Renal pathology

4. - Type IV collagen forms network to which glycoprotein's attach c) visceral epithelial cells (podocytes; “foot processes”) i) composed of interdigitating processes embedded to basement membrane ii) adjacent foot processes are separated by 20-30 nm filtration slits bridged by thin diaphragm (nephrin) d) entire glomerulus is supported by mesangial cells i) lying between capillaries www.freelivedoctor.com

6. ii) podocytes important in maintaining this “function” - slit diaphragm maintain size- selectivity by specific proteins 1.- NEPHRIN : extend towards each other from neighboring podocytes comprising the slit diaphragm !! 2.- PODOCIN : intracellular (podocyte) protein where nephrin attaches - mutations in genes encoding these proteins give rise to nephrotic syndrome (i.e., glomerular disease) www.freelivedoctor.com

7. www.freelivedoctor.com

13. c) Acute renal failure : oliguria/anuria, recent onset of azotemia, can result from GN, tubular or interstitial disease d) Nephroliathiasis : renal stones, renal colic, hematuria, recurrent stone formation e) Chronic renal failure : 4 stages i)  renal reserve: GFR ~ 50% normal BUN & creatinine normal, pt. asymptomatic, more susceptible to develop azotemia ii) renal insufficiency: GFR 20-50% of normal, azotemia, anemia,  BP, polyuria/nocturia (via  concentrating ability) www.freelivedoctor.com

14. iii) renal failure : GFR less than 20-25% kidneys cannot regulate volume, ions: edema, hypocalcemia, metabolic acidosis, uremia with neurological, CV and GI complications iv) end stage renal disease : GFR < 5% of normal, terminal stage of uremia www.freelivedoctor.com

18. b) basement membrane thickening i) deposition of immune complexes on either the endothelial or epithelial side of GBM or w/in GBM itself ii) thickening of GBM proper as with diabetes mellitus (diabetic glomerulosclerosis) c) hyalinization (hyalinosis) and sclerosis i) accumulation of material that is eosinophilic and homogeneous - obliterates capillary lumen of glomerulus (sclerotic feature) www.freelivedoctor.com

22. - Ab bind along GBM forming a “linear pattern” - sometimes AGBM Ab cross react with BM of lung  GOODPASTURE SYNDROME - < 1% of GN cases - some cases show severe glomerular damage and rapidly progressive crescentic GN ii) Heymann nephritis - a form of membranous GN - Ab bind along GBM in “granular pattern” www.freelivedoctor.com

29. b) renal ablation GN i) any renal disease   GFR (30-50% of normal) - lead to end stage renal failure ii) patients develop proteinuria and diffuse glomerulosclerosis - initiated by unaffected glomeruli  hypertrophy to maintain function  single nephron hypertension  damage www.freelivedoctor.com

34. e) believed to be caused by i) deposition of immune complexes w/in capillary wall - IgG and C3 ii) formation of in situ immune complexes iii) refer to Heymans nephritis and Goodpasture syndrome iv) classified as non inflammatory since there is NO cellular proliferation f) In adults, a frequent association is with carcinoma !! (i.e., melanoma, lung and colon) www.freelivedoctor.com

36. d) Progression of disease i) stage I : small granular subepithelial deposits ii) stage II : “spikes” of BM protrude between deposits of electron dense material (e.g., IgG, C3) iii) stage III : deposits of electron dense material are incorporated into GBM iv) stage iv : GBM very distorted and damaged www.freelivedoctor.com

39. Minimal Change Disease www.freelivedoctor.com

41. g) T cell involvement suggested i) HL patients present w/ similar S & S ii) epithelial cell diseases have altered T ell function h) loss of lipoproteins through the glomeruli  accumulates lipids in proximal tubule cells  foamy cytoplasm. Together with lipids in the urine  LIPOID NEPHROSIS i) remission w/in 8 weeks with use of corticosteroid use (very dramatic response is one hallmark of this disease) j) relapses do not tend to progress to chronic renal failure www.freelivedoctor.com

