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Nephritis-associated plasmin receptor (NAPlr)-positive glomerulonephritis in a case of ANCA-negative small vessel vasculitis

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Abstract

A 75-year-old man with fever was diagnosed with alveolar hemorrhage. Antineutrophil cytoplasmic antibodies for myeloperoxidase and proteinase 3 were absent. He received corticosteroid therapy, which immediately improved his symptoms and chest radiological findings. After the discontinuation of corticosteroids, fever and general fatigue relapsed, and renal function deteriorated with hematuria and proteinuria. A nerve conduction study revealed mononeuritis multiplex. Renal biopsy demonstrated focal necrotizing crescentic glomerulonephritis with endocapillary proliferative lesions, immunofluorescence C3 deposits, and electron-microscopic subepithelial hump-like deposits. Nephritis-associated plasmin receptor (NAPlr) and plasmin activity, biomarkers of infection-related glomerulonephritis, were positive in glomeruli. Although pathological findings suggested infection-related glomerulonephritis (IRGN), clinical manifestations, such as alveolar hemorrhage and mononeuritis multiplex, suggested systemic small vessel vasculitis. After corticosteroid therapy, systemic symptoms disappeared, and the gradual amelioration of hematuria and proteinuria was observed. Based on the clinical symptoms for which steroid therapy was effective, the patient was considered to have systemic small vessel vasculitis, the etiology of which may have been associated with infection.

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References

  1. Yamakami K, Yoshizawa N, Wakabayashi K, Takeuchi A, Tadakuma T, Boyle MD. The potential role for nephritis-associated plasmin receptor in acute poststreptococcal glomerulonephritis. Methods. 2000;21:185–97.

    Article  CAS  Google Scholar 

  2. Yoshizawa N, Yamakami K, Fujino M, Oda T, Tamura K, Matsumoto K, et al. Nephritis-associated plasmin receptor and acute poststreptococcal glomerulonephritis: characterization of the antigen and associated immune response. J Am Soc Nephrol. 2004;15:1785–93.

    Article  CAS  Google Scholar 

  3. Oda T, Yoshizawa N, Yamakami K, Sakurai Y, Takechi H, Yamamoto K, et al. The role of nephritis-associated plasmin receptor (NAPlr) in glomerulonephritis associated with streptococcal infection. J Biomed Biotechnol. 2012. https://doi.org/10.1155/2012/417675.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Odaka J, Kanai T, Ito T, Saito T, Aoyagi J, Betsui H, et al. A case of post-pneumococcal acute glomerulonephritis with glomerular depositions of nephritis-associated plasmin receptor. CEN Case Rep. 2015;4:112–6.

    Article  Google Scholar 

  5. Hirano D, Oda T, Ito A, Yamada A, Kakegawa D, Miwa S, et al. Glyceraldehyde-3-phosphate dehydrogenase of Mycoplasma pneumoniae induces infection-related glomerulonephritis. Clin Nephrol. 2019;92:263–72.

    Article  Google Scholar 

  6. Uchida T, Oda T. Glomerular deposition of nephritis-associated plasmin receptor (naplr) and related plasmin activity: key diagnostic biomarkers of bacterial infection-related glomerulonephritis. Int J Mol Sci. 2020;21:2595.

    Article  CAS  Google Scholar 

  7. Chen M, Kallenberg CG, Zhao MH. ANCA-negative pauci-immune crescentic glomerulonephritis. Nat Rev Nephrol. 2009;5:313–8.

    Article  CAS  Google Scholar 

  8. Jennette JC, Nachman PH. ANCA glomerulonephritis and vasculitis. Clin J Am Soc Nephrol. 2017;12:1680–91.

    Article  Google Scholar 

  9. Falk RJ, Jennette JC. ANCA small-vessel vasculitis. J Am Soc Nephrol. 1997;8:314–22.

    Article  CAS  Google Scholar 

  10. Sethi S, Zand L, De Vriese AS, Specks U, Vrana JA, Kanwar S, et al. Complement activation in pauci-immune necrotizing and crescentic glomerulonephritis: results of a proteomic analysis. Nephrol Dial Transplant. 2017;32:i139–45.

    Article  CAS  Google Scholar 

  11. Nasr SH, Fidler ME, Valeri AM, Cornell LD, Sethi S, Zoller A, et al. Postinfectious glomerulonephritis in the elderly. J Am Soc Nephrol. 2011;22:187–95.

