Glomerulonephritis

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  • Glomerulonephritis

    1. 1. GLOMERULONEPHRITIDES SIMON E. PRINCE, D.O., F.A.C.P., F.A.S.N. Clinical Assistant Professor of Medicine NYU School of Medicine Sunday, March 15, 2009
    2. 2. Classic Patterns of Glomerular Diseases Sunday, March 15, 2009
    3. 3. Classic Patterns of Glomerular Diseases Distinguished by clinical factors and urinary sediment NEPHROTIC NEPHRITIC Sunday, March 15, 2009
    4. 4. NEPHROTIC Sunday, March 15, 2009
    5. 5. NEPHROTIC Nephrosis: Urinary sediment: “Bland” Heavy proteinuria and lipiduria Few cells or casts Less often HTN Relatively preserved renal function Sunday, March 15, 2009
    6. 6. Sunday, March 15, 2009
    7. 7. NEPHRITIC SYNDROME Sunday, March 15, 2009
    8. 8. NEPHRITIC SYNDROME Focal vs Diffuse Nephritic (Glomerulonephritis) Rapidly progressive GN Acute GN Chronic GN Sunday, March 15, 2009
    9. 9. NEPHRITIC SYNDROME Focal vs Diffuse Nephritic (Glomerulonephritis) Rapidly progressive GN Acute GN Chronic GN Usually caused by Immunocomplex (Ag-Ab) reaction and deposition in the GBM Sunday, March 15, 2009
    10. 10. NEPHRITIC SYNDROME Focal vs Diffuse Nephritic (Glomerulonephritis) Rapidly progressive GN Acute GN Chronic GN Usually caused by Immunocomplex (Ag-Ab) reaction and deposition in the GBM Some disorders Immunocomplex formation happens distal to the GBM and deposit in the subepithelial space Sunday, March 15, 2009
    11. 11. Clinical Characteristics of NEPHRITIC SYNDROME Sunday, March 15, 2009
    12. 12. Clinical Characteristics of NEPHRITIC SYNDROME Urinary sediment: “Active” RBCs (dysmorphic), RBC Casts, Granular casts Sunday, March 15, 2009
    13. 13. Clinical Characteristics of NEPHRITIC SYNDROME Urinary sediment: “Active” RBCs (dysmorphic), RBC Casts, Granular casts Varying degrees of proteinuria (often not nephrotic range) Sunday, March 15, 2009
    14. 14. Clinical Characteristics of NEPHRITIC SYNDROME Urinary sediment: “Active” RBCs (dysmorphic), RBC Casts, Granular casts Varying degrees of proteinuria (often not nephrotic range) Hypertension Sunday, March 15, 2009
    15. 15. Nephritic Characteristics Decreased renal function: Sudden onset of ARF can be seen Days-weeks: Rapidly progressive GN (RPGN) Oliguria: <400 ml urine output/day Extracellular fluid expansion- Edema Sunday, March 15, 2009
    16. 16. Nephritic syndrome Sunday, March 15, 2009
    17. 17. Focal Glomerulonephritis Sunday, March 15, 2009
    18. 18. Focal Glomerulonephritis Associated with inflammatory lesions in less than ½ glomeruli on LM Sunday, March 15, 2009
    19. 19. Focal Glomerulonephritis Associated with inflammatory lesions in less than ½ glomeruli on LM Usually mild proteinuria (<1.5 g/d) Sunday, March 15, 2009
    20. 20. Focal Glomerulonephritis Associated with inflammatory lesions in less than ½ glomeruli on LM Usually mild proteinuria (<1.5 g/d) Dysmorphic RBCs and occasional RBC cast Sunday, March 15, 2009
    21. 21. Focal Glomerulonephritis Associated with inflammatory lesions in less than ½ glomeruli on LM Usually mild proteinuria (<1.5 g/d) Dysmorphic RBCs and occasional RBC cast Can present with asymptomatic hematuria and proteinuria Sunday, March 15, 2009
    22. 22. Focal Glomerulonephritis Associated with inflammatory lesions in less than ½ glomeruli on LM Usually mild proteinuria (<1.5 g/d) Dysmorphic RBCs and occasional RBC cast Can present with asymptomatic hematuria and proteinuria Gross hematuria can be seen (IgA Nephropathy) Sunday, March 15, 2009
    23. 23. Focal Glomerulosclerosis Sunday, March 15, 2009
    24. 24. Focal Glomerulosclerosis Less than 15 yrs old Post Infectious GN, IgA Nephropathy, Thin Basement Membrane Disease, Hereditary Nephritis, HSP, MPGN Sunday, March 15, 2009
    25. 25. Focal Glomerulosclerosis Less than 15 yrs old Post Infectious GN, IgA Nephropathy, Thin Basement Membrane Disease, Hereditary Nephritis, HSP, MPGN 15-40 yrs old IgA Nephropathy, Thin Basement Membrane Disease, SLE, Hereditary Nephritis, MPGN Sunday, March 15, 2009
    26. 26. Focal Glomerulosclerosis Less than 15 yrs old Post Infectious GN, IgA Nephropathy, Thin Basement Membrane Disease, Hereditary Nephritis, HSP, MPGN 15-40 yrs old IgA Nephropathy, Thin Basement Membrane Disease, SLE, Hereditary Nephritis, MPGN >40 yr old IgA Nephropathy, MPGN Sunday, March 15, 2009
    27. 27. Diffuse Glomerulonephritis Sunday, March 15, 2009
    28. 28. Diffuse Glomerulonephritis Affects most or all glomeruli Sunday, March 15, 2009
    29. 29. Diffuse Glomerulonephritis Affects most or all glomeruli More active urine Sunday, March 15, 2009
    30. 30. Diffuse Glomerulonephritis Affects most or all glomeruli More active urine Greater degree of proteinuria up to nephrotic range can be seen Sunday, March 15, 2009
    31. 31. Diffuse Glomerulonephritis Affects most or all glomeruli More active urine Greater degree of proteinuria up to nephrotic range can be seen HTN more common Sunday, March 15, 2009
    32. 32. Diffuse Glomerulonephritis Affects most or all glomeruli More active urine Greater degree of proteinuria up to nephrotic range can be seen HTN more common Renal insufficiency Sunday, March 15, 2009
    33. 33. Diffuse Glomerulonephritis Affects most or all glomeruli More active urine Greater degree of proteinuria up to nephrotic range can be seen HTN more common Renal insufficiency Edema Sunday, March 15, 2009
    34. 34. Diffuse Glomerulonephritis Sunday, March 15, 2009
    35. 35. Diffuse Glomerulonephritis Less than 15 yr old Postinfectious GN, MPGN Sunday, March 15, 2009
    36. 36. Diffuse Glomerulonephritis Less than 15 yr old Postinfectious GN, MPGN 15-40 yr old Postinfectious GN, SLE, RPGN, MPGN Sunday, March 15, 2009
    37. 37. Diffuse Glomerulonephritis Less than 15 yr old Postinfectious GN, MPGN 15-40 yr old Postinfectious GN, SLE, RPGN, MPGN 40+ yr old RPGN, Vasculitis, Postinfectious Sunday, March 15, 2009
    38. 38. Hypocomplementemia Sunday, March 15, 2009
    39. 39. Hypocomplementemia Lab findings: Low levels of C3, C4, CH50 Sunday, March 15, 2009
    40. 40. Hypocomplementemia Lab findings: Low levels of C3, C4, CH50 Due to complement activation by immune deposits at a rate greater than that at which new complement proteins can be synthesized Sunday, March 15, 2009