43. c) occurs in the following setting: i) associated with other conditions - HIV - heroin addiction - sickle cell disease - morbid obesity ii) secondary event - IgA nephropathy iii) adaptive process to loss of kidney - renal ablation - advanced stages of other renal diseases (e.g., hypertension) iv) primary disease (e.g., idiopathic focal segmental glomerulosclerosis) www.freelivedoctor.com

44. d) most common cause of nephrotic syndrome in USA i) Hispanics and African Americans e) differs from minimal change disease i) higher incidence of hematuria, reduced GFR, and hypertension ii) poor response to corticosteroids iii) proteinuria is non selective iv) progression to chronic glomerulosclerosis v) IgM and C3 trapping on sclerotic segments www.freelivedoctor.com

45. vi) whether this is a specific disease or is an evolution of minimal change disease is unresolved !! - degeneration of visceral epithelial cells hallmark of FSGN - similar cell damage as seen in minimal change disease vii) genetic basis - NPHS1 gene  encodes nephrin - several mutations of this gene give rise to congenital nephrotic syndrome of the Finnish type (CNF) www.freelivedoctor.com

46. Focal segmental glomerulosclerosis www.freelivedoctor.com

49. 2. Waldenstrom macroglobulinemia a)  serum IgM b)  blood viscosity c) renal involvement results in partial or total occlusion of glomerular capillaries 3. Cryoglobulinemia a) IgM or IgG b) defined by their capacity to precipitate at 4  C c) renal disease is often immune complex mediated d) microthrombi in glomeruli e) mesangial & endothelial cell proliferation www.freelivedoctor.com

53. ii) occur by progressive accumulation of GBM material - severity and duration of hyperglycemia !! ?? b) etiology of proteinuria not known i) non-nephrotic proteinuria ii) nephrotic c) earliest lesion is thickening of GBM d) followed by glomerular enlargement e) “diffuse glomerulosclerosis” refers to enlarged glomeruli w/expanded mesangium and diffusely thickened GBM www.freelivedoctor.com

54. f) nodular glomerulosclerosis (i.e., Kimmelstiel-Wilson Disease) – highly specific for diabetes g) one of leading causes of chronic renal failure in USA h) 2 processes play role in diabetic glomerular lesions i) metabolic defect (i.e., glycosylation end products that: -  GBM thickening -  mesangial matrix ii) hemodynamic effects: - glomerular hypertrophy (  GFR) - develop of glomerulosclerosis www.freelivedoctor.com

55. Diabetic glomerulosclerosis www.freelivedoctor.com

57. Amyloid nephropathy www.freelivedoctor.com

59. d) disease most common in children i) 3-8 yrs e) does occur in adults where disease is more severe i) may develop rapidly progressive form of glomerulornephritis with many crescents f) onset often follows upper respiratory infection i) IgA deposits in mesangium which has led to concept that IgA nephropathy (purely a renal disease) and Henoch-Schönlein Purpura are spectra of same disease www.freelivedoctor.com

61. c) glomerular injury may be either global (entire glomerulus) or segmental d) glomerulornephritis clinically characterized by the NEPHRITIC SYNDROME i) may present with only portions of these S & S (i.e., hematuria) ii) on occasion, proteinuria may predominate e) different forms of GN are differentiated via microscopy www.freelivedoctor.com

62. f) neutrophils contribute to  cellularity, particularly in children i) many neutrophils in glomeruli referred to as “exudative” g) proliferation of podocytes and Bowman’s capsule (visceral and parietal epithelium, respectively) i) leads to formation of “crescents” - highly cellular lesions - extend from Bowman’s capsule into glomerulus having shape of crescents. These are severe lesions (associated with necrosis, early and fibrosis, late) www.freelivedoctor.com

