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Lupus-related podocytopathy. Could it be a new entity within the spectrum of lupus nephritis?
Lupus-related podocytopathy. Could it be a new entity within the spectrum of lupus nephritis?
Silvina Gutiérreza, Juan P. Petitib, Juan P Petitia, Ana L. De Paulb, Ana L De Paula, Alicia I. Torresb, Alicia I Torresa, Jorge H. Mukdsib, Jorge H Mukdsia
a Centro de Microscopía Electrónica. Facultad de Ciencias Médicas. Universidad Nacional de Córdoba, Córdoba, Córdoba, Argentina,
b Centro de Microscopía Electrónica. Facultad de Ciencias Médicas. Universidad Nacional de Córdoba, Córdoba, Argentina,
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    "textoCompleto" => "<p class="elsevierStylePara">The classification of lupus nephritis was revised by the ISN&#47;RPS in 2003&#46; The increasingly recognized phenomenon of apparent minimal change disease &#40;MCD&#41; in the context of systemic lupus erythematosus &#40;SLE&#41;&#44; is not accepted in the above classification and is associated to a recent new pathological entity called lupus podocitopathy&#46;<span class="elsevierStyleSup">1</span> A 32-year-old caucasian woman presented with arthralgia and swelling of the face&#44; hands&#44; and legs&#46; Physical exam revealed pretibial edema and a patch of skin thickening on the left flank&#44; consistent with morphea&#46; Blood presure was 130&#47;70mmHg&#59; proteinuria 4&#46;5g&#47;dl&#59; serum creatinine 0&#46;9mg&#47;dl&#59; and albumin 2 g&#47;dl&#46; Urinalysis revealed fat casts&#46; Serology was negative for hepatitis B&#44; C&#44; HIV-1 and HIV-2&#46; ANA titer was 1&#47;1300&#44; C3 70mg&#47;dl and anti ds-DNA was elevated&#46; There was no history of nonsteroidal anti-inflammatory drug use in the patient&#46; A diagnosis of SLE was made&#46; Sections from the needle renal biopsy showed cortex with 10 normocellular glomeruli with mild mesangial hypercellularity and mesangial matrix increased&#46; There were no evident tubular&#44; interstitial&#44; and vascular lesions &#40;Figure 1 A&#41;&#46; Immunofluorescence microscopy revealed mesangial granular deposition of IgG &#40;2&#43;&#41; &#40;Figure 1 B&#41;&#44; IgA &#40;1&#43;&#41;&#44; IgM &#40;1&#43;&#41;&#44; C3 &#40;2&#43;&#41; &#40;Figure 1 C&#41; and C1q &#40;3&#43;&#41; &#40;Figure 1 D&#41;&#46; Ultrastructural analysis showed diffuse effacement &#40;~80&#37;&#41; of the epithelial cell food processes and vacuoles &#40;Figure 2 A&#41;&#46; Moreover few electron-dense deposits were noted in mildly expanded mesangium &#40;Figure 2 B&#41;&#46; Subepithelial or subendothelial deposits were not observed in the biopsy&#46; Numerous tubulorreticular inclusions within endothelial cells of glomerular capillary were also seen &#40;Figure 2 C&#41;&#46; A diagnosis of lupus podocytopathy and lupus nephritis Class I &#40;ISN&#47;RPS&#41; was made&#46; Of particular interest is the podocyte involvement in different types of lupus glomerulonephritis&#46; For example&#44; patients with non-nephrotic proteinuria and lupus nephritis Class I and II &#40;ISN-RPS&#41; have not revealed significant evidence of effacement of the foot processes&#46; Nevertheless&#44; some adult and children show minimal or proliferative mesangial lupus nephritis and nephrotic proteinuria without peripheral immune complex&#44; exhibiting extensive podocyte effacement&#44; consistent with lupus podocytopathy<span class="elsevierStyleSup">1</span>&#46; It is difficult to propose an exact pathogenic mechanism for this lesion given that immune deposits are no detected in glomerular basement membrane&#44; even though it has been hypothesized different mechanisms&#46; Abnormal release of IL-13 from aberrant T cell<span class="elsevierStyleSup">2</span>&#44; crosstalk between renal dendritic cells and Th cells<span class="elsevierStyleSup">3</span> may directly damage to podocytes&#46; Our patient was treated with high-dose prednisone&#46; Six month later she remained normotensive&#44; had no edema&#44; with normal serum creatinine and decreased urinary protein excretion &#40;0&#44;5g&#47;d&#41;&#46; In agreement with this result Kraft et al<span class="elsevierStyleSup">1</span> have shown a significant reduction in proteinuria at last follow-up&#46; Therefore&#44; the podocytopathy in the SLE context responded to oral corticosteroids&#44; remarking the important therapeutic implications of the diagnosis of this particular entity&#46; In summary&#44; lupus podocytopathy has become an intersting point both clinical discussion and futures investigations about the role of podocyte and it should be added to the classification of lupus nephritis&#46;</p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">Conflict of interest</span></p><p class="elsevierStylePara">The authors declare that there is no conflict of interest associated with this manuscript&#46;</p><p class="elsevierStylePara"><a href="grande&#47;11138&#95;108&#95;20876&#95;en&#95;11138&#95;f1&#46;jpg" class="elsevierStyleCrossRefs"><img src="11138_108_20876_en_11138_f1.jpg" alt="Light microscopy and immunofluorescence findings"></img></a></p><p class="elsevierStylePara">Figure 1&#46; Light microscopy and immunofluorescence findings</p><p class="elsevierStylePara"><a href="grande&#47;11138&#95;108&#95;20877&#95;en&#95;11138&#95;f2&#46;jpg" class="elsevierStyleCrossRefs"><img src="11138_108_20877_en_11138_f2.jpg" alt="Ultrastructural findings"></img></a></p><p class="elsevierStylePara">Figure 2&#46; Ultrastructural findings</p>"
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Información del artículo
ISSN: 02116995
Idioma original: Inglés
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