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A rare case of atypical ANCA-associated vasculitis without crescents overlapping with invasive pulmonary aspergillosis, successfully treated to remission with intravenous immunoglobulin therapy

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Abstract

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is life-threatening without treatment, but aggressive immunosuppression increases the risk of exacerbating a coexisting infection. Finding the balance between efficacy and safety of immunosuppression is challenging. We describe a 74-year-old man who was diagnosed with AAV following the aggravation of chronic pulmonary aspergillosis that required an aggressive antifungal agent. The laboratory data on admission demonstrated severe kidney failure requiring hemodialysis. Due to the active infection, we chose intravenous immunoglobulin (IVIg) as a low-risk initial treatment, which remarkably improved renal dysfunction (serum creatinine; 16.7 mg/dL–3.7 mg/dL) and systemic inflammation. Renal biopsy that was performed after renal recovery revealed atypical ANCA-associated nephritis without cellular crescents but with massive arteritis with multiple vascular sizes and diffuse interstitial inflammation. Despite these active AAV findings, adding plasma exchange therapy (PE) and low-dose steroids were sufficient to induce remission. The main pathogenesis of severe renal impairment was probably the reduction of blood flow, resulting from occlusions of small arteries by inflammatory cell infiltration and vascular endothelial injury due to AAV. Combination treatment with antifungal agents, IVIg, PE, and low-dose steroid treatment led to complete resolution of vasculitis. The specific histological findings and the good response to treatments suggest that pulmonary aspergillosis might trigger vasculitis through induction of ANCA antigen expression. IVIg could be an important option especially for cases of AAV associated with pulmonary aspergillosis.

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References

  1. Hellmich B, Flossmann O, Gross WL, Bacon P, Cohen-Tervaert JW, Guillevin L, et al. EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis. 2007;66:605–17.

    Article  PubMed  CAS  Google Scholar 

  2. Limper AH, Knox KS, Sarosi GA, Ampel NM, Bennett JE, Catanzaro A, et al. An official American Thoracic Society statement: treatment of fungal infections in adult pulmonary and critical care patients. Am J Respir Crit Care Med. 2011;183:96–128.

    Article  PubMed  CAS  Google Scholar 

  3. Menez S, Hruskova Z, Scott J, Cormican S, Chen M, Salama AD, et al. Predictors of renal outcomes in sclerotic class anti-neutrophil dytoplasmic antibody glomerulonephritis. Am J Nephrol. 2018;48:465–71.

    Article  PubMed  CAS  Google Scholar 

  4. Hassan TM, Hassan AS, Igoe A, Logan M, Gunaratnam C, McElvaney NG, et al. Lung involvement at presentation predicts disease activity and permanent organ damage at 6, 12 and 24 months follow-up in ANCA-associated vasculitis. BMC Immunol. 2014;15:20.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S, et al. Modification and validation of the Birmingham vasculitis activity score (version 3). Ann Rheum Dis. 2009;68:1827–32.

    Article  PubMed  CAS  Google Scholar 

  6. Kitagawa K, Furuichi K, Sagara A, Shinozaki Y, Kitajima S, Toyama T, et al. Risk factors associated with relapse or infectious complications in Japanese patients with microscopic polyangiitis. Clin Exp Nephrol. 2016;20:703–11.

    Article  PubMed  Google Scholar 

  7. Ito-Ihara T, Ono T, Nogaki F, Suyama K, Tanaka M, Yonemoto S, et al. Clinical efficacy of intravenous immunoglobulin for patients with MPO-ANCA-associated rapidly progressive glomerulonephritis. Nephron Clin Pract. 2006;102:c35-42.

    Article  PubMed  CAS  Google Scholar 

  8. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. Revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65:1–11.

    Article  PubMed  CAS  Google Scholar 

  9. Furusho K, Kamiya T, Nakano H, Kiyosawa N, Shinomiya K, Hayashidera T, et al. High-dose intravenous gammaglobulin for Kawasaki disease. Lancet. 1984;2:1055–8.

    Article  PubMed  CAS  Google Scholar 

  10. Watanabe T. Clinical features of acute kidney injury in patients with Kawasaki disease. World J Clin Pediatr. 2018;7(3):83–8.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Dumestre-Pérard C, Lamy B, Aldebert D, Lemaire-Vieille C, Grillot R, Brion JP, et al. Aspergillus conidia activate the complement by the mannan-binding lectin C2 bypass mechanism. J Immunol. 2008;181:7100–5.

    Article  PubMed  Google Scholar 

  12. Meng XC, Jiang T, Yi SH, Xie PY, Guo YF, Quan L, et al. Renal aspergillosis after liver transplantation: clinical and imaging manifestations in two cases. World J Gastroenterol. 2014;20:18495–502.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Park J, Banno S, Sugiura Y, Yoshikawa K, Naniwa T, Wakita K, et al. Microscopic polyangiitis associated with diffuse panbronchiolitis. Intern Med. 2004;43:331–5.

    Article  PubMed  Google Scholar 

  14. Asano S, Mizuno S, Okachi S, Aso H, Wakahara K, Hashimoto N, et al. Antineutrophil cytoplasmic antibody-associated vasculitis superimposed on infection-related glomerulonephritis secondary to pulmonary Mycobacterium avium complex infection. Intern Med. 2016;55:2439–45.