64. b) confirmed by EM i) presence of subepithelial “humps” ii) peripheral granular staining directed - IgG - C3 c) alternatively, circulating immune complex do not penetrate GBM but localize to i) mesangium or subendothelium d) trapping is affected by: i) size and charge of aggregates ii) glomerular hemodynamics iii) presence of vasoactive substances www.freelivedoctor.com

65. e) Ag may be exogenous or endogenous i) exogenous - bacterial Ag induced by 1. Strep infections 2. bacterial endocarditis 3. viruses (HBV) ii) endogenous - DNA in pathogenesis of lupus 2. In Situ immune complex formation a) Goodpasture Syndrome b) Ag (endogenous) already embedded in GBM i) Ab binds !! ii) linear localization of IgG along GBM www.freelivedoctor.com

66. c) Ag-Ab interaction activates complement ! i) rapidly progressive GN occurs 3. Alternative complement activation a) focal glomerulornephritis i) caused by IgA b) form of membranoproliferative GN 4. Cell-mediated Immunity a) no direct evidence for any specific GN caused by cell mediated processesi) indirect evidence for a delayed (type iv) cell type GN - lymphocytes from some patients with GN react in vitro with a glomerular Ag www.freelivedoctor.com

70. e) latent period of ~ 10-14 days f) diffuse enlargement and hypercellularity of glomeruli, hypercellularity due to: i) proliferation of endothelial and mesangial cells and infiltration of neutrophils and monocytes g) characteristics: i) subepithelial “humps” of GBM ii) granular IgG and C3 along GBM in association with “humps” h) Clinical: i) most resolve but in rare occasions can progress to develop many crescents and renal failure www.freelivedoctor.com

71. Acute GN (post infectious GN) www.freelivedoctor.com

73. b) two major groups: Types I and II (+ III) i) Type I: majority of cases are idiopathic. Associations with - HBV - HCV - bacterial endocarditis - strep infections - granular deposition of Ig (IgG, IgM) and complement (C3) and C1q and C4 ii) type II (and III) - circulating C3 Ab (C3 nephritic factor)   C3 (hypocomplementemia) www.freelivedoctor.com

74. - characteristic “ribbon-like” zone of  cellularity on thickened GBM (“dense deposit disease”) c) Clinical: i) occurs primarily in older children and young adults ii) nephritic or nephrotic syndrome iii) low levels of C3 iv) do not have postinfectious GN v) no systemic inflammatory condition vi) most progress to end-stage renal failure, regardless of treatment !! www.freelivedoctor.com

75. Membranoproliferative GN Type I www.freelivedoctor.com

77. i) involves the glomeruli w/more severe inflammation h) hematoxylin bodies  only light microscopic feature of tissue damage i) episodic inflammation – usually present with old lesions j) IgG most common. IgA and IgM also present. Complement present i) IgG, IgA, IgM, C3, C4 and C1q present in same glomerulus “FULL HOUSE” www.freelivedoctor.com

78. k) 5 classes based on WHO classification i) Class I – histologically normal ii) Class II – pure mesangial lesion iii) Class III – focal and segmental GN iv) Class IV – diffuse proliferative GN v) Class V – diffuse membranous - Class II and V have more benign course relative to Class III and IV l) renal disease  major consequence of SLE i) renal failure  cause of death in ~ 33 % of patients with SLE www.freelivedoctor.com

80. d) IgA nephropathy (Berger Disease) i) association with chronic liver disease - impaired capacity to remove circulating immune complexes ii) IgA and fibronectin found in > 70 % of IgA nephropathy patients. iii) Ag involve bacterial, viral and dietary - infectious agents is suggested from data showing hematuria following upper respiratory or GI infection !! - dietary agents  milk proteins I in mesangium; gluten-sensitivity www.freelivedoctor.com

81. iv) C3 and properdin (via activation of alternate pathway) usually present together with IgA in mesangium - C1q and C4 (classic pathway activation) are typically absent v) IgA nephropathy is a mesangial proliferative lesion (granular deposits) vi) clinical - common in young men (15-30) - presents with hematuria - nephrotic type proteinuria is uncommon (may indicate more severe glomerular damage) www.freelivedoctor.com

