A patient with Behcet's disease and IgA nephropathy in China.
Behcet’s disease
Biopsy
IgA nephropathy
Renal
Journal
BMC nephrology
ISSN: 1471-2369
Titre abrégé: BMC Nephrol
Pays: England
ID NLM: 100967793
Informations de publication
Date de publication:
18 Sep 2024
18 Sep 2024
Historique:
received:
19
04
2024
accepted:
06
09
2024
medline:
19
9
2024
pubmed:
19
9
2024
entrez:
18
9
2024
Statut:
epublish
Résumé
Behcet's disease (BD) is an inflammatory disorder of unknown cause that is characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Local vasculitis can cause damage to the visceral system, but it is rare in kidney patients, especially those with IgA nephropathy (IgAN). In China, a small number of related cases have been reported. Here we present a case of co-occurrence of BD and IgAN. An 18-year-old female who presented with a history of recurrent oral ulcers was found ten years ago. Four years later, the patient presented with reddish nodules on the skin of both lower limbs and then presented with vulvar ulcers. This patient was clinically diagnosed with Behcet's disease after left calf skin biopsy and presented severe proteinuria and hematuria during this period. IgAN was diagnosed after percutaneous renal biopsy. The patient was treated with hormonal, anti-inflammatory, immunomodulatory, kidney protective, and protein-lowering urine agents. After 3 years of follow-up, the patient reappears oral ulcers, reddish nodules on the skin of both lower limbs and renal dysfunction. BD is less common in China and is clinically prone to missed diagnosis and misdiagnosis. BD with IgAN is rarer. We should regularly pay attention to the routine urine and renal function of BD patients for early detection and treatment and to prevent further progression of the disease.
Sections du résumé
BACKGROUND
BACKGROUND
Behcet's disease (BD) is an inflammatory disorder of unknown cause that is characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Local vasculitis can cause damage to the visceral system, but it is rare in kidney patients, especially those with IgA nephropathy (IgAN). In China, a small number of related cases have been reported. Here we present a case of co-occurrence of BD and IgAN.
CASE PRESENTATION
METHODS
An 18-year-old female who presented with a history of recurrent oral ulcers was found ten years ago. Four years later, the patient presented with reddish nodules on the skin of both lower limbs and then presented with vulvar ulcers. This patient was clinically diagnosed with Behcet's disease after left calf skin biopsy and presented severe proteinuria and hematuria during this period. IgAN was diagnosed after percutaneous renal biopsy. The patient was treated with hormonal, anti-inflammatory, immunomodulatory, kidney protective, and protein-lowering urine agents. After 3 years of follow-up, the patient reappears oral ulcers, reddish nodules on the skin of both lower limbs and renal dysfunction.
CONCLUSIONS
CONCLUSIONS
BD is less common in China and is clinically prone to missed diagnosis and misdiagnosis. BD with IgAN is rarer. We should regularly pay attention to the routine urine and renal function of BD patients for early detection and treatment and to prevent further progression of the disease.
Identifiants
pubmed: 39294591
doi: 10.1186/s12882-024-03748-y
pii: 10.1186/s12882-024-03748-y
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
310Subventions
Organisme : Guizhou Province Science & Technology Project of Guizhou Province Science and Technology Foundation
ID : ZK[2021] General 385
Organisme : Guizhou Provincial People's Hospital Youth Fund
ID : GZSYQN[2022]15
Informations de copyright
© 2024. The Author(s).
Références
Hatemi G, Christensen R, Bang D, Bodaghi B, Celik AF, Fortune F, et al. 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis. 2018;77(6):808–18. https://doi.org/10.1136/annrheumdis-2018-213225 .
doi: 10.1136/annrheumdis-2018-213225
pubmed: 29625968
Akpolat T, Diri B, Oguz Y, Yilmaz E, Yavuz M, Dilek M. Behcet’s disease and renal failure. Nephrol Dial Transpl. 2003;18(5):888–91. https://doi.org/10.1093/ndt/gfg084 .
doi: 10.1093/ndt/gfg084
Guan J. New concept of Behest’s disease. Shanghai: Fudan University; 2021.
Zheng W, Zhang N, Zhu X, Chi S, Zhang W, Wei W, et al. Norms for the diagnosis and treatment of Behçet’s syndrome. Chin J Intern Med. 2021;60(10):860–7. https://doi.org/10.3760/cma.j.cn112138-20210604-00398 .
doi: 10.3760/cma.j.cn112138-20210604-00398
Altay M, Secilmis S, Unverdi S, Ceri M, Duranay M. Behcet’s disease and IgA nephropathy. Rheumatol Int. 2012; 32(7): 2227-9. https://doi:.10.1007/s00296-011-2051-3.
Zheng W, Li G, Zhou M, Chen L, Tian X, Zhang F. Renal involvement in Chinese patients with Behcet’s disease: a report of 16 cases. Int J Rheum Dis. 2015;18(8):892–7. https://doi.org/10.1111/1756-185X.12529 .
doi: 10.1111/1756-185X.12529
pubmed: 25557053
Tong B, Liu X, Xiao J, Su G. Immunopathogenesis of Behcet’s disease. Front Immunol. 2019;10:665. https://doi.org/10.3389/fimmu.2019.00665 .
doi: 10.3389/fimmu.2019.00665
pubmed: 30984205
pmcid: 6449449
Geri G, Terrier B, Rosenzwajg M, Wechsler B, Touzot M, Seilhean D, et al. Critical role of IL-21 in modulating TH17 and regulatory T cells in Behcet disease. J Allergy Clin Immunol. 2011;128(3):655–64. https://doi.org/10.1016/j.jaci.2011.05.029 .
doi: 10.1016/j.jaci.2011.05.029
pubmed: 21724243
Watanabe-Kusunoki K, Kato M, Oki Y, Shimizu T, Kusunoki Y, Furukawa S. Parallel disease activity of Behçet’s disease with renal and entero involvements: a case report. BMC Nephrol. 2021;22(1):1–6. https://doi.org/10.1186/s12882-021-02327-9 .
doi: 10.1186/s12882-021-02327-9
Trifari S, Kaplan CD, Tran EH, Crellin NK, Spits H. Identification of a human helper T-cell population that has abundant production of interleukin 22 and is distinct from T(H)-17, T(H)1 and T(H)2 cells. Nat Immunol. 2009;10(8):864–71. https://doi.org/10.1038/ni.1770 .
doi: 10.1038/ni.1770
pubmed: 19578368
Lai KW, Wei CL, Tan LK, Tan PH, Chiang GS, Lee CG, et al. Overexpression of interleukin-13 induces minimal-change-like nephropathy in rats. J Am Soc Nephrol. 2007;18(5):1476–85. https://doi.org/10.1681/ASN.2006070710 .
doi: 10.1681/ASN.2006070710
pubmed: 17429054
Hashimoto T, Toya Y, Kihara M, Yabana M, Inayama Y, Tanaka K, et al. Behcet’s disease complicated by IgA nephropathy with nephrotic syndrome. Clin Exp Nephrol. 2008;12(3):224–7. https://doi.org/10.1007/s10157-008-0029-6 .
doi: 10.1007/s10157-008-0029-6
pubmed: 18224274