    41. 41. Hypocomplementemia Lab findings: Low levels of C3, C4, CH50 Due to complement activation by immune deposits at a rate greater than that at which new complement proteins can be synthesized In addition to increased consumption, two other mechanisms can account for hypocomplementemia in glomerular disease: hereditary complement deficiency presence of circulating factors that promote complement activation Sunday, March 15, 2009
    42. 42. DDx of Glomerular Disease with Hypocomplementemia Sunday, March 15, 2009
    43. 43. DDx of Glomerular Disease with Hypocomplementemia Membranoproliferative GN Sunday, March 15, 2009
    44. 44. DDx of Glomerular Disease with Hypocomplementemia Membranoproliferative GN PSGN Sunday, March 15, 2009
    45. 45. DDx of Glomerular Disease with Hypocomplementemia Membranoproliferative GN PSGN SLE Sunday, March 15, 2009
    46. 46. DDx of Glomerular Disease with Hypocomplementemia Membranoproliferative GN PSGN SLE Cryoglobulinemia Sunday, March 15, 2009
    47. 47. DDx of Glomerular Disease with Hypocomplementemia Membranoproliferative GN PSGN SLE Cryoglobulinemia Also seen: Endocarditis Atheroembolic disease Sunday, March 15, 2009
    48. 48. Hereditary Nephritis Sunday, March 15, 2009
    49. 49. Hereditary Nephritis Sunday, March 15, 2009
    50. 50. Alport’s Syndrome Sunday, March 15, 2009
    51. 51. Alport’s Syndrome Classically X-linked (80-85%) Sunday, March 15, 2009
    52. 52. Alport’s Syndrome Classically X-linked (80-85%) Mutation on the COL4A5 gene responsible for the alpha 5 chain of type IV collagen in the GBM Sunday, March 15, 2009
    53. 53. Alport’s Syndrome Classically X-linked (80-85%) Mutation on the COL4A5 gene responsible for the alpha 5 chain of type IV collagen in the GBM Sensorineural hearing loss Sunday, March 15, 2009
    54. 54. Alport’s Syndrome Classically X-linked (80-85%) Mutation on the COL4A5 gene responsible for the alpha 5 chain of type IV collagen in the GBM Sensorineural hearing loss Ocular defects Anterior lenticonus, posterior polymorphous corneal dystrophy and retinal flecks Sunday, March 15, 2009
    55. 55. Alport’s Syndrome Pathology Sunday, March 15, 2009
    56. 56. Alport’s Syndrome Pathology Thickened, fraying, laminated GBM seen with EM Sunday, March 15, 2009
    57. 57. Alport’s Syndrome Clinical Course Sunday, March 15, 2009
    58. 58. Alport’s Syndrome Clinical Course Microscopic hematuria occurs from birth Sunday, March 15, 2009
    59. 59. Alport’s Syndrome Clinical Course Microscopic hematuria occurs from birth Gross hematuria after exercise or fever Sunday, March 15, 2009
    60. 60. Alport’s Syndrome Clinical Course Microscopic hematuria occurs from birth Gross hematuria after exercise or fever Urinary RBCs dysmorphic with RBC casts Sunday, March 15, 2009
    61. 61. Alport’s Syndrome Clinical Course Microscopic hematuria occurs from birth Gross hematuria after exercise or fever Urinary RBCs dysmorphic with RBC casts Proteinuria varies; occasional nephrotic range Sunday, March 15, 2009
    62. 62. Alport’s Syndrome Clinical Course Microscopic hematuria occurs from birth Gross hematuria after exercise or fever Urinary RBCs dysmorphic with RBC casts Proteinuria varies; occasional nephrotic range Hemizygous males inevitably progress to ESRD; females generally less severe Sunday, March 15, 2009
    63. 63. Alport’s Syndrome Clinical Course Microscopic hematuria occurs from birth Gross hematuria after exercise or fever Urinary RBCs dysmorphic with RBC casts Proteinuria varies; occasional nephrotic range Hemizygous males inevitably progress to ESRD; females generally less severe No specific therapy Sunday, March 15, 2009
    64. 64. Thin Basement Membrane Disease Sunday, March 15, 2009
    65. 65. Thin Basement Membrane Disease AKA: Benign Familial Hematuria Sunday, March 15, 2009
    66. 66. Thin Basement Membrane Disease AKA: Benign Familial Hematuria Relatively common with EM findings of thin GBM (<225nm… normal 300-400nm) Sunday, March 15, 2009
    67. 67. Thin Basement Membrane Disease AKA: Benign Familial Hematuria Relatively common with EM findings of thin GBM (<225nm… normal 300-400nm) Usually AD inheritance Sunday, March 15, 2009
    68. 68. Thin Basement Membrane Disease AKA: Benign Familial Hematuria Relatively common with EM findings of thin GBM (<225nm… normal 300-400nm) Usually AD inheritance Asymptomatic microscopic hematuria most often presentation Sunday, March 15, 2009
    69. 69. Thin Basement Membrane Disease AKA: Benign Familial Hematuria Relatively common with EM findings of thin GBM (<225nm… normal 300-400nm) Usually AD inheritance Asymptomatic microscopic hematuria most often presentation Rare episodes of gross hematuria possible Sunday, March 15, 2009
    70. 70. Thin Basement Membrane Disease AKA: Benign Familial Hematuria Relatively common with EM findings of thin GBM (<225nm… normal 300-400nm) Usually AD inheritance Asymptomatic microscopic hematuria most often presentation Rare episodes of gross hematuria possible Rarely associated with FSGS and progressive renal decline Sunday, March 15, 2009
    71. 71. Thin Basement Membrane Disease Sunday, March 15, 2009
    72. 72. IgA Nephropathy Sunday, March 15, 2009
    73. 73. IgA Nephropathy Sunday, March 15, 2009
    74. 74. IgA Nephropathy Most common cause of primary glomerular disease in the world Sunday, March 15, 2009
    75. 75. IgA Nephropathy Most common cause of primary glomerular disease in the world First described in 1968 by Berger in France (AKA Berger’s Disease) Sunday, March 15, 2009
    76. 76. IgA Nephropathy Most common cause of primary glomerular disease in the world First described in 1968 by Berger in France (AKA Berger’s Disease) Affects all ages most commonly children/young adults Sunday, March 15, 2009
    77. 77. IgA Nephropathy Most common cause of primary glomerular disease in the world First described in 1968 by Berger in France (AKA Berger’s Disease) Affects all ages most commonly children/young adults Male > Female 2:1 Sunday, March 15, 2009
    78. 78. IgA Nephropathy Most common cause of primary glomerular disease in the world First described in 1968 by Berger in France (AKA Berger’s Disease) Affects all ages most commonly children/young adults Male > Female 2:1 Most common in Asians and Caucasians Sunday, March 15, 2009
    79. 79. Presentation of IgA Nephropathy Sunday, March 15, 2009
    80. 80. Presentation of IgA Nephropathy 40-50% cases have URI with episode of gross hematuria as presenting Sx (synpharyngitic) Sunday, March 15, 2009
    81. 81. Presentation of IgA Nephropathy 40-50% cases have URI with episode of gross hematuria as presenting Sx (synpharyngitic) 30-40% mild proteinuria and microscopic hematuria Sunday, March 15, 2009