82. - ~ 20 % of IgA nephropathy patients progress to end-stage renal failure !! - most common type of 1  GN in several parts of the world (France, Italy, Japan, Singapore and Austria) -- ~ 20 %. In USA is responsible for ~ 3-10 % of 1  GN www.freelivedoctor.com

83. IgA nephropathy (Berger Disease) www.freelivedoctor.com

85. iii) does not denote a specific etiologic form of GN b) most patients with substantial (~ 80%) crescents progress to renal failure c) Fibrin in Bowman’s capsule is important for the formation of glomerular crescents i) Tx with anticoagulants d) associated with areas of segmental necrosis within glomeruli e) Types: i) Type I – anti-GBM antibody disease (GOODPASTURE SYNDROME) or idiopathic www.freelivedoctor.com

86. - plasmapheresis to remove circulating Ab is helpful in this type of RPGN (i.e., crescentic) - etiology unknown ii) Type II – immune-complex mediated disease - can be complication of any of the immune complex nephritides  SLE, IgA nephropathy,  HS Purpura  all these show granular pattern (characteristic of immune complex) - not helped with plasmapheresis www.freelivedoctor.com

87. iii) Type III – pauci-immune type - lack of anti-GBM Ab or immune complexes - patients do have ANCA (~90%)  either c or p patterns } in some cases, is a component of vasculitides (i.e., Wegener Granulomatosis) f) clinical: i) hematuria with red cell cast in urine ii) transplant or chronic dialysis in most patients www.freelivedoctor.com

88. Crescentic GN www.freelivedoctor.com

110. c) This leads to appearance of fibroid necrosis in small arteries and arterioles and intravascular thrombosis d) platelets (platelet derived growth factors) and plasma cause intimal hyperplasia of vessels resulting in hyperplastic arteriosclerosis, which is typical of malignant hypertension e) narrowing of renal afferent arteriole stimulates angiotensin II production (ischemic-induced) with aldosterone secretion increases www.freelivedoctor.com

139. v) deep invasive Cancer, survival (5yr) is <20% with overall 5 yr survival at 50-60% b) - Cancer of ureters is very rare i) 5 yr survival <10% www.freelivedoctor.com

143. - Type IV collagen forms network to which glycoprotein's attach c) visceral epithelial cells (podocytes; “foot processes”) i) composed of interdigitating processes embedded to basement membrane ii) adjacent foot processes are separated by 20-30 nm filtration slits bridged by thin diaphragm (nephrin) d) entire glomerulus is supported by mesangial cells i) lying between capillaries www.freelivedoctor.com

145. ii) podocytes important in maintaining this “function” - slit diaphragm maintain size- selectivity by specific proteins 1.- NEPHRIN : extend towards each other from neighboring podocytes comprising the slit diaphragm !! 2.- PODOCIN : intracellular (podocyte) protein where nephrin attaches - mutations in genes encoding these proteins give rise to nephrotic syndrome (i.e., glomerular disease) www.freelivedoctor.com

152. c) Acute renal failure : oliguria/anuria, recent onset of azotemia, can result from GN, tubular or interstitial disease d) Nephroliathiasis : renal stones, renal colic, hematuria, recurrent stone formation e) Chronic renal failure : 4 stages i)  renal reserve: GFR ~ 50% normal BUN & creatinine normal, pt. asymptomatic, more susceptible to develop azotemia ii) renal insufficiency: GFR 20-50% of normal, azotemia, anemia,  BP, polyuria/nocturia (via  concentrating ability) www.freelivedoctor.com

153. iii) renal failure : GFR less than 20-25% kidneys cannot regulate volume, ions: edema, hypocalcemia, metabolic acidosis, uremia with neurological, CV and GI complications iv) end stage renal disease : GFR < 5% of normal, terminal stage of uremia www.freelivedoctor.com

