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T Cells and Minimal Change Disease EDITORIALS J Am Soc Nephrol 13: 1409–1411, 2002 T Cells and Minimal Change Disease ROBYN CUNARD AND CAROLYN J. KELLY Research Service, VA San Diego Healthcare System; and Department of Medicine, University of California, San Diego, California. It is one of the ironies of medical practice that as physicians we the lymphokine may exert a direct effect on the GBM or can competently and confidently treat diseases of whose patho- activate mesangial cells to produce a factor that altered glo- genesis we remain woefully ignorant. merular permeability (2). Such is the case with minimal change disease, the most This publication preceded by over a decade the molecular common diagnosis associated with the nephrotic syndrome in definition of the T cell antigen receptor as a heterodimeric children (MCNS). The disease manifestations of nephrotic- structure derived from gene rearrangements (3) and the cloning range proteinuria, hypoalbuminemia, and hyperlipidemia in of the first cytokine or lymphokine (4). It preceded by several such patients are typically reversible with corticosteroid ther- years the earliest attempts to characterize subsets of T lympho- apy. MCNS has, by definition, no abnormalities apparent on cytes by their functional and phenotypic heterogeneity (5,6). analysis of kidney biopsy specimens by light microscopy. Shalhoub’s hypothesis has resurfaced multiple times since Humoral components of the immune system, such as Ig and 1974, as clinical investigators take advantage of increased complement, are absent on immunofluorescent analysis of cor- knowledge about immunology and technical breakthroughs to tical kidney sections. The sole abnormalities seen in the kidney reexamine the role of T cells in MCNS. In 2002, it is fair to say are at the ultrastructural level. The most prominent of these is that a role for T cells in MCNS continues to be an intriguing effacement of the visceral epithelial cell foot processes, a hypothesis that has been neither proven nor refuted. In this finding seen with many forms of glomerular pathology that are issue of JASN, Sahali et al. (7) describe the application of associated with nephrotic-range proteinuria. These ultrastruc- differential screening of a subtractive cDNA library to examine tural abnormalities typically resolve with corticosteroid-in- which, if any, T cell genes are upregulated during relapse of duced clinical remissions (1). MCNS. Their novel findings provide intriguing additional sup- What is the pathogenesis of this acquired and frequently port for Shalhoub’s hypothesis. reversible abnormality in glomerular ultrastructure and glomer- To understand why this is a difficult hypothesis to prove, it ular basement membrane (GBM) permeability? Almost 30 yr is helpful to step back and outline how T cells come to be ago, R. J. Shalhoub (2) proposed that lipoid nephrosis, an older implicated as necessary participants in the pathogenesis of term for nil lesion or MCNS, was a systemic disorder of T cell disease. In doing this, we make a distinction between the role function and cell-mediated immunity. This proposal, devel- of T cells in providing a helper function for B cell maturation oped in the format of a hypothesis paper, sought to reconcile a and antibody production and the role of T cells as more direct number of clinical observations with the rudimentary knowl- effectors of disease. Nephrologists have of course long been edge of T lymphocytes that existed in 1974. Shalhoub hypoth- cognizant of the importance of humoral effectors, such as esized that the abnormal expansion of a clone of T cells might antibody and complement, in the mediation of renal injury. result in the production of a lymphokine toxic to the GBM, Recognition that activated T cells can directly mediate renal resulting in markedly altered glomerular permeability to pro- injury, lead to alterations in renal function, and be associated tein. The evidence supporting this hypothesis included the with renal pathologic abnormalities, such as interstitial nephri- observations that minimal change disease frequently remits tis (8), glomerulonephritis (9), and glomerular crescents (10), with measles infection (viral-associated immunosuppression), has not been widely appreciated among practicing nephrolo- that the patients are highly susceptible to pneumococcal infec- gists (11). Why? tions (despite minimal losses of IgG in urine), that remissions Our clinicopathologic approaches to the diagnosis and clas- are induced with corticosteroid therapy and prolonged with sification of inflammatory renal disease have been heavily cyclophosphamide, and that similar lesions can occur in biased in favor of humoral immune reactants, such as IgG and Hodgkin disease (2). Shalhoub additionally hypothesized