    82. 82. Presentation of IgA Nephropathy 40-50% cases have URI with episode of gross hematuria as presenting Sx (synpharyngitic) 30-40% mild proteinuria and microscopic hematuria HTN and peripheral edema rare Sunday, March 15, 2009
    83. 83. Presentation of IgA Nephropathy 40-50% cases have URI with episode of gross hematuria as presenting Sx (synpharyngitic) 30-40% mild proteinuria and microscopic hematuria HTN and peripheral edema rare Less commonly presents with NS Sunday, March 15, 2009
    84. 84. Presentation of IgA Nephropathy 40-50% cases have URI with episode of gross hematuria as presenting Sx (synpharyngitic) 30-40% mild proteinuria and microscopic hematuria HTN and peripheral edema rare Less commonly presents with NS Cross over presentation with Henoch Schonlein purpura(HSP), more common in children Sunday, March 15, 2009
    85. 85. Diagnosis of IgA Nephropathy Sunday, March 15, 2009
    86. 86. Diagnosis of IgA Nephropathy Based on clinical and lab data but only can be confirmed by renal biopsy Sunday, March 15, 2009
    87. 87. Diagnosis of IgA Nephropathy Based on clinical and lab data but only can be confirmed by renal biopsy Often since benign course a renal biopsy not done. Usually only if proteinuria >1g/d, HTN, edema, decreased GFR Sunday, March 15, 2009
    88. 88. Diagnosis of IgA Nephropathy Based on clinical and lab data but only can be confirmed by renal biopsy Often since benign course a renal biopsy not done. Usually only if proteinuria >1g/d, HTN, edema, decreased GFR Skin Bx looking for IgA1 deposition in dermal capillaries has been evaluated but unreliable Sunday, March 15, 2009
    89. 89. Diagnosis of IgA Nephropathy Based on clinical and lab data but only can be confirmed by renal biopsy Often since benign course a renal biopsy not done. Usually only if proteinuria >1g/d, HTN, edema, decreased GFR Skin Bx looking for IgA1 deposition in dermal capillaries has been evaluated but unreliable Plasma IgA1 elevated in 30-50% Sunday, March 15, 2009
    90. 90. Diagnosis of IgA Nephropathy Based on clinical and lab data but only can be confirmed by renal biopsy Often since benign course a renal biopsy not done. Usually only if proteinuria >1g/d, HTN, edema, decreased GFR Skin Bx looking for IgA1 deposition in dermal capillaries has been evaluated but unreliable Plasma IgA1 elevated in 30-50% DDx: Thin basement membrane disease and hereditary nephritis Sunday, March 15, 2009
    91. 91. Pathology of IgA Nephropathy Sunday, March 15, 2009
    92. 92. Pathology of IgA Nephropathy LM: Mesangial expansion increased matrix and cellularity Sunday, March 15, 2009
    93. 93. Pathology of IgA Nephropathy Sunday, March 15, 2009
    94. 94. Pathology of IgA Nephropathy IF: IgA mesangial staining Sunday, March 15, 2009
    95. 95. Pathology of IgA Nephropathy Sunday, March 15, 2009
    96. 96. Pathology of IgA Nephropathy EM: Deposits in mesangial and paramesangial areas Sunday, March 15, 2009
    97. 97. Clinical Course of IgA Nephropathy Sunday, March 15, 2009
    98. 98. Clinical Course of IgA Nephropathy Kidney function progressively worsens in ~40-60%... 50% will reach ESRD after 20 yrs of clinically apparent disease. Sunday, March 15, 2009
    99. 99. Clinical Course of IgA Nephropathy Kidney function progressively worsens in ~40-60%... 50% will reach ESRD after 20 yrs of clinically apparent disease. <10% develop nephrotic syndrome Sunday, March 15, 2009
    100. 100. Clinical Course of IgA Nephropathy Kidney function progressively worsens in ~40-60%... 50% will reach ESRD after 20 yrs of clinically apparent disease. <10% develop nephrotic syndrome 1/3 patients benign course with chronic microscopic hematuria, normal serum creatinine and <1g/day proteinuria Sunday, March 15, 2009
    101. 101. Clinical Course of IgA Nephropathy Kidney function progressively worsens in ~40-60%... 50% will reach ESRD after 20 yrs of clinically apparent disease. <10% develop nephrotic syndrome 1/3 patients benign course with chronic microscopic hematuria, normal serum creatinine and <1g/day proteinuria <10% RPGN Sunday, March 15, 2009
    102. 102. Risk Factors for worse prognosis with IgA Nephropathy Sunday, March 15, 2009
    103. 103. Risk Factors for worse prognosis with IgA Nephropathy HTN Sunday, March 15, 2009
    104. 104. Risk Factors for worse prognosis with IgA Nephropathy HTN Absence of history of macroscopic hematuria Sunday, March 15, 2009
    105. 105. Risk Factors for worse prognosis with IgA Nephropathy HTN Absence of history of macroscopic hematuria Persistent microscopic hematuria Sunday, March 15, 2009
    106. 106. Risk Factors for worse prognosis with IgA Nephropathy HTN Absence of history of macroscopic hematuria Persistent microscopic hematuria Older age at onset Sunday, March 15, 2009
    107. 107. Risk Factors for worse prognosis with IgA Nephropathy HTN Absence of history of macroscopic hematuria Persistent microscopic hematuria Older age at onset Renal dysfunction at onset Sunday, March 15, 2009
    108. 108. Risk Factors for worse prognosis with IgA Nephropathy HTN Absence of history of macroscopic hematuria Persistent microscopic hematuria Older age at onset Renal dysfunction at onset Proteinuria >0.5 g/day Sunday, March 15, 2009
    109. 109. Treatment of IgA Nephropathy Sunday, March 15, 2009
    110. 110. Treatment of IgA Nephropathy Non-immunosupressive ACEi/ ARB Statin based cholesterol control FISH OILS (rich in omega 3FA) Sunday, March 15, 2009
    111. 111. Treatment of IgA Nephropathy Non-immunosupressive ACEi/ ARB Statin based cholesterol control FISH OILS (rich in omega 3FA) Immunosupression Corticosteroids Corticosteroids + cytoxan Others: Cyclosporin, CellCept, Imuran Sunday, March 15, 2009
    112. 112. Treatment of IgA Nephropathy Non-immunosupressive ACEi/ ARB Statin based cholesterol control FISH OILS (rich in omega 3FA) Immunosupression Corticosteroids Corticosteroids + cytoxan Others: Cyclosporin, CellCept, Imuran Other: IVIg, ASA, dapsone, danazol, gluten free diet, low anigen diet, tonsillectomy Sunday, March 15, 2009
    113. 113. Postinfectious Glomerulonephritis Sunday, March 15, 2009
    114. 114. Postinfectious Glomerulonephritis Sunday, March 15, 2009
    115. 115. Postinfectious Glomerulonephritis Infection is a common cause of GN and can be seen with a variety of bacterial and viral infections. Sunday, March 15, 2009