157. b) basement membrane thickening i) deposition of immune complexes on either the endothelial or epithelial side of GBM or w/in GBM itself ii) thickening of GBM proper as with diabetes mellitus (diabetic glomerulosclerosis) c) hyalinization (hyalinosis) and sclerosis i) accumulation of material that is eosinophilic and homogeneous - obliterates capillary lumen of glomerulus (sclerotic feature) www.freelivedoctor.com

161. - Ab bind along GBM forming a “linear pattern” - sometimes AGBM Ab cross react with BM of lung  GOODPASTURE SYNDROME - < 1% of GN cases - some cases show severe glomerular damage and rapidly progressive crescentic GN ii) Heymann nephritis - a form of membranous GN - Ab bind along GBM in “granular pattern” www.freelivedoctor.com

168. b) renal ablation GN i) any renal disease   GFR (30-50% of normal) - lead to end stage renal failure ii) patients develop proteinuria and diffuse glomerulosclerosis - initiated by unaffected glomeruli  hypertrophy to maintain function  single nephron hypertension  damage www.freelivedoctor.com

173. e) believed to be caused by i) deposition of immune complexes w/in capillary wall - IgG and C3 ii) formation of in situ immune complexes iii) refer to Heymans nephritis and Goodpasture syndrome iv) classified as non inflammatory since there is NO cellular proliferation f) In adults, a frequent association is with carcinoma !! (i.e., melanoma, lung and colon) www.freelivedoctor.com

175. d) Progression of disease i) stage I : small granular subepithelial deposits ii) stage II : “spikes” of BM protrude between deposits of electron dense material (e.g., IgG, C3) iii) stage III : deposits of electron dense material are incorporated into GBM iv) stage iv : GBM very distorted and damaged www.freelivedoctor.com

178. Minimal Change Disease www.freelivedoctor.com

180. g) T cell involvement suggested i) HL patients present w/ similar S & S ii) epithelial cell diseases have altered T ell function h) loss of lipoproteins through the glomeruli  accumulates lipids in proximal tubule cells  foamy cytoplasm. Together with lipids in the urine  LIPOID NEPHROSIS i) remission w/in 8 weeks with use of corticosteroid use (very dramatic response is one hallmark of this disease) j) relapses do not tend to progress to chronic renal failure www.freelivedoctor.com

182. c) occurs in the following setting: i) associated with other conditions - HIV - heroin addiction - sickle cell disease - morbid obesity ii) secondary event - IgA nephropathy iii) adaptive process to loss of kidney - renal ablation - advanced stages of other renal diseases (e.g., hypertension) iv) primary disease (e.g., idiopathic focal segmental glomerulosclerosis) www.freelivedoctor.com

183. d) most common cause of nephrotic syndrome in USA i) Hispanics and African Americans e) differs from minimal change disease i) higher incidence of hematuria, reduced GFR, and hypertension ii) poor response to corticosteroids iii) proteinuria is non selective iv) progression to chronic glomerulosclerosis v) IgM and C3 trapping on sclerotic segments www.freelivedoctor.com

184. vi) whether this is a specific disease or is an evolution of minimal change disease is unresolved !! - degeneration of visceral epithelial cells hallmark of FSGS - similar cell damage as seen in minimal change disease vii) genetic basis - NPHS1 gene  encodes nephrin - several mutations of this gene give rise to congenital nephrotic syndrome of the Finnish type (CNF) www.freelivedoctor.com

185. Focal segmental glomerulosclerosis www.freelivedoctor.com

188. 2. Waldenstrom macroglobulinemia a)  serum IgM b)  blood viscosity c) renal involvement results in partial or total occlusion of glomerular capillaries 3. Cryoglobulinemia a) IgM or IgG b) defined by their capacity to precipitate at 4  C c) renal disease is often immune complex mediated d) microthrombi in glomeruli e) mesangial & endothelial cell proliferation www.freelivedoctor.com

