Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by development of autoantibodies to nuclear and cytoplasmic antigens. A small subset of SLE patients who had the typical clinical features of SLE was reported to show persistently negative antinuclear antibody tests. Our report describes a 5-year-old male who presented with histopathological findings suggestive of lupus nephritis with no clinical signs or symptoms of SLE and negative autoantibodies. He was treated with corticosteroids, mycophenolate mofetil, and monthly intravenous cyclophosphamide. During the 2-year follow-up period, the proteinuria resolved and kidney function improved with continued negative autoantibody workup. This case presents a category of renal-limited "lupus-like" glomerulonephritis which can be challenging to treat and carries a poor prognosis.
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http://dx.doi.org/10.1159/000475493 | DOI Listing |
Lupus
July 2021
Hospital Central "Dr. Ignacio Morones Prieto" and Faculty of Medicine, Universidad Autónoma de San Luis Potosí, S.L.P., México.
Introduction: Lupus nephritis requires antinuclear antibodies as classification criteria. There is a group of patients with nephrotic syndrome and conclusive histopathological findings for lupus nephritis, without classification criteria for systemic lupus erythematosus (SLE) or extrarenal manifestations. These groups of patients have been described as "lupus-like" nephritis or "renal-limited lupus nephritis".
View Article and Find Full Text PDFCase Rep Nephrol Dial
May 2017
cDivision of Renal Pathology, Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by development of autoantibodies to nuclear and cytoplasmic antigens. A small subset of SLE patients who had the typical clinical features of SLE was reported to show persistently negative antinuclear antibody tests. Our report describes a 5-year-old male who presented with histopathological findings suggestive of lupus nephritis with no clinical signs or symptoms of SLE and negative autoantibodies.
View Article and Find Full Text PDFNephrol Dial Transplant
June 2012
Department of Nephrology, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Madrid, Spain.
In the setting of an IgG-dominant immune complex-mediated glomerulonephritis, there are multiple pathological findings that strongly suggest the diagnosis of lupus nephritis (LN) including (i) 'full-house' immunofluorescence staining for IgG, IgM, IgA, C3 and C1; (ii) extraglomerular immune deposits; (iii) combined mesangial, subendothelial and subepithelial immune deposits and (iv) the presence of endothelial tubuloreticular inclusions. We report four female adult patients with renal biopsy findings which are highly suggestive of LN but without extrarenal signs, symptoms or serologies of systemic lupus erythematosus at the time of biopsy or over a mean follow-up period of 3 years. Despite aggressive therapy, outcomes were poor in this small cohort.
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