A case of minimal-change nephrotic syndrome in pediatric lupus erythematosus:                 just a coincidence?

Wang, Y. T.; Chou, H. H.; Chen, F. F.; Chen, M. J.; Chiou, Yuan Yow

doi:10.1191/0961203306lu2285cr

Cited by 17 publications

(18 citation statements)

References 10 publications

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“…Accumulated evidence from clinical and epidemiologic studies suggested that MCD in these patients was indeed related to SLE (9,10), rather than concomitant with SLE. According to subsequent reports, SLE patients who presented with NS also showed mesangial proliferation (MsP) (11)(12)(13)(14), FSGS, or even collapsing FSGS (15,16).…”

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

103

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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: 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%

Clinical–Morphological Features and Outcomes of Lupus Podocytopathy

Chen

Wang

et al. 2016

Clinical Journal of the American Society of Nephrology

103

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“…9,10 In review of reported literature, the youngest patient is a 14-year-old girl. 12 To the best of our knowledge, no case of podocytopathy in SLE has ever been reported in children under 12 years of age. Podocytopathy in SLE was initially considered to be a chance association; however, more recent literature suggests that minimal change disease is not only a chance association, it is a part of a spectrum of lupus nephritis.…”

Section: Discussionmentioning

confidence: 94%

Chylous ascites and podocytopathy as the presentation of childhood lupus—an unusual occurrence

Pilania

Jindal

Sandesh

et al. 2018

Lupus

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Systemic lupus erythematosus (SLE) is an autoimmune disorder that can affect virtually any organ. Chylous ascites as a presenting manifestation of SLE has been described in a handful of cases in adults. However, to the best of our knowledge this presentation has never been reported in the pediatric age group. Podocytopathy in SLE was initially considered to be a chance association. However, more recently it has been suggested that minimal change disease is not only a chance association; it is part of the lupus nephritis spectrum.

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“…Recently, Wang et al reviewed 19 histories of patients with SLE and minimal-change disease [7], none of them having proliferative LN before. To our knowledge, only one other case of minimal-change disease occurring after a biopsy proven proliferative form of LN has been reported [8].…”

Section: Discussionmentioning

confidence: 99%

Re-Occurring Proteinuria in a SLE Patient: Always Lupus Nephritis?

Martens¹,

Bijl²,

Van³

et al. 2017

Lupus Open Access

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Introduction:Renal disease, designated as lupus nephritis (LN), is one of the severe manifestations of Systemic Lupus Erythematosus (SLE). The proliferative forms require aggressive treatment with corticosteroids and cytotoxic drugs. In a substantial number of patients, renal disease relapses after treatment. Case report:In this case report, we present a 27-year old woman, who had been treated for a membranoproliferative LN in the past. Seventeen years later she presented with chest pain and nephrotic-range proteinuria and severe hypoalbuminemia. The diagnosis of pulmonary embolism was made. Renal biopsy revealed only minimal abnormalities, confined to the mesangium. There was no glomerulonephritis. Based on this finding and the nephrotic-range proteinuria, the diagnosis of minimal-change disease was made. The patient was treated with prednisolone, after which the proteinuria diminished, but did not disappear. After the addition of azathioprine, the proteinuria resolved completely. Conclusion:This case illustrates that, although the diagnosis of LN has been made in the past, this does not exclude the occurrence of renal pathology caused by other diseases than SLE. Therefore, a renal biopsy is mandatory in every SLE patient with occurrence of renal abnormality es, even when a history of LN is present.

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A case of minimal-change nephrotic syndrome in pediatric lupus erythematosus: just a coincidence?

Cited by 17 publications

References 10 publications

Clinical–Morphological Features and Outcomes of Lupus Podocytopathy

Clinical–Morphological Features and Outcomes of Lupus Podocytopathy

Chylous ascites and podocytopathy as the presentation of childhood lupus—an unusual occurrence

Re-Occurring Proteinuria in a SLE Patient: Always Lupus Nephritis?

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