Renal-limited 'lupus-like' nephritis

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 immun...

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Published inNephrology, dialysis, transplantation Vol. 27; no. 6; pp. 2337 - 2342
Main Authors HUERTA, Ana, BOMBACK, Andrew S, LIAKOPOULOS, Vassilis, PALANISAMY, Amudha, BARRY STOKES, M, D'AGATI, Vivette D, RADHAKRISHNAN, Jai, MARKOWITZ, Glen S, APPEL, Gerald B
Format Journal Article
LanguageEnglish
Published Oxford Oxford University Press 01.06.2012
Subjects
Adult
Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy
Biological and medical sciences
Biopsy
Emergency and intensive care: renal failure. Dialysis management
Female
Humans
Intensive care medicine
Kidney - drug effects
Kidney - immunology
Kidney - pathology
Lupus Erythematosus, Systemic - classification
Lupus Erythematosus, Systemic - immunology
Lupus Erythematosus, Systemic - pathology
Lupus Nephritis - drug therapy
Lupus Nephritis - immunology
Lupus Nephritis - pathology
Medical sciences
Middle Aged
Prognosis
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Young Adult
Kidney disease
Lupus
Extrarenal dialysis
Skin disease
Urinary system disease
Hemodialysis
Renal failure
lupus nephritis
renal limited
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Summary: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. We refer to these cases as renal-limited 'lupus-like' nephritis.
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ISSN:0931-0509
1460-2385
DOI:10.1093/ndt/gfr663