Lupus Podocytopathy

Bomback, Andrew S.; Markowitz, Glen S.

doi:10.2215/cjn.01880216

Cited by 52 publications

(36 citation statements)

References 8 publications

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“…treat / don't treat) to the current classification and hampers the introduction of diagnostic subtleties such as lupus podocytopathy. 15 Another issue is that while nephrologists are sensible to findings such as tubular atrophy and interstitial fibrosis, until the last update, ISN/RPS failed to account for tubulointerstitial lesions adequately. Injury to the tubulointerstitial compartment appears to poorly correlate with glomerular lesions, 16,17 suggesting independent immunological pathways, which were oversimplified by the ISN/RPS classification -this raises the question if the tubulointerstitial activity should itself be an indication for immunosuppressive therapy.…”

Section: Discussionmentioning

confidence: 99%

Morphological Indexes: Can They Predict Lupus Nephritis Outcomes? A Retrospective Study

et al. 2019

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Introduction: Lupus nephritis is a serious complication of systemic lupus erythematosus. Currently, therapy is guided by findings in the renal biopsy, following the International Society of Nephrology / Renal Pathology Society classification. Austin and Hill’s histomorphological indexes are not routinely obtained. In this retrospective single-centre study, we aimed to analyze the importance and applicability of the different morphological indexes in predicting response to treatment and prognosis.Material and Methods: Patients with kidney biopsy demonstrating lupus nephritis from the 2010 – 2016 period were included. We analyzed their demographic data, comorbidities, clinical presentation and laboratorial evaluation at the time of renal biopsy. We evaluated the following outcomes: clinical remission, renal function and proteinuria at end of follow-up. Histologic analysis was performed using the International Society of Nephrology / Renal Pathology Society classification and the morphological indexes described by Austin (Activity and Chronicity) and Hill. Univariate and multivariate statistical analysis was performed using STATA software.Results: We analyzed 46 biopsy-proven lupus nephritis cases, with a median follow-up of 31.9 (13.2 – 45.6) months. Based on biopsy findings, 35 patients were started on immunosuppressive therapy. We observed that Class IV patients had, at presentation, lower estimated glomerular filtration rate (67.3 vs 94.6 mL/min; p = 0.02), higher proteinuria (4.26 vs 2.37 g/24 hours; p = 0.02) and a non-significantly higher C3 consumption (58.9 vs 77.4 mg/dL; p = 0.06). We did not observe correlations between International Society of Nephrology / Renal Pathology Society classification and the outcomes at the end of follow-up. In contrast, both the Hill biopsy index and Austin’s Chronicity index were correlated with renal function and proteinuria at the end of follow-up. Austin’s Activity index correlated with the immunological findings (C3, C4 and anti-dsDNA) at presentation.Discussion: Because clinical activity poorly correlates with histologic activity, histological findings are fundamental when assessing patients with suspected lupus nephritis. The most recent International Society of Nephrology / Renal Pathology Society report supports the European League Against Rheumatism guidelines, encouraging the adoption of histomorphological indexes when evaluating lupus nephritis. Our data, showing a correlation between the renal outcomes and the indexes described by Austin and Hill, supports this view.Conclusion: The histomorphological indexes in lupus nephritis are easily obtainable, can predict renal outcomes and may help in the management of such patients.

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Section: Discussionmentioning

confidence: 99%

Morphological Indexes: Can They Predict Lupus Nephritis Outcomes? A Retrospective Study

et al. 2019

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“…(57) Podocyte injuries are common and stem from the loss of expression of the proteins present in the slit diaphragm (nephrin and podocin) and the disorganization of the podocyte cytoskeleton, culminating with the flattening, effacement, and microvillus transformation of the foot processes. (58) These changes can be viewed only through an electron microscope. (58) Affected patients develop marked proteinuria.…”

Section: Pathologymentioning

confidence: 99%

“…(58) These changes can be viewed only through an electron microscope. (58) Affected patients develop marked proteinuria. Podocyte injuries may be used to identify patients potentially responsive to calcineurin inhibitors. Crescentic injuries arise from immune deposits or direct attack by inflammatory cells.…”

Section: Pathologymentioning

confidence: 99%

Pediatric lupus nephritis

et al. 2019

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Involvement of the kidneys by lupus nephritis (LN) is one of the most severe clinical manifestations seen in individuals with systemic lupus erythematosus (SLE). LN is more frequent and severe in pediatric patients and has been associated with higher morbidity and mortality rates. This narrative review aimed to describe the general aspects of LN and its particularities when affecting children and adolescents, while focusing on the disease's etiopathogenesis, clinical manifestations, renal tissue alterations, and treatment options.

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“…(1) clinical presentation of nephrotic syndrome in a patient with lupus, (2) diffuse and severe foot process effacement on electron microscopy, and (3) the absence of subendothelial or subepithelial immune deposits on light, immunofluorescence, and electron microscopy (11). An altered systemic cytokine milieu rather than immune complex deposition is thought to mediate direct podocyte injury in these patients.…”

Section: Questionmentioning

confidence: 99%