192. ii) occur by progressive accumulation of GBM material - severity and duration of hyperglycemia !! ?? b) etiology of proteinuria not known i) non-nephrotic proteinuria ii) nephrotic c) earliest lesion is thickening of GBM d) followed by glomerular enlargement e) “diffuse glomerulosclerosis” refers to enlarged glomeruli w/expanded mesangium and diffusely thickened GBM www.freelivedoctor.com

193. f) nodular glomerulosclerosis (i.e., Kimmelstiel-Wilson Disease) – highly specific for diabetes g) one of leading causes of chronic renal failure in USA h) 2 processes play role in diabetic glomerular lesions i) metabolic defect (i.e., glycosylation end products that: -  GBM thickening -  mesangial matrix ii) hemodynamic effects: - glomerular hypertrophy (  GFR) - develop of glomerulosclerosis www.freelivedoctor.com

194. Diabetic glomerulosclerosis www.freelivedoctor.com

196. Amyloid nephropathy www.freelivedoctor.com

198. d) disease most common in children i) 3-8 yrs e) does occur in adults where disease is more severe i) may develop rapidly progressive form of glomerulornephritis with many crescents f) onset often follows upper respiratory infection i) IgA deposits in mesangium which has led to concept that IgA nephropathy (purely a renal disease) and Henoch-Schönlein Purpura are spectra of same disease www.freelivedoctor.com

200. c) glomerular injury may be either global (entire glomerulus) or segmental d) glomerulornephritis clinically characterized by the NEPHRITIC SYNDROME i) may present with only portions of these S & S (i.e., hematuria) ii) on occasion, proteinuria may predominate e) different forms of GN are differentiated via microscopy www.freelivedoctor.com

201. f) neutrophils contribute to  cellularity, particularly in children i) many neutrophils in glomeruli referred to as “exudative” g) proliferation of podocytes and Bowman’s capsule (visceral and parietal epithelium, respectively) i) leads to formation of “crescents” - highly cellular lesions - extend from Bowman’s capsule into glomerulus having shape of crescents. These are severe lesions (associated with necrosis, early and late fibrosis) www.freelivedoctor.com

203. b) confirmed by EM i) presence of subepithelial “humps” ii) peripheral granular staining directed - IgG - C3 c) alternatively, circulating immune complex do not penetrate GBM but localize to i) mesangium or subendothelium d) trapping is affected by: i) size and charge of aggregates ii) glomerular hemodynamics iii) presence of vasoactive substances www.freelivedoctor.com

204. e) Ag may be exogenous or endogenous i) exogenous - bacterial Ag induced by 1. Strep infections 2. bacterial endocarditis 3. viruses (HBV) ii) endogenous - DNA in pathogenesis of lupus 2. In Situ immune complex formation a) Goodpasture Syndrome b) Ag (endogenous) already embedded in GBM i) Ab binds !! ii) linear localization of IgG along GBM www.freelivedoctor.com

205. c) Ag-Ab interaction activates complement ! i) rapidly progressive GN occurs 3. Alternative complement activation a) focal glomerulornephritis i) caused by IgA b) form of membranoproliferative GN 4. Cell-mediated Immunity a) no direct evidence for any specific GN caused by cell mediated processesi) indirect evidence for a delayed (type iv) cell type GN - lymphocytes from some patients with GN react in vitro with a glomerular Ag www.freelivedoctor.com

209. e) latent period of ~ 10-14 days f) diffuse enlargement and hypercellularity of glomeruli, hypercellularity due to: i) proliferation of endothelial and mesangial cells and infiltration of neutrophils and monocytes g) characteristics: i) subepithelial “humps” of GBM ii) granular IgG and C3 along GBM in association with “humps” h) Clinical: i) most resolve but in rare occasions can progress to develop many crescents and renal failure www.freelivedoctor.com