    116. 116. Postinfectious Glomerulonephritis Infection is a common cause of GN and can be seen with a variety of bacterial and viral infections. Poststreptococcal GN is most common and classic form Induced by Nephritic strain of beta-hemolytic Group A Strep Seen after acute pharyngitis or skin infection Sunday, March 15, 2009
    117. 117. Postinfectious Glomerulonephritis Infection is a common cause of GN and can be seen with a variety of bacterial and viral infections. Poststreptococcal GN is most common and classic form Induced by Nephritic strain of beta-hemolytic Group A Strep Seen after acute pharyngitis or skin infection Also can be seen with: Hepatits B, Hepatitis C, malaria, syphilis, infectious endocarditis, HIV, chronic visceral abscess Sunday, March 15, 2009
    118. 118. Poststreptococcal GN Sunday, March 15, 2009
    119. 119. Poststreptococcal GN The clinical presentation can vary from asymptomatic, microscopic hematuria to the full-blown acute nephritic syndrome, characterized by red to brown urine, proteinuria (which can reach the nephrotic range), edema, hypertension, and acute renal failure Sunday, March 15, 2009
    120. 120. Poststreptococcal GN The clinical presentation can vary from asymptomatic, microscopic hematuria to the full-blown acute nephritic syndrome, characterized by red to brown urine, proteinuria (which can reach the nephrotic range), edema, hypertension, and acute renal failure Presentation may be similar to IgA Nephropathy Sunday, March 15, 2009
    121. 121. Distinguishing PSGN vs IgAN Sunday, March 15, 2009
    122. 122. Distinguishing PSGN vs IgAN Latent period from infection until hematuria PSGN: 10 days- pharyngitis; 21 days- impetigo IgAN: <5 days Sunday, March 15, 2009
    123. 123. Distinguishing PSGN vs IgAN Latent period from infection until hematuria PSGN: 10 days- pharyngitis; 21 days- impetigo IgAN: <5 days Recurrent episodes of gross hematuria: common in IgA nephropathy but rare in poststreptococcal glomerulonephritis Sunday, March 15, 2009
    124. 124. Distinguishing PSGN vs IgAN Latent period from infection until hematuria PSGN: 10 days- pharyngitis; 21 days- impetigo IgAN: <5 days Recurrent episodes of gross hematuria: common in IgA nephropathy but rare in poststreptococcal glomerulonephritis Postive serology with PSGN ASLO (antistreptolysin O titers), anti-DNAse B, anti-hyaluronidase Sunday, March 15, 2009
    125. 125. Distinguishing PSGN vs IgAN Latent period from infection until hematuria PSGN: 10 days- pharyngitis; 21 days- impetigo IgAN: <5 days Recurrent episodes of gross hematuria: common in IgA nephropathy but rare in poststreptococcal glomerulonephritis Postive serology with PSGN ASLO (antistreptolysin O titers), anti-DNAse B, anti-hyaluronidase Hypocomplementemia with PSGN Sunday, March 15, 2009
    126. 126. Postinfectious GN Pathology Sunday, March 15, 2009
    127. 127. Postinfectious GN Pathology LM: Diffuse hypercellularity involving all glomeruli. Sunday, March 15, 2009
    128. 128. Postinfectious GN Pathology LM: Diffuse hypercellularity involving all glomeruli. Glomerular tufts enlarged Sunday, March 15, 2009
    129. 129. Postinfectious GN Pathology Sunday, March 15, 2009
    130. 130. Postinfectious GN Pathology EM: characteristic subepithelial humps Sunday, March 15, 2009
    131. 131. Poststreptococcal GN Sunday, March 15, 2009
    132. 132. Poststreptococcal GN Usually self limited Sunday, March 15, 2009
    133. 133. Poststreptococcal GN Usually self limited Spontaneous recovery is the rule Sunday, March 15, 2009
    134. 134. Poststreptococcal GN Usually self limited Spontaneous recovery is the rule Rarely patients can develop HTN, recurrent proteinuria and renal insufficiency Sunday, March 15, 2009
    135. 135. Poststreptococcal GN Usually self limited Spontaneous recovery is the rule Rarely patients can develop HTN, recurrent proteinuria and renal insufficiency Second episode is very rare Sunday, March 15, 2009
    136. 136. Other Infectious Causes of GN Sunday, March 15, 2009
    137. 137. Hepatitis B related GN Sunday, March 15, 2009
    138. 138. Hepatitis B related GN Mainly Membranous Nephropathy Sunday, March 15, 2009
    139. 139. Hepatitis B related GN Mainly Membranous Nephropathy Second most common is MPGN Sunday, March 15, 2009
    140. 140. Hepatitis B related GN Mainly Membranous Nephropathy Second most common is MPGN Pts usually carry HBsAg, HBcAg and HBeAg Sunday, March 15, 2009
    141. 141. Hepatitis B related GN Mainly Membranous Nephropathy Second most common is MPGN Pts usually carry HBsAg, HBcAg and HBeAg Steroids are contraindicated Sunday, March 15, 2009
    142. 142. Hepatitis B related GN Mainly Membranous Nephropathy Second most common is MPGN Pts usually carry HBsAg, HBcAg and HBeAg Steroids are contraindicated Antiviral treatment is indicated Sunday, March 15, 2009
    143. 143. Hepatitis C related GN Sunday, March 15, 2009
    144. 144. Hepatitis C related GN Typically related to cryoglobulinemia Arthlagias, peripheral neuropathy and purpura Sunday, March 15, 2009
    145. 145. Hepatitis C related GN Typically related to cryoglobulinemia Arthlagias, peripheral neuropathy and purpura Hypocomplementemia Sunday, March 15, 2009
    146. 146. Hepatitis C related GN Typically related to cryoglobulinemia Arthlagias, peripheral neuropathy and purpura Hypocomplementemia MPGN most commonly Sunday, March 15, 2009
    147. 147. Hepatitis C related GN Typically related to cryoglobulinemia Arthlagias, peripheral neuropathy and purpura Hypocomplementemia MPGN most commonly Treatment of underlying HepC with interferon and ribavirin is typical, other immunosuppressive treatment is controversial Sunday, March 15, 2009
    148. 148. HIV associated nephropathy (HIVAN) Sunday, March 15, 2009
    149. 149. HIV associated nephropathy (HIVAN) Usually presents as a collapsing form of FSGS with severe tubulointerstitial injury Sunday, March 15, 2009
    150. 150. HIV associated nephropathy (HIVAN) Usually presents as a collapsing form of FSGS with severe tubulointerstitial injury Clinical characteristics Severe nephrosis with massive proteinuria Large echogenic kidneys on sonogram Usually normotensive Progressive renal decline to ESRD (1-4 months) Sunday, March 15, 2009
    151. 151. HIV associated nephropathy (HIVAN) Usually presents as a collapsing form of FSGS with severe tubulointerstitial injury Clinical characteristics Severe nephrosis with massive proteinuria Large echogenic kidneys on sonogram Usually normotensive Progressive renal decline to ESRD (1-4 months) Treatment HAART and ACEi Sunday, March 15, 2009
    152. 152. HIVAN Pathology Sunday, March 15, 2009
