| Pathologic Classification of Diabetic Nephropathy
Thijs W. Cohen Tervaert,* Antien L. Mooyaart,* Kerstin Amann,† Arthur H. Cohen,‡ H. Terence Cook,§ Cinthia B. Drachenberg,_ Franco Ferrario,¶ Agnes B. Fogo,** Mark Haas,‡ Emile de Heer,* Kensuke Joh,†† Laure H. Noe¨ l,‡‡ Jai Radhakrishnan,§§ Surya V. Seshan,_ _ Ingeborg M. Bajema,* and Jan A. Bruijn,* on behalf of the Renal Pathology Society
*Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands; †Department of Pathology, University of Erlangen-Nuernberg, Erlangen, Germany; ‡Department of Pathology, Cedars-Sinai Medical Center, Los Angeles, California; §Department of Histopathology, Hammersmith Hospital, London, United Kingdom; _Department of Pathology, University of Maryland, Baltimore, Maryland; ¶Renal Immunopathology Center, San Carlo Borromeo Hospital, Milan, Italy; **Department of Pathology, Vanderbilt University Medical Center, Nashville, Tennessee; ††Division of Pathology, Sendai-Shaho Hospital, Sendai City, Japan; ‡‡Department of Pathology, Hoˆ pital Necker, Universite´ Rene´ Descartes, Paris, France; §§Department of Medicine, Columbia University, New York, New York; and _ _Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, New York, New York
ABSTRACT Although pathologic classifications exist for several renal diseases, including IgA nephropathy, focal segmental glomerulosclerosis, and lupus nephritis, a uniform classification for diabetic nephropathy is lacking. Our aim, commissioned by the Research Committee of the Renal Pathology Society, was to develop a consensus classification combining type1 and type 2 diabetic nephropathies. Such a classification should discriminate lesions by various degrees of severity that would be-easy to use internationally in clinical practice. We divide diabetic nephropathy into four hierarchical glomerular lesions with a separate evaluation for degrees of interstitial and vascular involvement. Biopsies diagnosed as diabetic nephropathy are classified as follows: Class I, glomerular basement membrane thickening: isolated glomerular basement membrane thickening and only mild, nonspecific changes by light microscopy that do not meet the criteria of classes II through IV. Class II, mesangial expansion, mild (IIa) or severe (IIb): glomeruli classified as mild or severe mesangial expansion but without nodular sclerosis (Kimmelstiel–Wilson lesions) or global glomerulosclerosis in more than 50% of glomeruli. Class III, nodular sclerosis (Kimmelstiel–Wilson lesions): at least one glomerulus with nodular increase in mesangial matrix (Kimmelstiel–Wilson) without changes described in class IV. Class IV, advanced diabetic glomerulosclerosis: more than 50% global glomerulosclerosis with other clinical or pathologic evidence that sclerosis is attributable to diabetic nephropathy. A good interobserver reproducibility for the four classes of DN was shown (intraclass correlation coefficient _ 0.84) in a test of this classification J Am Soc Nephrol ●●: –, 2010. doi: 10.1681/ASN.2010010010
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Pediatr Nephrol DOI 10.1007/s00467-009-1317-4
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| Small vessel vasculitis Paul Brogan & Despina Eleftheriou &Michael Dillon
Abstract The pediatric small vessel vasculitides reviewed in this article are Henoch–Schönlein purpura (HSP) and the anti-neutrophil cytoplasmic antibody-associated vasculitides (AAV). The new classification criteria for HSP and Wegener’s granulomatosis are now validated and will facilitate the conduct of future epidemiological studies and clinical trials. The clinical manifestations of small vessel vasculitis in children are described, and current therapies discussed. There is a lack of good clinical trial data on which to base therapy for HSP. Similarly, data based on randomized controlled trials (RCTs) for pediatric AAV are lacking, although children with AAV are for the first time now included in a RCT of mycophenolate mofetil versus cyclophosphamide. Significant challenges remain in the field of pediatric small vessel vasculitis, including the development of validated disease outcome measures and biomarkers to be used in clinical trials. Lastly, long-term outcome data are lacking in survivors of pediatric small vessel vasculitis.
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