Advertisement

Lupus Podocytopathy: An Overview

      In systemic lupus erythematosus, nephrotic-range proteinuria typically signals the presence of a proliferative lupus nephritis (class III/IV) and/or membranous lupus nephritis (class V, with or without concomitant class III or IV lesions). However, in rare instances, systemic lupus erythematosus patients with nephrotic syndrome have kidney biopsy findings of normal glomeruli or focal segmental glomerulosclerosis lesions, with or without mesangial proliferation, on light microscopy; the absence of subepithelial or subendothelial deposits on immunofluorescence and electron microscopy; and diffuse foot process effacement on electron microscopy. This pattern, termed lupus podocytopathy, is a unique form of lupus nephritis that mimics minimal change disease or primary focal segmental glomerulosclerosis and represents approximately 1% of lupus nephritis biopsies. Here we review the clinical features, histological manifestations, diagnostic criteria and classification, pathogenesis, treatment, and prognosis of lupus podocytopathy.

      Key Words

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic and Personal

      Subscribe:

      Subscribe to Advances in Chronic Kidney Disease
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Kraft S.W.
        • Schwartz M.M.
        • Korbet S.M.
        • Lewis E.J.
        Glomerular podocytopathy in patients with systemic lupus erythematosus.
        J Am Soc Nephrol. 2005; 16: 175-179
        • Hu W.
        • Chen Y.
        • Wang S.
        • et al.
        Clinical-morphological features and outcomes of lupus podocytopathy.
        Clin J Am Soc Nephrol. 2016; 11: 585-592
        • Dube G.K.
        • Markowitz G.S.
        • Radhakrishnan J.
        • Appel G.B.
        • D'agati V.D.
        Minimal change disease in systemic lupus erythematosus.
        Clin Nephrol. 2002; 57: 120-126
        • Weening J.J.
        • D'agati V.D.
        • Schwartz M.M.
        • et al.
        The classification of glomerulonephritis in systemic lupus erythematosus revisited.
        Kidney Int. 2004; 65: 521-530
        • Abuelo J.G.
        • Esparza A.R.
        • Garella S.
        Steroid-dependent nephrotic syndrome in lupus nephritis. Response to chlorambucil.
        Arch Intern Med. 1984; 144: 2411-2412
        • Matsumura N.
        • Dohi K.
        • Shiki H.
        • et al.
        Three cases presenting with systemic lupus erythematosus and minimal change nephrotic syndrome (in Japanese).
        Jpn J Nephrol. 1989; 31: 991-999
        • Okai T.
        • Soejima A.
        • Suzuki M.
        • et al.
        A case report of lupus nephritis associated with minimal change nephrotic syndrome: comparison of various histological types of 67 cases with lupus nephritis (in Japanese).
        Jpn J Nephrol. 1992; 34: 835-840
        • Makino H.
        • Haramoto T.
        • Shikata K.
        • Ogura T.
        • Ota Z.
        Minimal-change nephrotic syndrome associated with systemic lupus erythematosus.
        Am J Nephrol. 1995; 15: 439-441
        • Hickman P.L.
        • Nolph K.D.
        • Jacobs R.
        • Luger A.M.
        • Walker S.E.
        Idiopathic focal segmental glomerulosclerosis in a patient with systemic lupus erythematosus: an unusual combination.
        Am J Kidney Dis. 1994; 23: 582-586
        • Hertig A.
        • Droz D.
        • Lesavre P.
        • Grünfeld J.P.
        • Rieu P.
        SLE and idiopathic nephrotic syndrome: coincidence or not?.
        Am J Kidney Dis. 2002; 40: 1179-1184
        • Bomback A.S.
        • Markowitz G.S.
        Lupus podocytopathy: a distinct entity.
        Clin J Am Soc Nephrol. 2016; 11: 547-548
        • Chen D.
        • Hu W.
        Lupus podocytopathy: a distinct entity of lupus nephritis.
        J Nephrol. 2018; 31: 629-634
        • Wang Y.T.
        • Chou H.H.
        • Chen F.F.
        • Chen M.J.
        • Chiou Y.Y.
        A case of minimal-change nephrotic syndrome in pediatric lupus erythematosus: just a coincidence?.
        Lupus. 2006; 15: 244-247
        • Bomback A.S.
        Nonproliferative forms of lupus nephritis: an overview.
        Rheum Dis Clin North Am. 2018; 44: 561-569
        • Bomback A.S.
        A pregnant woman with lupus and nephrotic-range proteinuria.
        Clin J Am Soc Nephrol. 2017; 12: 1534-1537
        • Salvatore S.P.
        • Barisoni L.M.
        • Herzenberg A.M.