    153. 153. HIVAN Pathology LM: Collapsing severe FSGS Sunday, March 15, 2009
    154. 154. HIVAN Pathology LM: Collapsing severe FSGS EM: TRI (tubuloreticular inclusion bodies) Sunday, March 15, 2009
    155. 155. Lupus Nephritis Sunday, March 15, 2009
    156. 156. Systemic Lupus Erythematosus (SLE) Sunday, March 15, 2009
    157. 157. Systemic Lupus Erythematosus (SLE) SLE predominantly effects women 10x> men Peak incidence: age 15-40 AA 3 fold higher incidence & develop younger age More often +anti-Sm and RNP Ab Increased severity and mortality compared with whites Sunday, March 15, 2009
    158. 158. Systemic Lupus Erythematosus (SLE) SLE predominantly effects women 10x> men Peak incidence: age 15-40 AA 3 fold higher incidence & develop younger age More often +anti-Sm and RNP Ab Increased severity and mortality compared with whites Children and males develop nephritis more frequently Sunday, March 15, 2009
    159. 159. Systemic Lupus Erythematosus (SLE) SLE predominantly effects women 10x> men Peak incidence: age 15-40 AA 3 fold higher incidence & develop younger age More often +anti-Sm and RNP Ab Increased severity and mortality compared with whites Children and males develop nephritis more frequently Nephritis usually associated with high titer anti- dsDNA, anemia, family history of SLE, and hypocomplementia Sunday, March 15, 2009
    160. 160. WHO Classification of Lupus Nephritis Sunday, March 15, 2009
    161. 161. WHO Classification of Lupus Nephritis Class I: Minimal mesangial LN Sunday, March 15, 2009
    162. 162. WHO Classification of Lupus Nephritis Class I: Minimal mesangial LN Class II: Mesangial proliferative LN Sunday, March 15, 2009
    163. 163. WHO Classification of Lupus Nephritis Class I: Minimal mesangial LN Class II: Mesangial proliferative LN Class III: Focal LN (<50% glomeruli involved) Sunday, March 15, 2009
    164. 164. WHO Classification of Lupus Nephritis Class I: Minimal mesangial LN Class II: Mesangial proliferative LN Class III: Focal LN (<50% glomeruli involved) Class IV: Diffuse LN Sunday, March 15, 2009
    165. 165. WHO Classification of Lupus Nephritis Class I: Minimal mesangial LN Class II: Mesangial proliferative LN Class III: Focal LN (<50% glomeruli involved) Class IV: Diffuse LN Class V: Membranous LN Sunday, March 15, 2009
    166. 166. WHO Classification of Lupus Nephritis Class I: Minimal mesangial LN Class II: Mesangial proliferative LN Class III: Focal LN (<50% glomeruli involved) Class IV: Diffuse LN Class V: Membranous LN Class VI: Advanced sclerotic LN (ESRD) Sunday, March 15, 2009
    167. 167. Lupus Nephritis Pathology Sunday, March 15, 2009
    168. 168. Lupus Nephritis Pathology LM: Mesangial proliferation Sunday, March 15, 2009
    169. 169. Lupus Nephritis Pathology LM: Mesangial proliferation Sunday, March 15, 2009
    170. 170. Lupus Nephritis Pathology LM: Mesangial proliferation Diffuse proliferative nephritis with “wire loop” (thickened GBM with immune deposits) Sunday, March 15, 2009
    171. 171. Lupus Nephritis Pathology Sunday, March 15, 2009
    172. 172. Lupus Nephritis Pathology IF: massive “lumpy bumpy” IgG distribution Sunday, March 15, 2009
    173. 173. Lupus Nephritis Pathology IF: massive “lumpy bumpy” IgG distribution EM: classic subENDOthelial deposits (Class III and IV) and TRI Sunday, March 15, 2009
    174. 174. Treatment of Lupus Nephritis Sunday, March 15, 2009
    175. 175. Treatment of Lupus Nephritis Class I and II LN have excellent renal prognosis and no specific therapy indicated Sunday, March 15, 2009
    176. 176. Treatment of Lupus Nephritis Class I and II LN have excellent renal prognosis and no specific therapy indicated Proliferative LN (Class IV) : Poorest prognosis Treatment of choice: Pulse Solumedrol followed by IV pulse Cytoxan and po Prednisone Other agents: Imuran, CellCept, Cyclosporin Sunday, March 15, 2009
    177. 177. Treatment of Lupus Nephritis Class I and II LN have excellent renal prognosis and no specific therapy indicated Proliferative LN (Class IV) : Poorest prognosis Treatment of choice: Pulse Solumedrol followed by IV pulse Cytoxan and po Prednisone Other agents: Imuran, CellCept, Cyclosporin Membranous LN (Class V) No clear consensus; attempt immunosuppresion as per idiopathic MN Sunday, March 15, 2009
    178. 178. Crescentic Glomerulonephritis Sunday, March 15, 2009
    179. 179. Rapidly Progressive Glomerulonephritis (RPGN) Sunday, March 15, 2009
    180. 180. Rapidly Progressive Glomerulonephritis (RPGN) Severe form of GN Sunday, March 15, 2009
    181. 181. Rapidly Progressive Glomerulonephritis (RPGN) Severe form of GN Progression to ESRD in weeks to months Sunday, March 15, 2009
    182. 182. Rapidly Progressive Glomerulonephritis (RPGN) Severe form of GN Progression to ESRD in weeks to months Characterized by crescentic lesions Severity of disease corresponds to degree of crescent Crescent formation caused by severe injury to glomerular capillary wall Crescents may be more acute (cellular) or older (fibrotic)….. or mixed (fibrocellular) Sunday, March 15, 2009
    183. 183. Crescentic Glomerulonephritis Sunday, March 15, 2009
    184. 184. Sunday, March 15, 2009
    185. 185. Sunday, March 15, 2009
    186. 186. Types of Crescentic Glomerulonephritis Sunday, March 15, 2009
    187. 187. Types of Crescentic Glomerulonephritis Anti-GBM Goodpasture’s syndrome; Anti-GBM Disease Sunday, March 15, 2009
    188. 188. Types of Crescentic Glomerulonephritis Anti-GBM Goodpasture’s syndrome; Anti-GBM Disease Immune mediated IgA N, postinfectious GN, SLE, Cryoglobulinemia Sunday, March 15, 2009
    189. 189. Types of Crescentic Glomerulonephritis Anti-GBM Goodpasture’s syndrome; Anti-GBM Disease Immune mediated IgA N, postinfectious GN, SLE, Cryoglobulinemia Pauci-immune RPGN with little to no immune deposits on IF/EM Majority: ‘ANCA associated vasculitis’ ANCA= Anti-Neutrophilic Cytoplasmic Antibody Wegner’s granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome Sunday, March 15, 2009
    190. 190. Clinical presentation of RPGN Sunday, March 15, 2009
    191. 191. Clinical presentation of RPGN Most commonly insidious: fatigue and /or edema Sunday, March 15, 2009
    192. 192. Clinical presentation of RPGN Most commonly insidious: fatigue and /or edema Renal insufficiency seen at presentation Sunday, March 15, 2009
    193. 193. Clinical presentation of RPGN Most commonly insidious: fatigue and /or edema Renal insufficiency seen at presentation Urinalysis: RBCs, RBC casts, varying proteinuria (NS unusual) Sunday, March 15, 2009
