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Proliferative lupus nephritis in the absence of overt systemic lupus erythematosus: A historical study of 12 adult patients

Severe lupus nephritis in the absence of systemic lupus erythematosus (SLE) is a rare condition with an unclear clinical presentation and outcome. We conducted a historical observational study of 12 adult (age >18 years) patients with biopsy-proven severe lupus nephritis or lupus-like nephritis w...

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Autores principales: Touzot, Maxime, Terrier, Cécile Saint-Pastou, Faguer, Stanislas, Masson, Ingrid, François, Hélène, Couzi, Lionel, Hummel, Aurélie, Quellard, Nathalie, Touchard, Guy, Jourde-Chiche, Noémie, Goujon, Jean-Michel, Daugas, Eric
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5728820/
https://www.ncbi.nlm.nih.gov/pubmed/29310419
http://dx.doi.org/10.1097/MD.0000000000009017
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author Touzot, Maxime
Terrier, Cécile Saint-Pastou
Faguer, Stanislas
Masson, Ingrid
François, Hélène
Couzi, Lionel
Hummel, Aurélie
Quellard, Nathalie
Touchard, Guy
Jourde-Chiche, Noémie
Goujon, Jean-Michel
Daugas, Eric
author_facet Touzot, Maxime
Terrier, Cécile Saint-Pastou
Faguer, Stanislas
Masson, Ingrid
François, Hélène
Couzi, Lionel
Hummel, Aurélie
Quellard, Nathalie
Touchard, Guy
Jourde-Chiche, Noémie
Goujon, Jean-Michel
Daugas, Eric
author_sort Touzot, Maxime
collection PubMed
description Severe lupus nephritis in the absence of systemic lupus erythematosus (SLE) is a rare condition with an unclear clinical presentation and outcome. We conducted a historical observational study of 12 adult (age >18 years) patients with biopsy-proven severe lupus nephritis or lupus-like nephritis without SLE immunological markers at diagnosis or during follow-up. Excluded were patients with chronic infections with HIV or hepatitis B or C; patients with a bacterial infectious disease; and patients with pure membranous nephropathy. Electron microscopy was retrospectively performed when the material was available. End points were the proportion of patients with a complete response (urine protein to creatinine ratio <0.5 g/day and a normal or near-normal eGFR), partial response (≥50% reduction in proteinuria to subnephrotic levels and a normal or near-normal eGFR), or nonresponse at 12 months or later after the initiation of the treatment. The study included 12 patients (66% female) with a median age of 36.5 years. At diagnosis, median creatinine and proteinuria levels were 1.21 mg/dL (range 0.5–11.6) and 7.5 g/day (1.4–26.7), respectively. Six patients had nephrotic syndrome and acute kidney injury. Renal biopsy examinations revealed class III or class IV A/C lupus nephritis in all cases. Electron microscopy was performed on samples from 5 patients. The results showed mesangial and subendothelial dense deposits consistent with LN in 4 cases, and a retrospective diagnosis of pseudo-amyloid fibrillary glomerulonephritis was made in 1 patient. Patients received immunosuppressive therapy consisting of induction therapy followed by maintenance therapy, similar to treatment for severe lupus nephritis. Remission was recorded in 10 patients at 12 months after the initiation of treatment. One patient reached end-stage renal disease. After a median follow-up of 24 months, 2 patients relapsed. Lupus nephritis in the absence of overt SLE is a nosological entity requiring careful etiological investigation, including systematic electron microscopy examination of renal biopsies to rule out fibrillary glomerulonephritis. In this series, most patients presented with severe glomerulonephritis, which was highly similar to lupus nephritis at presentation and in terms of response to immunosuppressive therapy.
