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Case 155
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Diagnosis: Lupus Podocytopathy

In 2002, Dube et al. (Dube GK, et al. Minimal change disease in systemic lupus erythematosus. Clin Nephrol. 2002;57(2):120-6. [Pubmed link]) and Hertig et al. (Hertig A, et al.SLE and idiopathic nephrotic syndrome: coincidence or not? Am J Kidney Dis. 2002;40(6):1179-84. [PubMed link]) described small series of patients with SLE, nephrotic syndrome, and biopsy findings of minimal change disease (MCD) or FSGS. Eight of 18 patients in these reports had mesangial deposits, including seven of 11 with MCD and one of seven with FSGS, consistent with concurrent mesangial LN (class I or II). The patients with MCD universally showed rapid remission of nephrotic syndrome with steroid therapy; the response to steroids was inconsistent in patients with FSGS lesions. In 2005, Kraft et al. (Kraft SW, et al. Glomerular podocytopathy in patients with systemic lupus erythematosus. J Am Soc Nephrol. 2005;16(1):175-9. [PubMed link]) reported eight additional patients with SLE, nephrotic syndrome, and light microscopic findings of MCD (i.e., normal appearing), FSGS, or mesangial proliferative GN. Kraft et al. argued that the “development of nephrotic-range proteinuria in patients with SLE without peripheral immune aggregate deposition or endocapillary proliferation on renal biopsy is more likely a manifestation of SLE than the coexistence of idiopathic minimal-change glomerulopathy and SLE”. The term lupus podocytopathy thus arose to describe these lesions as part of the renal involvemement spectrum in SLE. (The previous paragraph taken from: Bomback AS, Markowitz GS. Lupus Podocytopathy: A Distinct Entity. Clin J Am Soc Nephrol. 2016;11(4):547-8. [PubMed link]).

On electronic microscopy, diffuse podocyte foot process effacement in absence of sub-epithelial or sub-endothelial deposition is the morphological feature of lupus podocytopathy. Lupus podocytopathy with glomerular morphology of MCD or mesangial proliferation usually presents with typical nephrotic syndrome and sensitive to glucocorticoid treatment, but the relapse rate could reach up to 90% on maintenance treatment with glucocorticoid alone. Glucocorticoid plus other immunosuppressive agents could significantly decrease the relapse rate. Lupus podocytopathy with FSGS presents with a higher rate of acute kidney injury and less sensitivity to glucocorticoid treatment. The long-term outcomes of lupus podocytopathy are optimistic, but pathological transition could occur after renal relapses (Chen D, Hu W. Lupus podocytopathy: a distinct entity of lupus nephritis J Nephrol. 2018;31(5):629-634. [PubMed link]).

See the chapter: Lupus Nephritis of our Tutorial.

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References

  • Chen D, Hu W. Lupus podocytopathy: a distinct entity of lupus nephritis J Nephrol. 2018;31(5):629-634. [PubMed link]
  • Wang SF, Chen YH, Chen DQ, Liu ZZ, Xu F, Zeng CH, Hu WX. Mesangial proliferative lupus nephritis with podocytopathy: a special entity of lupus nephritis. Lupus. 2018;27(2):303-311. [PubMed link]
  • Hu W, Chen Y, Wang S, Chen H, Liu Z, Zeng C, Zhang H, Liu Z. Clinical-Morphological Features and Outcomes of Lupus Podocytopathy. Clin J Am Soc Nephrol. 2016;11(4):585-92. [PubMed link]
  • Bomback AS, Markowitz GS. Lupus Podocytopathy: A Distinct Entity. Clin J Am Soc Nephrol. 2016;11(4):547-8. [PubMed link]
  • Hanaoka H, Hashiguchi A, Konishi K, Kuwana M, Takeuchi T. An unusual association between focal segmental sclerosis and lupus nephritis: a distinct concept from lupus podocytopathy? CEN Case Rep. 2015;4(1):70-75. [PubMed link]
  • Hu WX, Chen YH, Bao H, Liu ZZ, Wang SF, Zhang HT, Liu ZH. Glucocorticoid with or without additional immunosuppressant therapy for patients with lupus podocytopathy: a retrospective single-center study. Lupus. 2015;24(10):1067-75. [PubMed link]

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