Glomerulonephritis Associated with Systemic Lupus Erythematosus

Chapter 508 Glomerulonephritis Associated with Systemic Lupus Erythematosus



Cynthia G. Pan, Ellis D. Avner



Systemic lupus erythematosus (SLE) is characterized by fever, weight loss, dermatitis, hematologic abnormalities, arthritis, and involvement of the heart, lungs, central nervous system, and kidneys (Chapter 152). Glomerulonephritis is the most important cause of morbidity and mortality in SLE. Renal disease in childhood SLE is present in up 80% patients and is more active than that seen in adults. Occasionally, renal disease is the only presenting clinical manifestation.



Pathogenesis and Pathology


The clinical manifestations of SLE are mediated by immune complexes. The classification of lupus nephritis of the World Health Organization (WHO) is based on a combination of light microscopy, immunofluorescence, and electron microscopy features. In patients with WHO class I nephritis (minimal mesangial lupus nephritis), no histologic abnormalities are detected on light microscopy but mesangial immune deposits are present on immunofluorescence or electron microscopy. In WHO class II nephritis (mesangial proliferative nephritis), light microscopy shows both mesangial hypercellularity and increased matrix along with mesangial deposits containing immunoglobulin and complement.


WHO class III nephritis and WHO class IV nephritis are interrelated lesions characterized by both mesangial and endocapillary lesions. Class III nephritis is defined by <50% glomeruli with involvement and class IV has ≥50% glomerular involvement. Immune deposits are present in both the mesangium and subendothelial areas. A subclassification scheme helps grade severity of the proliferative lesion based on whether the glomerular lesions are segmental (<50% glomerular tuft involved) or global (≥50% glomerular tuft involved). The WHO classification scheme also delineates whether there is a predominance of chronic disease versus active disease. Chronic injury results in glomerular sclerosis and is felt to be the consequence of significant proliferative disease seen in class III and IV. Other signs of active disease include capillary walls that are thickened secondary to subendothelial deposits (creating the wire-loop lesion), necrosis, and crescent formation. WHO class IV nephritis is associated with poorer outcomes but can be successfully treated with aggressive immunosuppressive therapy.


WHO class V nephritis

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Jun 18, 2016 | Posted by in PEDIATRICS | Comments Off on Glomerulonephritis Associated with Systemic Lupus Erythematosus

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