Minimal-Change Nephrotic Syndrome Associated with Systemic Lupus Erythematosus

Makino, Hírofumi; Haramoto, Toshinori; Shikata, Kenichi; Ogura, Toshio; Ota, Zensuke

doi:10.1159/000168880

Cited by 23 publications

(25 citation statements)

References 11 publications

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“…Lupus podocytopathy was divided into three groups according to morphologic features: MCD (characterized by normal glomeruli or minimal MsP [4][5][6][7][8]), MsP ( defined as $3 mesangial cells per mesangial area [11][12][13][14]), or FSGS (at least one segmental solidification of the glomerular tuft with accumulation of extracellular matrix, hyalinosis and foam cells could be present). Collapsing FSGS was defined by the presence of segmental capillary tuft collapse in at least one glomerulus with overlying podocyte hyperplasia and/or hypertrophy (25).…”

Section: Renal Morphologymentioning

confidence: 99%

“…NS is particularly characteristic of proliferative LN (class III, class IV) or membranous LN (class V) (1,2). However, it was observed 20 years ago that a subset of SLE patients who presented with NS were found to have normal glomeruli or only mild glomerular mesangial proliferation (3)(4)(5)(6)(7)(8). In these patients, the most prominent electron microscopy (EM) findings were diffuse foot process effacement (FPE) without immune deposits in the wall of peripheral capillaries, suggestive of podocytopathy.…”

Section: Introductionmentioning

confidence: 99%

“…Patients with lupus podocytopathy have been presented in case reports or small series over the last 20 years (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22). The prognosis of these patients remains uncertain.…”

Section: Introductionmentioning

confidence: 99%

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Clinical–Morphological Features and Outcomes of Lupus Podocytopathy

Chen

Wang

et al. 2016

Clinical Journal of the American Society of Nephrology

111

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Background and objectives Lupus podocytopathy, which is characterized by diffuse foot process effacement without peripheral capillary wall immune deposits and glomerular proliferation, has been described in SLE patients with nephrotic syndrome in case reports and small series. This study aimed to better characterize the incidence, clinical-morphologic features, and outcomes of such patients from a large Chinese cohort.Design, setting, participants, & measurements Lupus podocytopathy was identified from 3750 biopsies of SLE patients obtained from 2000 to 2013 that showed mild glomerular histology in patients with a clinical sign of nephrotic syndrome. The biopsy results were divided into three groups: glomerular minimal change, mesangial proliferation, and FSGS.Results Fifty (1.33%) cases were identified as lupus podocytopathy and included minimal change in 13 cases, mesangial proliferation in 28 cases, and FSGS in nine cases. Extensive foot process effacement appeared in all the biopsies and mesangial electron-dense deposits were present in 47 biopsies. All patients demonstrated nephrotic syndrome, and the median proteinuria was 5.72 g/24 h (interquartile range [IQR], 3.82, 6.92). Seventeen (34%) cases presented with AKI. Forty-seven (94%) patients achieved remission after immunosuppressive therapy for a median time of 4 weeks (IQR,2,8). Compared with the patients with minimal change and mesangial proliferation, patients with FSGS showed significantly higher incidence of AKI and severe tubule-interstitial injury and a much lower complete remission rate. During follow-up of a median of 62 (IQR, 36, 84) months, renal relapses occurred in 28 (59.6%) patients. No patient died or developed ESRD. ConclusionsThe findings from this cohort study suggest that lupus podocytopathy may represent a special entity of lupus nephritis with distinct clinical-morphologic features. The differences in AKI incidence, tubular injury severity, and response to treatment between the patients with minimal change/mesangial proliferation and those with FSGS patterns indicate two different subtypes of lupus podocytopathy.

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Section: Renal Morphologymentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Clinical–Morphological Features and Outcomes of Lupus Podocytopathy

Chen

Wang

et al. 2016

Clinical Journal of the American Society of Nephrology

111

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“…Nephrotic syndrome or nephrotic range proteinuria in the context of SLE is often associated with identification of immune deposits in the glomerular capillary wall, with or without endocapillary proliferation and/or necrosis. However, there are reports of lupus patients who developed nephrotic syndrome in the absence of capillary wall immune deposits or cellular proliferation [3][4][5][6][7][8][9][10]. e lupus patient, who is described here, presented with nephrotic proteinuria with or without symptoms of nephrotic syndrome in four separate circumstances.…”

Section: Discussionmentioning

confidence: 99%

The Various Forms of Nephrotic Syndrome in a Patient with Systemic Lupus Erythematosus

Lionaki

Liapis

Vallianou

et al. 2020

Case Reports in Nephrology

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Kidney involvement is frequent in patients with systemic lupus erythematosus (SLE), although it may not be present from disease onset. Renal lupus itself is highly heterogenous with respect to the combination and/or severity of clinical and/or laboratory manifestations. This is a case of a 45-year-old Caucasian female with an established diagnosis of SLE, who presented four times with new onset of proteinuria during a follow-up time of ten years, since the diagnosis of SLE. Specifically, she experienced two episodes of lupus membranous nephropathy, and after she achieved remission, she developed twice overt nephrotic syndrome associated with new and biopsy proven lupus podocytopathy. All these episodes of nephrotic syndrome were combined with systemic symptoms, attributed to lupus itself, while serological activity of lupus was also noted. This case highlights the importance of performing a kidney biopsy in all patients with SLE who have new renal manifestations, including nephrotic proteinuria.

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“…Makino et al [4] reported a case of minimal change nephritic syndrome associated with SLE and treated with methylprednisolone pulse therapy. Dube et al [5] reported on seven patients with SLE and minimal change disease treated with prednisone and cessation of NSAIDs.…”

Section: Discussionmentioning

confidence: 99%

A Case of Minimal Change Disease Treated Successfully with Mycophenolate Mofetil in a Patient with Systemic Lupus Erythematosus

Hong

Yun

Jung

et al. 2011

Korean J Intern Med

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The World Health Organization classifies lupus nephritis as class I to V or VI. However, a few cases of minimal change glomerulopathy have been reported in association with systemic lupus erythematosus (SLE). Mycophenolate mofetil has been shown to be effective for treatment of minimal change disease and lupus nephritis. A 24-year-old woman diagnosed with SLE five years prior to presentation complained of a mild generalized edema. The urinalysis showed microscopic hematuria and proteinuria. The assessed amount of total proteinuria was 1,618 mg/24 hours. A renal biopsy demonstrated diffuse fusion of the foot processes of podocytes on electron microscopy. Mycophenolate mofetil was started in addition to the maintenance medications of prednisolone 10 mg/day and hydroxychloroquine 400 mg/day. After six months of treatment, the microscopic hematuria and proteinuria resolved, and the total urine protein decreased to 100 mg/24 hours.

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Minimal-Change Nephrotic Syndrome Associated with Systemic Lupus Erythematosus

Cited by 23 publications

References 11 publications

Clinical–Morphological Features and Outcomes of Lupus Podocytopathy

Clinical–Morphological Features and Outcomes of Lupus Podocytopathy

The Various Forms of Nephrotic Syndrome in a Patient with Systemic Lupus Erythematosus

A Case of Minimal Change Disease Treated Successfully with Mycophenolate Mofetil in a Patient with Systemic Lupus Erythematosus

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