The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
권호 표지
DOI QR코드

DOI QR Code

Polyarteritis Nodosa Confined to the Kidneys in a Patient with Proteinuria and Mild Renal Impairment

단백뇨와 경도 신기능장애가 있는 환자에서 진단된 신장에 국한된 결절성 다발성 동맥염 1예: 증례 보고

  • Young Kyeong Seo (Division of Nephrology, Department of Internal Medicine, Inje University Busan Paik Hospital) ;
  • Taehee Kim (Division of Nephrology, Department of Internal Medicine, Inje University Busan Paik Hospital) ;
  • Yeong Hoon Kim (Division of Nephrology, Department of Internal Medicine, Inje University Busan Paik Hospital) ;
  • Yunmi Kim (Division of Nephrology, Department of Internal Medicine, Inje University Busan Paik Hospital) ;
  • Hyuk Huh (Division of Nephrology, Department of Internal Medicine, Inje University Busan Paik Hospital) ;
  • Byeong Woo Kim (Division of Nephrology, Department of Internal Medicine, Inje University Busan Paik Hospital)
  • 서영경 (인제대학교 부산백병원 신장내과) ;
  • 김태희 (인제대학교 부산백병원 신장내과) ;
  • 김영훈 (인제대학교 부산백병원 신장내과) ;
  • 김윤미 (인제대학교 부산백병원 신장내과) ;
  • 허혁 (인제대학교 부산백병원 신장내과) ;
  • 김병우 (인제대학교 부산백병원 신장내과)
  • Received : 2023.10.21
  • Accepted : 2023.11.15
  • Published : 2024.04.01
Download PDF
⟨ Previous Next ⟩

Abstract

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis predominantly involving medium- or small-sized arteries, typically of the kidneys and other internal organs. Given the rarity of PAN and the variable clinical presentation, diagnosis is challenging and, to date, no definitive diagnostic marker has been identified. A patient diagnosed with immunoglobulin A nephropathy was observed to exhibit deterioration in renal function. To determine whether new structural abnormalities had developed, computed tomography scans of the kidneys, ureters, and bladder were obtained. Both kidneys exhibited multiple cortical defects, and a renal angiogram was performed to determine the cause. Angiography revealed partial obliteration of the left distal renal artery branches and multifocal extensive infarctions in both kidneys, and the patient was diagnosed with renal-limited PAN. Following steroid monotherapy, an improvement in renal function was observed. We believe that this case report may be helpful to physicians who assess and treat patients with suspected renal-limited PAN.

Keywords

Acknowledgement

The study was supported by research grants from Inje University.

References

  1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum 2013;65:1-11. https://doi.org/10.1002/art.37715
  2. Lhote F, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Clinical aspects and treatment. Rheum Dis Clin North Am 1995;21:911-947. https://doi.org/10.1016/S0889-857X(21)00479-8
  3. Lhote F, Cohen P, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis and Churg-Strauss syndrome. Lupus 1998;7:238-258. https://doi.org/10.1191/096120398678920055
  4. Pagnoux C, Mahr A, Cohen P, Guillevin L. Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis. Medicine (Baltimore) 2005;84:115-128. https://doi.org/10.1097/01.md.0000158825.87055.0b
  5. De Virgilio A, Greco A, Magliulo G, et al. Polyarteritis nodosa: a contemporary overview. Autoimmun Rev 2016;15:564-570. https://doi.org/10.1016/j.autrev.2016.02.015
  6. Pagnoux C, Seror R, Henegar C, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group database. Arthritis Rheum 2010;62:616-626. https://doi.org/10.1002/art.27240
  7. Pourafshar N, Sobel E, Segal M. A case of isolated renal involvement of polyarteritis nodosa successfully treated with steroid monotherapy. BMJ Case Rep 2016;2016:bcr2016215702.
  8. Guillevin L, Lhote F. Polyarteritis nodosa and microscopic polyangiitis. Clin Exp Immunol 1995;101 Suppl 1:22-23. https://doi.org/10.1111/j.1365-2249.1995.tb06157.x
  9. Gonzalez-Gay MA, Garcia-Porrua C, Guerrero J, Rodriguez-Ledo P, Llorca J. The epidemiology of the primary systemic vasculitides in northwest Spain: implications of the Chapel Hill Consensus Conference definitions. Arthritis Rheum 2003;49:388-393. https://doi.org/10.1002/art.11115
  10. Lie JT. Systemic and isolated vasculitis. A rational approach to classification and pathologic diagnosis. Pathol Annu 1989;24:25-114.
  11. Albert DA, Rimon D, Silverstein MD. The diagnosis of polyarteritis nodosa. I. A literature-based decision analysis approach. Arthritis Rheum 1988;31:1117-1127. https://doi.org/10.1002/art.1780310906
  12. Greco A, De Virgilio A, Rizzo MI, et al. Microscopic polyangiitis: advances in diagnostic and therapeutic approaches. Autoimmun Rev 2015;14:837-844. https://doi.org/10.1016/j.autrev.2015.05.005