title: Renal amyloidosis revisited: amyloid distribution, dynamics and biochemical type.
abstract: BACKGROUND
  Renal amyloidosis results from protein misfolding and leads to progressive renal insufficiency. Few data are available concerning the relevance of the histomorphological patterns and the dynamics of the disease process.
  
  
  METHODS
  Cases of renal amyloidosis in native kidney biopsies (n = 203) were retrospectively evaluated for the pattern of amyloid distribution, the extent of glomerular amyloid deposition and the amount of interstitial fibrosis and tubular atrophy. One hundred and fifty-eight cases were characterized by immunohistochemistry to determine the biochemical amyloid type. Morphological findings were correlated with available clinical data.
  
  
  RESULTS
  According to the predominant site of amyloid deposition, 84.6% showed a glomerular, 9.4% a vascular and 6% a tubulointerstitial distribution pattern. Within the glomeruli, amyloid was initially deposited in a focal segmental fashion that became diffuse and global in later stages. Most cases were identified as AL lambda (84/158) or AA (68/158). There was no correlation between the biochemical type and the distribution pattern. Serum creatinine correlated well with interstitial fibrosis and tubular atrophy and proteinuria with the glomerular amyloid load.
  
  
  CONCLUSIONS
  The relevance of the different distribution patterns is unclear at the moment, but they may be due to the physicochemical properties of the amyloid fibrils in a given patient. This may become important in future anti-fibrillar therapies.
authors.affiliation: Pathology, University Hospital Basel, Basel, Switzerland. hhopfer@uhbs.ch
authors.forename: Helmut
authors.initials: H
authors.lastname: Hopfer
authors.forename: Thorsten
authors.initials: T
authors.lastname: Wiech
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Mihatsch
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000503:physiopathology*; D002648:Child; D003404:Creatinine / Q000097:blood; D005260:Female; D005500:Follow-Up Studies; D005919:Glomerular Filtration Rate; D006801:Humans; D007124:Immunoenzyme Techniques; D007674:Kidney Diseases / Q000503:physiopathology*; D007678:Kidney Glomerulus / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D003404:Creatinine