    194. 194. Clinical presentation of RPGN Most commonly insidious: fatigue and /or edema Renal insufficiency seen at presentation Urinalysis: RBCs, RBC casts, varying proteinuria (NS unusual) Systemic complaints Pauci-immune ANCA positive or negative AntiGBM- Goodpasture’s Immune complex disease- i.e.. SLE Sunday, March 15, 2009
    195. 195. Goodpasture’s Syndrome Sunday, March 15, 2009
    196. 196. Goodpasture’s Syndrome Sunday, March 15, 2009
    197. 197. Goodpasture’s Syndrome First used term in 1957 after a report detailing 9 pts with pulmonary renal syndrome first described by Goodpasture in 1919. Sunday, March 15, 2009
    198. 198. Goodpasture’s Syndrome First used term in 1957 after a report detailing 9 pts with pulmonary renal syndrome first described by Goodpasture in 1919. Autoimmune disease to the antigen found on the alpha3 chain of type IV collagen of the GBM Sunday, March 15, 2009
    199. 199. Goodpasture’s Syndrome First used term in 1957 after a report detailing 9 pts with pulmonary renal syndrome first described by Goodpasture in 1919. Autoimmune disease to the antigen found on the alpha3 chain of type IV collagen of the GBM The syndrome describes combination of: RPGN Pulmonary hemorrhage Anti-GBM Ab Sunday, March 15, 2009
    200. 200. Goodpasture’s Syndrome First used term in 1957 after a report detailing 9 pts with pulmonary renal syndrome first described by Goodpasture in 1919. Autoimmune disease to the antigen found on the alpha3 chain of type IV collagen of the GBM The syndrome describes combination of: RPGN Pulmonary hemorrhage Anti-GBM Ab anti-GBM disease refers to any pt with the Ab regardless of clinical features Sunday, March 15, 2009
    201. 201. Goodpasture’s Syndrome Sunday, March 15, 2009
    202. 202. Goodpasture’s Syndrome Bimodal age distribution: 3rd and 6th decades Sunday, March 15, 2009
    203. 203. Goodpasture’s Syndrome Bimodal age distribution: 3rd and 6th decades Males > Female; predominantly white race Sunday, March 15, 2009
    204. 204. Goodpasture’s Syndrome Bimodal age distribution: 3rd and 6th decades Males > Female; predominantly white race Most have RPGN and lung hemorrhage Sunday, March 15, 2009
    205. 205. Goodpasture’s Syndrome Bimodal age distribution: 3rd and 6th decades Males > Female; predominantly white race Most have RPGN and lung hemorrhage 1/3 isolated GN Sunday, March 15, 2009
    206. 206. Goodpasture’s Syndrome Bimodal age distribution: 3rd and 6th decades Males > Female; predominantly white race Most have RPGN and lung hemorrhage 1/3 isolated GN Rarely isolated lung hemorrhage without renal disease Sunday, March 15, 2009
    207. 207. Goodpasture’s Syndrome Bimodal age distribution: 3rd and 6th decades Males > Female; predominantly white race Most have RPGN and lung hemorrhage 1/3 isolated GN Rarely isolated lung hemorrhage without renal disease Malaise, fatigue, weight loss are the most common systemic features Sunday, March 15, 2009
    208. 208. Goodpasture’s Syndrome Sunday, March 15, 2009
    209. 209. Goodpasture’s Syndrome Pulmonary hemorrhage occurs in 2/3rds; more common in younger men May proceed renal disease Triggered by smoking, toxins, sepsis, fluid overload Sunday, March 15, 2009
    210. 210. Goodpasture’s Syndrome Pulmonary hemorrhage occurs in 2/3rds; more common in younger men May proceed renal disease Triggered by smoking, toxins, sepsis, fluid overload Renal Disease Most commonly acute renal failure with RPGN Urine micro: RBC, RBC casts, mild-mod proteinuria (nephrotic syndrome rare) HTN and oliguria are late features Rarely isolated hematuria or mild renal dysfunction Sunday, March 15, 2009
    211. 211. Goodpasture’s Syndrome Pathology Sunday, March 15, 2009
    212. 212. Goodpasture’s Syndrome Pathology LM: diffuse crescentic glomerulonephritis Sunday, March 15, 2009
    213. 213. Goodpasture’s Syndrome Pathology LM: diffuse crescentic glomerulonephritis Prominent interstitial cellular infiltrate Sunday, March 15, 2009
    214. 214. Goodpasture’s Syndrome Pathology Sunday, March 15, 2009
    215. 215. Goodpasture’s Syndrome Pathology IF: Classic linear IgG and C3 along the GBM Sunday, March 15, 2009
    216. 216. Goodpasture’s Syndrome Treatment Sunday, March 15, 2009
    217. 217. Goodpasture’s Syndrome Treatment Untreated: Fatal Sunday, March 15, 2009
    218. 218. Goodpasture’s Syndrome Treatment Untreated: Fatal Combination therapy: Plasma exchange, Cytoxan and Corticosteroids Plasmapharesis: plasma exchange is used to remove circulating anti-GBM Ab Cytoxan: prevents further Ab synthesis Steroids: Pulse IV Solumedrol followed by Prednisone Sunday, March 15, 2009
    219. 219. Goodpasture’s Syndrome Treatment Untreated: Fatal Combination therapy: Plasma exchange, Cytoxan and Corticosteroids Plasmapharesis: plasma exchange is used to remove circulating anti-GBM Ab Cytoxan: prevents further Ab synthesis Steroids: Pulse IV Solumedrol followed by Prednisone Prognosis depends on the severity of disease upon presentation. If dialysis is need up to presentation, renal recovery is rare Sunday, March 15, 2009
    220. 220. ANCA-associated Vasculitis Sunday, March 15, 2009
    221. 221. Vasculitis Sunday, March 15, 2009
    222. 222. ANCA Sunday, March 15, 2009
    223. 223. Wegner’s Granulomatosis Sunday, March 15, 2009
    224. 224. Wegner’s Granulomatosis Systemic vasculitis medium and small size arteries Sunday, March 15, 2009
    225. 225. Wegner’s Granulomatosis Systemic vasculitis medium and small size arteries 90 % ANCA positive 70-80% c-ANCA + Anti-Proteinase 3 (PR3) 10-15% p-ANCA + Anti-Myeloperoxidase (MPO) Sunday, March 15, 2009
    226. 226. Wegner’s Granulomatosis Systemic vasculitis medium and small size arteries 90 % ANCA positive 70-80% c-ANCA + Anti-Proteinase 3 (PR3) 10-15% p-ANCA + Anti-Myeloperoxidase (MPO) Classic WG: involvement of the upper and lower respiratory tract and kidneys Sunday, March 15, 2009
    227. 227. Wegner’s Granulomatosis Manifestations Sunday, March 15, 2009
    228. 228. Wegner’s Granulomatosis Manifestations In addition to renal and lung….: Joints: myalgias, arthalgias, arthritis Eyes: conjunctivitis, corneal ulcers Skin: palpable purpura Nervous system: mononeuritis multiplex Less commonly: GI, heart, GU tract, thyroid High incidence of DVT Sunday, March 15, 2009