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spelling pubmed-57288202017-12-20 Proliferative lupus nephritis in the absence of overt systemic lupus erythematosus: A historical study of 12 adult patients Touzot, Maxime Terrier, Cécile Saint-Pastou Faguer, Stanislas Masson, Ingrid François, Hélène Couzi, Lionel Hummel, Aurélie Quellard, Nathalie Touchard, Guy Jourde-Chiche, Noémie Goujon, Jean-Michel Daugas, Eric Medicine (Baltimore) 3600 Severe lupus nephritis in the absence of systemic lupus erythematosus (SLE) is a rare condition with an unclear clinical presentation and outcome. We conducted a historical observational study of 12 adult (age >18 years) patients with biopsy-proven severe lupus nephritis or lupus-like nephritis without SLE immunological markers at diagnosis or during follow-up. Excluded were patients with chronic infections with HIV or hepatitis B or C; patients with a bacterial infectious disease; and patients with pure membranous nephropathy. Electron microscopy was retrospectively performed when the material was available. End points were the proportion of patients with a complete response (urine protein to creatinine ratio <0.5 g/day and a normal or near-normal eGFR), partial response (≥50% reduction in proteinuria to subnephrotic levels and a normal or near-normal eGFR), or nonresponse at 12 months or later after the initiation of the treatment. The study included 12 patients (66% female) with a median age of 36.5 years. At diagnosis, median creatinine and proteinuria levels were 1.21 mg/dL (range 0.5–11.6) and 7.5 g/day (1.4–26.7), respectively. Six patients had nephrotic syndrome and acute kidney injury. Renal biopsy examinations revealed class III or class IV A/C lupus nephritis in all cases. Electron microscopy was performed on samples from 5 patients. The results showed mesangial and subendothelial dense deposits consistent with LN in 4 cases, and a retrospective diagnosis of pseudo-amyloid fibrillary glomerulonephritis was made in 1 patient. Patients received immunosuppressive therapy consisting of induction therapy followed by maintenance therapy, similar to treatment for severe lupus nephritis. Remission was recorded in 10 patients at 12 months after the initiation of treatment. One patient reached end-stage renal disease. After a median follow-up of 24 months, 2 patients relapsed. Lupus nephritis in the absence of overt SLE is a nosological entity requiring careful etiological investigation, including systematic electron microscopy examination of renal biopsies to rule out fibrillary glomerulonephritis. In this series, most patients presented with severe glomerulonephritis, which was highly similar to lupus nephritis at presentation and in terms of response to immunosuppressive therapy. Wolters Kluwer Health 2017-12-01 /pmc/articles/PMC5728820/ /pubmed/29310419 http://dx.doi.org/10.1097/MD.0000000000009017 Text en Copyright © 2017 the Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by-nc/4.0 This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (CCBY-NC), where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc/4.0
spellingShingle 3600
Touzot, Maxime
Terrier, Cécile Saint-Pastou
Faguer, Stanislas
Masson, Ingrid
François, Hélène
Couzi, Lionel
Hummel, Aurélie
Quellard, Nathalie
Touchard, Guy
Jourde-Chiche, Noémie
Goujon, Jean-Michel
Daugas, Eric
Proliferative lupus nephritis in the absence of overt systemic lupus erythematosus: A historical study of 12 adult patients
title Proliferative lupus nephritis in the absence of overt systemic lupus erythematosus: A historical study of 12 adult patients
title_full Proliferative lupus nephritis in the absence of overt systemic lupus erythematosus: A historical study of 12 adult patients
title_fullStr Proliferative lupus nephritis in the absence of overt systemic lupus erythematosus: A historical study of 12 adult patients
title_full_unstemmed Proliferative lupus nephritis in the absence of overt systemic lupus erythematosus: A historical study of 12 adult patients
title_short Proliferative lupus nephritis in the absence of overt systemic lupus erythematosus: A historical study of 12 adult patients
title_sort proliferative lupus nephritis in the absence of overt systemic lupus erythematosus: a historical study of 12 adult patients
topic 3600
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5728820/
https://www.ncbi.nlm.nih.gov/pubmed/29310419
http://dx.doi.org/10.1097/MD.0000000000009017
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