    Article  PubMed  Google Scholar 

  15. Nagata T, Hara K, Oka T, Yamaguchi Y, Igarashi K, Hazama H, et al. Microscopic polyangiitis associated with sinobronchial syndrome. Intern Med. 2001;40:544–7.

    Article  PubMed  CAS  Google Scholar 

  16. Sitara D, Hoffbrand BI. Chronic bronchial suppuration and antineutrophil cytoplasmic antibody (ANCA) positive systemic vasculitis. Postgrad Med J. 1990;66:669–71.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  17. Saku N, Sugiyama Y, Kitamura S, Fujii T. Diffuse panbronchiolitis with P-ANCA-positive arteritis and necrotizing glomerulitis. Nihon Kyobu Shikkan Gakkai Zasshi. 1996;34:434–8.

    PubMed  CAS  Google Scholar 

  18. Bruce IN, McAteer JA, Gardiner PV, McFarland RJ, Sloan JM, Bell AL. Chronic suppurative lung disease with associated vasculitis. Postgrad Med J. 1995;71:24–7.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  19. Geetha D, Jin Q, Scott J, Hruskova Z, Hanouneh M, Little MA, et al. Comparisons of guidelines and recommendations on managing antineutrophil cytoplasmic antibody-associated vasculitis. Kidney Int Rep. 2018;3:1039–49.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Crickx E, Machelart I, Lazaro E, Kahn JE, Cohen-Aubart F, Martin T, et al. Intravenous immunoglobulin as an immunomodulating agent in antineutrophil cytoplasmic antibody-associated vasculitides: a French nationwide study of ninety-two patients. Arthritis Rheum. 2016;68:702–12.

    Article  CAS  Google Scholar 

  21. Martino R, Lopez R, Sureda A, Brunet S, Domingo-Albós A. Risk of reactivation of a recent invasive fungal infection in patients with hematological malignancies undergoing further intensive chemo-radiotherapy. A single-center experience and review of the literature. Haematologica. 1997;82:297–304.

    PubMed  CAS  Google Scholar 

  22. Gelfand EW. Intravenous immune globulin in autoimmune and inflammatory diseases. N Engl J Med. 2013;368:777.

    PubMed  CAS  Google Scholar 

  23. Turhan A, Jenab P, Bruhns P, Ravetch JV, Coller BS, Frenette PS. Intravenous immune globulin prevents venular vaso-occlusion in sickle cell mice by inhibiting leukocyte adhesion and the interactions between sickle erythrocytes and adherent leukocytes. Blood. 2004;103:2397–400.

    Article  PubMed  CAS  Google Scholar 

  24. Chang J, Shi PA, Chiang EY, Frenette PS. Intravenous immunoglobulins reverse acute vaso-occlusive crises in sickle cell mice through rapid inhibition of neutrophil adhesion. Blood. 2008;111:915–23.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  25. 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  PubMed  PubMed Central  CAS  Google Scholar 

  26. Röhm M, Grimm MJ, D’Auria AC, Almyroudis NG, Segal BH, Urban CF. NADPH oxidase promotes neutrophil extracellular trap formation in pulmonary aspergillosis. Infect Immun. 2014;82:1766–77.

    Article  PubMed  PubMed Central  Google Scholar 

  27. Uozumi R, Iguchi R, Masuda S, Nishibata Y, Nakazawa D, Tomaru U, et al. Pharmaceutical immunoglobulins reduce neutrophil extracellular trap formation and ameliorate the development of MPO-ANCA-associated vasculitis. Mod Rheumatol. 2020;30:544–50.

    Article  PubMed  CAS  Google Scholar 

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Acknowledgements

The authors would like to thank Dr. Shoko Ohno for valuable discussion regarding the manuscript.

Author information

Author notes

  1. Keisuke Morita and Shinya Yamamoto have contributed equally to this work.

Authors and Affiliations

  1. Department of Nephrology, Graduate School of Medicine, Kyoto University, Shogoin-Kawahara-cho 54, Sakyo-ku, Kyoto, 606-8507, Japan

    Keisuke Morita, Shinya Yamamoto, Marina Ueda, Keisuke Taniguchi & Motoko Yanagita

  2. Department of Diagnostic Imaging and Nuclear Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan

    Hirotsugu Nakai

  3. Department of Diagnostic Pathology, Graduate School of Medicine, Kyoto University, Kyoto, Japan

    Sachiko Minamiguchi

  4. Division of Nephrology and Dialysis, Kitano Hospital, Osaka, Japan

    Eri Muso

  5. Department of Food and Nutrition, Faculty of Contemporary Home Economics, Kyoto Kacho University, Kyoto, Japan

    Eri Muso

  6. Institute for the Advanced Study of Human Biology (ASHBi), Kyoto University, Kyoto, Japan

    Motoko Yanagita

Authors
  1. Keisuke Morita
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  2. Shinya Yamamoto
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  3. Marina Ueda
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  4. Keisuke Taniguchi
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Keisuke Morita and Shinya Yamamoto have contributed equally to this work.

Corresponding author

Correspondence to Shinya Yamamoto.

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This case report was written in compliance with the Declaration of Helsinki.

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Morita, K., Yamamoto, S., Ueda, M. et al. A rare case of atypical ANCA-associated vasculitis without crescents overlapping with invasive pulmonary aspergillosis, successfully treated to remission with intravenous immunoglobulin therapy. CEN Case Rep 11, 428–435 (2022). https://doi.org/10.1007/s13730-022-00696-0

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  • DOI: https://doi.org/10.1007/s13730-022-00696-0

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