    Article  Google Scholar 

  12. Nasr SH, Radhakrishnan J, D’Agati VD. Bacterial infection-related glomerulonephritis in adults. Kidney Int. 2013;83:792–803.

    Article  CAS  Google Scholar 

  13. Kikuchi Y, Yoshizawa N, Oda T, Imakiire T, Suzuki S, Miura S. Streptococcal origin of a case of Henoch-Schoenlein purpura nephritis. Clin Nephrol. 2006;65:124–8.

    Article  CAS  Google Scholar 

  14. Sawanobori E, Umino A, Kanai H, Matsushita K, Iwasa S, Kitamura H, et al. A prolonged course of group A streptococcus-associated nephritis: a mild case of dense deposit disease (DDD)? Clin Nephrol. 2009;71:703–7.

    Article  CAS  Google Scholar 

  15. Kohatsu K, Suzuki T, Yazawa M, Yahagi K, Ichikawa D, Koike J, et al. Granulomatosis With polyangiitis induced by infection. Kidney Int Rep. 2019;4:341–5.

    Article  Google Scholar 

  16. Yano K, Suzuki H, Oda T, Ueda Y, Tsukamoto T, Muso E. Crescentic poststreptococcal acute glomerulonephritis accompanied by small vessel vasculitis: case report of an elderly male. BMC Nephrol. 2019;20:471.

    Article  CAS  Google Scholar 

  17. Traverso F, Martini F, Banchi L, Maritato F, Fazio B. Vasculitic neuropathy associated with beta-haemolytic streptococcal infection: a case report. Ital J Neurol Sci. 1997;18:105–7.

    Article  CAS  Google Scholar 

  18. Yoshida M, Yamakawa H, Yabe M, Ishikawa T, Takagi M, Matsumoto K, et al. Diffuse alveolar hemorrhage in a patient with acute poststreptococcal glomerulonephritis caused by impetigo. Intern Med. 2015;54:961–4.

    Article  Google Scholar 

  19. Okabe M, Takamura T, Tajiri A, Tsuboi N, Ishikawa M, Ogura M, et al. A case of infection-related glomerulonephritis with massive eosinophilic infiltration. Clin Nephrol. 2018;90:142–7.

    Article  Google Scholar 

  20. Al-Hussain T, Hussein MH, Conca W, Al Mana H, Akhtar M. Pathophysiology of ANCA-associated vasculitis. Adv Anat Pathol. 2017;24:226–34.

    Article  Google Scholar 

  21. Kessenbrock K, Krumbholz M, Schönermarck U, Back W, Gross WL, Werb Z, et al. Netting neutrophils in autoimmune small-vessel vasculitis. Nat Med. 2009;15:623–5.

    Article  CAS  Google Scholar 

  22. Kain R, Exner M, Brandes R, Ziebermayr R, Cunningham D, Alderson CA, et al. Molecular mimicry in pauci-immune focal necrotizing glomerulonephritis. Nat Med. 2008;14:1088–96.

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Division of Nephrology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kamigyo-ku, Kyoto, 602-8566, Japan

    Kazumi Komaki, Yayoi Shiotsu, Hiroya Adachi, Noriko Urata, Masayuki Hara, Mayuka Nakayama, Tetsuro Kusaba & Keiichi Tamagaki

  2. Division of Surgical Pathology, Department of Pathology, Kyoto Prefectural University of Medicine, Kyoto, Japan

    Naoko Masuzawa & Eiichi Konishi

  3. Department of Diagnostic Pathology, Otsu City Hospital, Shiga, Japan

    Naoko Masuzawa

  4. Department of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, Tokyo, Japan

    Takashi Oda

Authors
  1. Kazumi Komaki
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  2. Yayoi Shiotsu
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  3. Hiroya Adachi
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  4. Noriko Urata
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  5. Masayuki Hara
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  6. Mayuka Nakayama
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  7. Tetsuro Kusaba
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  8. Naoko Masuzawa
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  9. Eiichi Konishi
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  10. Takashi Oda
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  11. Keiichi Tamagaki
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Corresponding author

Correspondence to Yayoi Shiotsu.

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Komaki, K., Shiotsu, Y., Adachi, H. et al. Nephritis-associated plasmin receptor (NAPlr)-positive glomerulonephritis in a case of ANCA-negative small vessel vasculitis. CEN Case Rep 11, 90–96 (2022). https://doi.org/10.1007/s13730-021-00635-5

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  • DOI: https://doi.org/10.1007/s13730-021-00635-5

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