Chapter

Lupus podocytopathy

Edmund J. Lewis

in Lupus Nephritis

Second edition

Published on behalf of Oxford University Press

Published in print November 2010 | ISBN: 9780199568055
Published online November 2012 | e-ISBN: 9780191753374 | DOI: http://dx.doi.org/10.1093/med/9780199568055.003.0008

Series: Oxford Clinical Nephrology Series

Lupus podocytopathy

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The term lupus podocytopathy has been formulated in order to describe the renal lesion in a group of patients with systemic lupus erythematosus (SLE) who present with a marked degree of proteinuria, often causing the characteristic clinical changes of the nephrotic syndrome with massive amounts of albuminuria, hypoalbuminemia, and edema. Unlike the classic patient with membranous glomerulonephritis or severe inflammatory lupus nephritis, who may present in this manner, these patients have biopsy findings revealing either no glomerular immune deposits or sparse deposits, which are confined to the glomerular mesangium (Figure 8.1). The characteristic pathological glomerular abnormality observed among these patients is ultrastructural and resides in the visceral glomerular epithelial cells, and is characterized as glomerular epithelial foot process fusion (Figure 8.2). The glomerular lesions are identical to those described in idiopathic minimal change glomerulopathy (MCG) (Figure 8.3), the most common cause of the nephrotic syndrome in children. In some cases there may be glomerular scars, in which case the histological diagnosis fits with the diagnosis of focal and segmental glomerulosclerosis (FSGS) (Figure 8.4). Although usually a primary lesion, MCG can also rarely occur in patients with Hodgkin’s disease, has also been associated with lithium administration, and can occur in occasional cases of patients receiving nonsteroidal anti-inflammatory agents (NSAIA). Children with MCG almost always respond promptly to the use of high-dose prednisone or prednisolone therapy. Adults with MCG also tend to respond to high-dose steroid therapy, but often do so less rapidly than children.

The pathogenesis of scars in FSGS is variable and complex. As is the case with MCG, FSGS has also only recently been associated with SLE. A diverse number of inherited and acquired abnormalities of the structural proteins associated with the glomerular epithelial cell have been associated with FSGS. There is evidence that a circulating factor is responsible for the proteinuria that occurs in idiopathic FSGS. Some of this evidence implies the possibility that the factor that may promote proteinuria could be a T cell cytokine. As indicated by the histopathological term, FSGS is characterized by glomerular scars, which affect a portion of the glomerular tuft of some glomeruli (Figure 8.4). Often, when seen in SLE, very few glomeruli may be scarred. These patients are less likely to be prednisone-responsive than those with MCG, and other immunosuppressive agents have generally been required in order to induce a partial or complete remission of the proteinuria.

Chapter.  4875 words.  Illustrated.

Subjects: Nephrology

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