubMedGoogle Scholar
  14. 14.
    Schifferli JA, Merot Y, Cruchaud A, Chatelanat F. Immunotactoid glomerulopathy with leucocytoclastic skin vasculitis and hypocomplementemia: a case report. Clin Nephrol. 1987;27:151–5.PubMedGoogle Scholar
  15. 15.
    Da’as N, Kleinman Y, Polliack A, Amir G, Ne’eman Z, Kopolovic J, et al. Immunotactoid glomerulopathy with massive bone marrow deposits in a patient with IgM kappa monoclonal gammopathy and hypocomplementemia. Am J Kidney Dis. 2001;38:395–9.CrossRefPubMedGoogle Scholar
  16. 16.
    Mazzola L, Antoine JC, Camdessanche JP, Barral FG, Reynaud J, Michel D. Brain hemorrhage as a complication of type I cryoglobulinemia vasculopathy. J Neurol. 2003;250:1376–8.CrossRefPubMedGoogle Scholar
  17. 17.
    Ishii N, Nishihara Y, Horie A. Amyloid angiopathy and lobar cerebral haemorrhage. J Neurol Neurosurg Psychiatry. 1984;47:1203–10.CrossRefPubMedGoogle Scholar
  18. 18.
    Korbet SM, Schwartz MM, Lewis EJ. The fibrillary glomerulopathies. Am J Kidney Dis. 1994;23:751–65.PubMedGoogle Scholar
  19. 19.
    Kyle RA, Gertz MA. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Semin Hematol. 1995;32:45–59.PubMedGoogle Scholar

Copyright information

© Japanese Society of Nephrology 2009

Authors and Affiliations

  • Masaru Kinomura
    • 1
  • Yohei Maeshima
    • 1
  • Ryo Kodera
    • 1
  • Hiroshi Morinaga
    • 1
  • Daisuke Saito
    • 1
  • Kazushi Nakao
    • 1
  • Hiroyuki Yanai
    • 2
  • Kenei Sada
    • 1
  • Hitoshi Sugiyama
    • 3
  • Hirofumi Makino
    • 1
  1. 1.Department of Medicine and Clinical ScienceOkayama University Graduate School of Medicine, Dentistry and Pharmaceutical SciencesOkayamaJapan
  2. 2.Department of PathologyOkayama University Graduate School of Medicine, Dentistry and Pharmaceutical SciencesOkayamaJapan
  3. 3.Center for Chronic Kidney Disease and Peritoneal DialysisOkayama University Graduate School of Medicine, Dentistry and Pharmaceutical SciencesOkayamaJapan

Personalised recommendations