        • Chander P.N.
        • Nickeleit V.
        • Seshan S.V.
        Collapsing glomerulopathy in 19 patients with systemic lupus erythematosus or lupus-like disease.
        Clin J Am Soc Nephrol. 2012; 7: 914-925
        • Haas M.
        Collapsing glomerulopathy in systemic lupus erythematosus: an extreme form of lupus podocytopathy?.
        Clin J Am Soc Nephrol. 2012; 7: 878-880
        • Wang S.F.
        • Chen Y.H.
        • Chen D.Q.
        • et al.
        Mesangial proliferative lupus nephritis with podocytopathy: a special entity of lupus nephritis.
        Lupus. 2018; 27: 303-311
        • Han T.S.
        • Schwartz M.M.
        • Lewis E.J.
        Association of glomerular podocytopathy and nephrotic proteinuria in mesangial lupus nephritis.
        Lupus. 2006; 15: 71-75
        • D'agati V.D.
        • Kaskel F.J.
        • Falk R.J.
        Focal segmental glomerulosclerosis.
        N Engl J Med. 2011; 365: 2398-2411
        • Almaani S.
        • Meara A.
        • Rovin B.H.
        Update on lupus nephritis.
        Clin J Am Soc Nephrol. 2017; 12: 825-835
        • Yu F.
        • Haas M.
        • Glassock R.
        • Zhao M.H.
        Redefining lupus nephritis: clinical implications of pathophysiologic subtypes.
        Nat Rev Nephrol. 2017; 13: 483-495
        • Trivedi S.
        • Zeier M.
        • Reiser J.
        Role of podocytes in lupus nephritis.
        Nephrol Dial Transplant. 2009; 24: 3607-3612
        • Cunard R.
        • Kelly C.J.
        T cells and minimal change disease.
        J Am Soc Nephrol. 2002; 13: 1409-1411
        • Wei C.
        • El hindi S.
        • Li J.
        • et al.
        Circulating urokinase receptor as a cause of focal segmental glomerulosclerosis.
        Nat Med. 2011; 17: 952-960
        • Reiser J.
        • Nast C.C.
        • Alachkar N.
        Permeability factors in focal and segmental glomerulosclerosis.
        Adv Chronic Kidney Dis. 2014; 21: 417-421
        • Artero M.L.
        • Sharma R.
        • Savin V.J.
        • Vincenti F.
        Plasmapheresis reduces proteinuria and serum capacity to injure glomeruli in patients with recurrent focal glomerulosclerosis.
        Am J Kidney Dis. 1994; 23: 574-581
        • Gohh R.Y.
        • Yango A.F.
        • Morrissey P.E.
        • et al.
        Preemptive plasmapheresis and recurrence of FSGS in high-risk renal transplant recipients.
        Am J Transplant. 2005; 5: 2907-2912
        • Larsen C.P.
        • Beggs M.L.
        • Saeed M.
        • Walker P.D.
        Apolipoprotein L1 risk variants associate with systemic lupus erythematosus-associated collapsing glomerulopathy.
        J Am Soc Nephrol. 2013; 24: 722-725
        • Markowitz G.S.
        • Nasr S.H.
        • Stokes M.B.
        • D'Agati V.D.
        Treatment with IFN is associated with collapsing focal segmental glomerulosclerosis.
        Clin J Am Soc Nephrol. 2010; 5: 607-615
        • Rönnblom L.
        • Alm G.V.
        • Eloranta M.L.
        The type I interferon system in the development of lupus.
        Semin Immunol. 2011; 23: 113-121
        • Bomback A.S.
        • Appel G.B.
        Updates on the treatment of lupus nephritis.
        J Am Soc Nephrol. 2010; 21: 2028-2035
        • Hu W.X.
        • Chen Y.H.
        • Bao H.
        • et al.
        Glucocorticoid with or without additional immunosuppressant therapy for patients with lupus podocytopathy: a retrospective single-center study.
        Lupus. 2015; 24: 1067-1075
        • Kidney Disease: Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group
        KDIGO clinical practice guideline for glomerulonephritis.
        Kidney Int Suppl. 2012; 2: 139-274
        • Wang Y.
        • Yu F.
        • Song D.
        • Wang S.X.
        • Zhao M.H.
        Podocyte involvement in lupus nephritis based on the 2003 ISN/RPS system: a large cohort study from a single centre.
        Rheumatology (Oxford). 2014; 53: 1235-1244
        • Rovin B.H.
        • Caster D.J.
        • Cattran D.C.
        • et al.
        Management and treatment of glomerular diseases (part 2): conclusions from a Kidney Disease: improving Global Outcomes (KDIGO) controversies conference.
        Kidney Int. 2019; 95: 281-295
        • Shea-simonds P.
        • Cairns T.D.
        • Roufosse C.
        • Cook T.
        • Vyse T.J.
        Lupus podocytopathy.
        Rheumatology (Oxford). 2009; 48: 1616-1618