    229. 229. Wegner’s Granulomatosis Manifestations In addition to renal and lung….: Joints: myalgias, arthalgias, arthritis Eyes: conjunctivitis, corneal ulcers Skin: palpable purpura Nervous system: mononeuritis multiplex Less commonly: GI, heart, GU tract, thyroid High incidence of DVT “ELKS” = Eyes, Lungs, Kidneys, Sinsus involvement Sunday, March 15, 2009
    230. 230. Wegner’s Granulomatosis Presentation Sunday, March 15, 2009
    231. 231. Wegner’s Granulomatosis Presentation The most common presenting symptoms include: persistent rhinorrhea, purulent/bloody nasal discharge, oral and/or nasal ulcers, polyarthralgias, myalgias, or sinus pain. Sunday, March 15, 2009
    232. 232. Wegner’s Granulomatosis Presentation The most common presenting symptoms include: persistent rhinorrhea, purulent/bloody nasal discharge, oral and/or nasal ulcers, polyarthralgias, myalgias, or sinus pain. Less common symptoms of upper airway involvement: hoarseness, stridor, earache, both conductive and sensorineural hearing loss, or otorrhea Sunday, March 15, 2009
    233. 233. Wegner’s Granulomatosis Sunday, March 15, 2009
    234. 234. Wegner’s Granulomatosis Pulmonary Imaging findings: Nodules (which may cavitate) Alveolar opacities Pleural opacities Diffuse hazy opacities (which may reflect alveolar hemorrhage) Sunday, March 15, 2009
    235. 235. Wegner’s Granulomatosis Diagnosis Sunday, March 15, 2009
    236. 236. Wegner’s Granulomatosis Diagnosis The four clinical criteria are:  Nasal or oral inflammation (painful or 1. painless oral ulcers or purulent or bloody nasal discharge) Abnormal chest radiograph showing nodules, 2. fixed infiltrates, or cavities Abnormal urinary sediment (microscopic 3. hematuria with or without red cell casts) Granulomatous inflammation on biopsy of an 4. artery or perivascular area Sunday, March 15, 2009
    237. 237. Wegner’s Granulomatosis Diagnosis Cont. Sunday, March 15, 2009
    238. 238. Wegner’s Granulomatosis Diagnosis Cont. Labs: leukocytosis, thrombocytosis, anemia and increased ESR, normocomplementemia Sunday, March 15, 2009
    239. 239. Wegner’s Granulomatosis Diagnosis Cont. Labs: leukocytosis, thrombocytosis, anemia and increased ESR, normocomplementemia ANCA Most commonly C-ANCA positive (Anti-Proteinase 3) Sunday, March 15, 2009
    240. 240. Wegner’s Granulomatosis Diagnosis Cont. Labs: leukocytosis, thrombocytosis, anemia and increased ESR, normocomplementemia ANCA Most commonly C-ANCA positive (Anti-Proteinase 3) Biopsy is necessary for confirmation of diagnosis Skin, pulmonary Bx.. or… Renal biopsy: segmental necrotizing GN Granulomas Sunday, March 15, 2009
    241. 241. Wegner’s Granulomatosis Treatment Sunday, March 15, 2009
    242. 242. Wegner’s Granulomatosis Treatment Treatment of choice: Cytoxan (1.5-2 mg/kg) and Prednisone (1mg/kg) Daily until remission (usually atleast 3-6 months) Sunday, March 15, 2009
    243. 243. Wegner’s Granulomatosis Treatment Treatment of choice: Cytoxan (1.5-2 mg/kg) and Prednisone (1mg/kg) Daily until remission (usually atleast 3-6 months) Alternative Tx choices: IV monthly Cytoxan Methotrexate: best for non-renal WG Sunday, March 15, 2009
    244. 244. Wegner’s Granulomatosis Treatment Treatment of choice: Cytoxan (1.5-2 mg/kg) and Prednisone (1mg/kg) Daily until remission (usually atleast 3-6 months) Alternative Tx choices: IV monthly Cytoxan Methotrexate: best for non-renal WG Maintenance therapy once remission reached: Switch cytoxan to Imuran or MTX Taper off Prednisone Sunday, March 15, 2009
    245. 245. Wegner’s Granulomatosis Prognosis Sunday, March 15, 2009
    246. 246. Wegner’s Granulomatosis Prognosis Natural History: Untreated fatal: 90% mortality 2 years Sunday, March 15, 2009
    247. 247. Wegner’s Granulomatosis Prognosis Natural History: Untreated fatal: 90% mortality 2 years Otherwise prognosis is a consequence of: Irreversible organ damage prior to Tx SE of immunosuppression Glucocorticoid toxicity Superimposed infections Secondary malignancies Sunday, March 15, 2009
    248. 248. Wegner’s Granulomatosis Prognosis Natural History: Untreated fatal: 90% mortality 2 years Otherwise prognosis is a consequence of: Irreversible organ damage prior to Tx SE of immunosuppression Glucocorticoid toxicity Superimposed infections Secondary malignancies Renal outcome depends upon severity of disease at presentation Sunday, March 15, 2009
    249. 249. Microscopic Polyangiitis Sunday, March 15, 2009
    250. 250. Microscopic Polyangiitis Overlaps with Wegner’s granulomatosis Sunday, March 15, 2009
    251. 251. Microscopic Polyangiitis Overlaps with Wegner’s granulomatosis More often “renal-limited” with findings indistinguishable from WG Sunday, March 15, 2009
    252. 252. Microscopic Polyangiitis Overlaps with Wegner’s granulomatosis More often “renal-limited” with findings indistinguishable from WG Absence of granulomatous inflammation Sunday, March 15, 2009
    253. 253. Microscopic Polyangiitis Overlaps with Wegner’s granulomatosis More often “renal-limited” with findings indistinguishable from WG Absence of granulomatous inflammation 70% ANCA positive but more often p-ANCA (anti MPO) Sunday, March 15, 2009
    254. 254. Microscopic Polyangiitis Overlaps with Wegner’s granulomatosis More often “renal-limited” with findings indistinguishable from WG Absence of granulomatous inflammation 70% ANCA positive but more often p-ANCA (anti MPO) Treatment same as for WG Sunday, March 15, 2009
    255. 255. Microscopic Polyangiitis Overlaps with Wegner’s granulomatosis More often “renal-limited” with findings indistinguishable from WG Absence of granulomatous inflammation 70% ANCA positive but more often p-ANCA (anti MPO) Treatment same as for WG More often there are relapses with WG Sunday, March 15, 2009
    256. 256. Churg-Strauss Syndrome Sunday, March 15, 2009
    257. 257. Churg-Strauss Syndrome AKA: Allergic granulomatosis and angiitis Sunday, March 15, 2009
    258. 258. Churg-Strauss Syndrome AKA: Allergic granulomatosis and angiitis ANCA + ~50%, most commonly pANCA + Sunday, March 15, 2009
    259. 259. Churg-Strauss Syndrome AKA: Allergic granulomatosis and angiitis ANCA + ~50%, most commonly pANCA + Multisystemic disorder Allergic rhinitis Asthma- cardinal feature 95% occurrence Peripheral eosinophilia Sunday, March 15, 2009
    260. 260. Churg-Strauss Syndrome AKA: Allergic granulomatosis and angiitis ANCA + ~50%, most commonly pANCA + Multisystemic disorder Allergic rhinitis Asthma- cardinal feature 95% occurrence Peripheral eosinophilia All organ systems can be involved Most commonly: lungs and skin Also: Cardiac, Kidneys, GI and CNS Sunday, March 15, 2009
    261. 261. Churg-Strauss Syndrome Clinical Features Sunday, March 15, 2009