210. Acute GN (post infectious GN) www.freelivedoctor.com

212. b) two major groups: Types I and II (+ III) i) Type I: majority of cases are idiopathic. Associations with - HBV - HCV - bacterial endocarditis - strep infections - granular deposition of Ig (IgG, IgM) and complement (C3) and C1q and C4 ii) type II (and III) - circulating C3 Ab (C3 nephritic factor)   C3 (hypocomplementemia) www.freelivedoctor.com

213. - characteristic “ribbon-like” zone of  cellularity on thickened GBM (“dense deposit disease”) c) Clinical: i) occurs primarily in older children and young adults ii) nephritic or nephrotic syndrome iii) low levels of C3 iv) do not have postinfectious GN v) no systemic inflammatory condition vi) most progress to end-stage renal failure, regardless of treatment !! www.freelivedoctor.com

214. Membranoproliferative GN Type I www.freelivedoctor.com

216. i) involves the glomeruli w/more severe inflammation h) hematoxylin bodies  only light microscopic feature of tissue damage i) episodic inflammation – usually present with old lesions j) IgG most common. IgA and IgM also present. Complement present i) IgG, IgA, IgM, C3, C4 and C1q present in same glomerulus “FULL HOUSE” www.freelivedoctor.com

217. k) 5 classes based on WHO classification i) Class I – histologically normal ii) Class II – pure mesangial lesion iii) Class III – focal and segmental GN iv) Class IV – diffuse proliferative GN v) Class V – diffuse membranous - Class II and V have more benign course relative to Class III and IV l) renal disease  major consequence of SLE i) renal failure  cause of death in ~ 33 % of patients with SLE www.freelivedoctor.com

219. d) IgA nephropathy (Berger Disease) i) association with chronic liver disease - impaired capacity to remove circulating immune complexes ii) IgA and fibronectin found in > 70 % of IgA nephropathy patients. iii) Ag involve bacterial, viral and dietary - infectious agents is suggested from data showing hematuria following upper respiratory or GI infection !! - dietary agents  milk proteins I in mesangium; gluten-sensitivity www.freelivedoctor.com

220. iv) C3 and properdin (via activation of alternate pathway) usually present together with IgA in mesangium - C1q and C4 (classic pathway activation) are typically absent v) IgA nephropathy is a mesangial proliferative lesion (granular deposits) vi) clinical - common in young men (15-30) - presents with hematuria - nephrotic type proteinuria is uncommon (may indicate more severe glomerular damage) www.freelivedoctor.com

221. - ~ 20 % of IgA nephropathy patients progress to end-stage renal failure !! - most common type of 1  GN in several parts of the world (France, Italy, Japan, Singapore and Austria) -- ~ 20 %. In USA is responsible for ~ 3-10 % of 1  GN www.freelivedoctor.com

222. IgA nephropathy (Berger Disease) www.freelivedoctor.com

224. iii) does not denote a specific etiologic form of GN b) most patients with substantial (~ 80%) crescents progress to renal failure c) Fibrin in Bowman’s capsule is important for the formation of glomerular crescents i) Tx with anticoagulants d) associated with areas of segmental necrosis within glomeruli e) Types: i) Type I – anti-GBM antibody disease (GOODPASTURE SYNDROME) or idiopathic www.freelivedoctor.com

225. - plasmapheresis to remove circulating Ab is helpful in this type of RPGN (i.e., crescentic) - etiology unknown ii) Type II – immune-complex mediated disease - can be complication of any of the immune complex nephritides  SLE, IgA nephropathy,  HS Purpura  all these show granular pattern (characteristic of immune complex) - not helped with plasmapheresis www.freelivedoctor.com

226. iii) Type III – pauci-immune type - lack of anti-GBM Ab or immune complexes - patients do have ANCA (~90%)  either c or p patterns } in some cases, is a component of vasculitides (i.e., Wegener Granulomatosis) f) clinical: i) hematuria with red cell cast in urine ii) transplant or chronic dialysis in most patients www.freelivedoctor.com

227. Crescentic GN www.freelivedoctor.com

Renal pathology

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