that complement. If IgG and complement are detected on immuno- fluorescent analysis of a kidney biopsy, they are usually pre- sumed to be contributing to the pathogenesis of disease. (In Correspondence to Carolyn J. Kelly, ACOS for Research, 151, 3350 La Jolla Village Drive, San Diego, CA 92161. Phone: 858-552-8585 ext. 7015; Fax: fact experimental data would suggest that is not always the 858-642-6243; E-mail: [email protected] case [12]) For example, we can demonstrate that antibody 1046-6673/1305-1409 reactive with GBM is present in serum, present in the renal Journal of the American Society of Nephrology biopsy, and associated with glomerular inflammation and al- Copyright © 2002 by the American Society of Nephrology terations in GFR in patients with anti-GBM disease. In a DOI: 10.1097/01.ASN.0000016406.82019.B3 laboratory setting, antibodies with GBM specificity can be 1410 Journal of the American Society of Nephrology J Am Soc Nephrol 13: 1409–1411, 2002 infused into experimental animals and elicit inflammation and into rats (29). Serum from patients with MCNS alters the albumin proteinuria, thereby fulfilling Koch’s postulates (13). permeability of rat glomerular epithelial cell monolayers (30). The Activated T cells are frequently present within the glomeruli identity of the factor(s) has remained enigmatic. Recent studies and interstitial compartments of biopsies from patients with have suggested that IL-4 and IL-13 are overexpressed in patients glomerulonephritis (14–16). However, unlike IgG, we cannot with steroid-responsive nephrotic syndrome in relapse (31). It is of as readily identify T cells with the same antigenic specificity in interest that these same cytokines can increase transcellular ion the peripheral blood, nor, until more recently, could we defin- transport over glomerular visceral epithelial cell monolayers. itively demonstrate with adoptive transfer that T cells with These effects on ion transport are associated with basolateral specificity for an antigen expressed within the glomerulus secretion of lysosomal proteinases, such as procathepsin L (32). mediate glomerulonephritis (17). T cell Ag receptors recognize Such effects may link abnormal cytokine expression to altered short linear peptides, derived from processed antigen, in asso- glomerular permeability. ciation with class I or class II MHC molecules on the surface A common feature of the immune abnormalities documented of antigen-presenting cells or target cells (18). Therefore, to above in patients with active MCNS is that the investigators identify T cells of a given Ag specificity in the peripheral studying those abnormalities made choices about what to mea- blood, knowledge of the epitope recognized is required, and sure. Assessments of phenotypic or functional differences be- that antigen must be in a form that can be recognized by T tween T cells from active MCNS patients and those in remission cells. Presently, an approximation of numbers of T cells reac- or normal controls were conducted with a hypothesis that the tive with a particular antigen can be experimentally determined measured parameter might be different in relapse versus remis- using immunostaining with MHC tetramers complexed with sion. The beauty of the approach taken by Sahali et al. (7) in this Ag (19). However, such a technique is not yet available for issue of JASN is that the data generated is largely independent of routine clinical diagnosis. preconceived determinations about which genes might be differ- What about minimal change disease? In this form of ne- entially expressed in MCNS relapse versus remission. Using this phrotic syndrome, T cells are only rarely encountered within approach, the investigators identified a number of differentially the glomerular compartment (20). Thus it is difficult to incrim- expressed transcripts relevant to T cell activation, signal transduc- inate them given their relative absence in the glomerulus. But tion, cell division, and transcriptional activation. Confirmation Shalhoub’s hypothesis was that clinical MCNS resulted from that some of these genes are upregulated in MCNS relapse com- “episodic or sustained domination by a clone of T cells,” which pared with remission off corticosteroids was obtained by assessing secreted a lymphokine toxic to the GBM, thereby altering RNA and/or protein expression in peripheral blood mononuclear permeability to protein (2). Are there differences in circulating cells from other MCNS patients (7). The use of patients with T cells in MCNS patients? membranous nephropathy (although the clinical status of those In patients with active MCNS, there may be alterations in T cell patients and their therapy was not explicitly described) presum- subpopulations in the peripheral blood, including increased
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