    262. 262. Churg-Strauss Syndrome Clinical Features Prodromal phase 2nd-3rd decade of life; asthma and allergic rhinitis Sunday, March 15, 2009
    263. 263. Churg-Strauss Syndrome Clinical Features Prodromal phase 2nd-3rd decade of life; asthma and allergic rhinitis Eosinophilic phase Peripheral blood eosinophilia and eosinophilic infiltrates of organs (most commonly lung) Sunday, March 15, 2009
    264. 264. Churg-Strauss Syndrome Clinical Features Prodromal phase 2nd-3rd decade of life; asthma and allergic rhinitis Eosinophilic phase Peripheral blood eosinophilia and eosinophilic infiltrates of organs (most commonly lung) Vasculitic phase 3rd-4th decade of life; small-medium size systemic vasculitis Sunday, March 15, 2009
    265. 265. Churg-Strauss Syndrome Renal Manifestations Sunday, March 15, 2009
    266. 266. Churg-Strauss Syndrome Renal Manifestations Similar, but less severe than Wegner’s Granulomatosis typically Sunday, March 15, 2009
    267. 267. Churg-Strauss Syndrome Renal Manifestations Similar, but less severe than Wegner’s Granulomatosis typically Best renal prognosis of all the causes of RPGN with <10% progression to ESRD Sunday, March 15, 2009
    268. 268. Churg-Strauss Syndrome Renal Manifestations Similar, but less severe than Wegner’s Granulomatosis typically Best renal prognosis of all the causes of RPGN with <10% progression to ESRD Systemic HTN common Sunday, March 15, 2009
    269. 269. Churg-Strauss Syndrome Renal Manifestations Similar, but less severe than Wegner’s Granulomatosis typically Best renal prognosis of all the causes of RPGN with <10% progression to ESRD Systemic HTN common Renal infarction can be seen Sunday, March 15, 2009
    270. 270. Brief Urine Microscopy Review Sunday, March 15, 2009
    271. 271. Urine Microscopy Review Casts Urinary casts are formed only in the distal convoluted tubule (DCT) or the collecting duct (distal nephron). The proximal convoluted tubule (PCT) and loop of Henle are not locations for cast formation Hyaline casts not indicative of disease Hyaline casts are composed primarily of a mucoprotein (Tamm-Horsfall protein) secreted by tubule cells Sunday, March 15, 2009
    272. 272. Urinary Casts The Tamm-Horsfall protein secretion (green dots) is illustrated in the diagram below, forming a hyaline cast in the collecting duct Sunday, March 15, 2009
    273. 273. Hyaline Casts Hyaline casts, which appear very pale and slightly refractile, are common findings in urine. Sunday, March 15, 2009
    274. 274. RBC Casts Glomerular inflammation with leakage of RBC's to produce a red blood cell cast is shown in the diagram Sunday, March 15, 2009
    275. 275. RBC Casts Red cell casts — The finding of red cell casts, even if only one is seen, is virtually diagnostic of glomerulonephritis Sunday, March 15, 2009
    276. 276. WBC Casts White cell casts — The presence of white cell casts and pyuria alone is most consistent with a tubulointerstitial disease or acute pyelonephritis Sunday, March 15, 2009
    277. 277. Urine Microscopy Casts Epithelial cell casts — Acute tubular necrosis and acute glomerulonephritis, disorders in which epithelial cells are desquamated, may be associated with epithelial cell casts Fatty casts — Among patients with significant proteinuria, the degeneration of cells within epithelial casts may result in a fatty cast Sunday, March 15, 2009
    278. 278. Polarized Light Oval Fat Bodies appearance “Maltese Cross” Sunday, March 15, 2009
    279. 279. Granular Casts Granular casts — When cellular casts or aggregated proteins remain in the nephron for some time before they are flushed into the bladder urine, the cells may degenerate to become a coarsely granular cast and later a finely granular cast Sunday, March 15, 2009
    280. 280. Waxy Casts Waxy casts —thought to be the last stage of the degeneration of a granular cast. Since this degenerative process is probably slow, it is most likely observed in nephrons with very diminished flow. Therefore most consistent with the presence of advanced renal failure Sunday, March 15, 2009
    281. 281. Urine Microscopy Casts Sunday, March 15, 2009
    282. 282. Case Presentation Sunday, March 15, 2009
    283. 283. Case Presentation 30 yr old white male athlete CC: Elevated Creatinine Sunday, March 15, 2009
    284. 284. What do you want next? Past Medical History Family History Social History Medications Physical Exam Anything Else? Sunday, March 15, 2009
    285. 285. History HPI:Recurrent sinus infections. Some fatigue limiting his physical activity. Weight loss 10 lbs over last couple months PMHx: none Social Hx: Single, Denies tobacco or illicit drug use, Social EtOH Family Hx: No Kidney Disease, ESRD Medications: Zithromax Sunday, March 15, 2009
    286. 286. Physical Exam VS: BP 144/86 mmHg P 96 R 14 HEENT: NCAT OP lesion palate Neck: Supple no JVD no bruits Chest: CTA B/L Cor: S1 S2 RRR no M/R/G Abd: Soft NT ND +BS, no HSM Ext: no edema Skin: no rash Anything else? Sunday, March 15, 2009
    287. 287. Labs What do you want to order? Sunday, March 15, 2009
    288. 288. Lab Values CBC: WBC 7.6 Hb 10.9 PLT 415 CMP: Na 138 K 4.3 Cl 108 HCO3 23 BUN 41 Creat 3.4 TBili 0.6 AST 22 ALT 25 AlkP 106 TP 6.2 Alb 3.0 Cholesterol: Chol 222 HDL 29 LDL 168 UA Dip: +1 protein, +2 blood, no LE or nitrates Want anything else? Sunday, March 15, 2009
    289. 289. Other Tests to consider Hepatitis Panel Compliments, ANA, c-ANCA, p-ANCA, anti-GBM, ASLO, HIV 24 Hour urine Creatinine Clearance and total proteinuria (U TP/Creat) CXR Sunday, March 15, 2009
    290. 290. 24 Hour urine collection Creatinine Clearance 49 ml/min Total proteinuria: 1,890 mg Sunday, March 15, 2009
    291. 291. What next? Sunday, March 15, 2009
    292. 292. Renal Biopsy! Sunday, March 15, 2009
    293. 293. Chest XRAY Sunday, March 15, 2009
    294. 294. Biopsy Sunday, March 15, 2009
    295. 295. Serology Findings C3 / C4 / CH50 WNL Hepatitis Panel negative HIV negative c-ANCA 1:640 p-ANCA negative ANA negative anti-GBM negative ASLO negative Sunday, March 15, 2009
    296. 296. Diagnosis? Sunday, March 15, 2009
    297. 297. Wegner’s Granulomatosis Sunday, March 15, 2009
    298. 298. What Should We do Next? How Should we Treat? What is the prognosis? Sunday, March 15, 2009
    299. 299. FIN Simon Prince, DO, FACP, FASN Assistant Professor of Medicine; NYU School of Medicine email: sprince@nsneph.com www.nsneph.blogspot.com Sunday, March 15, 2009
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