Interstitial nephritis associated with PostInfectious GN

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Interstitial nephritis associated with
PostInfectious GN

• PRAET MARLEEN , MD, PhD
• UNIVERSITY HOSPITAL GHENT

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Clinical History Background

• Man
• 53 year
• Ethyl , smoking 10-12 cigars/day
• 1994 T3N0M0 Spinocellular Carcinoma of the
  glottis
• 2007-2010 recurrent hemoptoe presenting a
  cystic lesion at the Right Upper Lobe of the
  Lung.

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Clinical History Recent

• 04/10/2011 lobectomie
• Histology       
• Pachypleuritis met underlying scar of the
  pulmonary parenchyma. Bronchiectasy and chronic
  inflammation.
• No malignancy.    
• Follow up hydropneumothorax with infection crp
  15 mg/dL, WBC 19000 103/µL, fever 39C, sputum
  H.Influenza

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Admission in Emergency 3 weeks after lobectomy

• Acute renal failure
• - Creatinin 4,21 mg/dl
• - Proteinuria 4.3g/L
• - Macroscopic hematuria
• - Oliguria
• - WBC 21700 103/µL
• - CRP 10.6 mg/dl
• Normal temperature, normal BP
• Renal biopsy.

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AgMethanamine x 4
Kidney biopsy containing 30 glomeruli 4
glomeruli are completely sclerosed. 7 glomeruli
undergo proliferative changes with crescent
formation surrounding the glomeruli segmentally
or globally. Glomeruli, tubuli and interstitium
are infiltrated by neutrophils. No vasculitis
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AG Methanamine x10
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CONGORED x25
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CONGORED X 10
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PAS x40
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Differential Diagnosis

• (Focal) crescentic glomerulonephritis post
  infection (PIGN).
• Microangiopathic vasculitis with crescentic
  glomerulonephritis ANCA-associated systemic
  vasculitides (Wegener, microscopic polyangitis,
  Churg- Strauss)
• Sepsis with combined interstitial and glomerular
  changes.

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Immunofluorescence Findings

• Ig G, Ig A, Ig M, C1Q negative IF findings
• Kappa, Lambda negative IF findings
• C3 strong granular staining at capillary wall 3
• SUGGESTED DIAGNOSIS Post infectious
  glomerulonephritis with crescent formation in lt
  50 of the glomeruli. IF findings consistent with
  previous infection.

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C3 Deposition at capillary wall
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ORIGIN OF INFECTION

• 2 possibilities
• - Hydropneumothorax with infectious
• agent H. Influenzae was found in the
• sputum.
• - Bronchiectasy with ulcerative
• inflammation and presence of germs
• however no infectious agent was
• cultivated

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Treatment of the patient

• Original clinical diagnosis vasculitis
  plasmapheresis, cyclophosphamide, high dosed
  steroids. Creat levels up tot 6. 65 mg/dl.
  However ANCA negative, anti GBM negative
• Switch of treatment after IF findings stop
  plasmapheresis, stop cyclophosphamide
• Instead intravenous AB, steroids, dialysis.
• Creat level is decreasing with recovery of the
  patient.

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Discussion

• Glomerulonephritis and infection
• - is primarily a childhood disease occuring after
  upper respiratory infection(5-10 ) or impetigo
  (25) (Streptococcus A, beta hemolytic,
  serotypes 12, 49)
• - in older patients less well known
• Male/female ratio 2.81
• Immunocompromised background is present in 61 ,
  most often diabetes or malignancy
• Infectious agent most often found staphylococcus
  (46), streptococcus (16) and unusual gram-
  negative organisms.

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Discussion

• Glomerulonephritis and infection
• IF findings in PIGN IgG and C3, or C3 only
• IgA dominant PIGN strong association with
  staphylococcal infections of the skin with
  diabetes as a major risk. This variant of APIGN
  should be distinguished from the classic IgA
  nephropathy (Haas M Human Pathology 2008, 39,
  1309-1316, Nasr S, DAgati Nephron Clin Pract
  2011, 119, 18-26)
• EM findings classical PIGN large subepithelial
  deposits (humps). APIGN often no subepithelial
  deposits with varied findings (subendothelial,
  mesangial). Our patient NO glomeruli in EM
  material.

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DISCUSSION

• Glomerulonephritis and infection in our patient
  no definite infectious agent revealed
• But immunocompromised alcoholism
• NASR. ET AL. Acute Postinfectious
  Glomerulonephritis in the Modern Era. Medicine,
  8721-32, 2008

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NASR. ET AL. Acute Postinfectious
Glomerulonephritis in the Modern Era. Medicine,
8721-32, 2008

• In Western Europe, alcoholism had become the
  most important risk factor for Acute
  Postinfectious Glomerulonephritis
• Upper respiratory tract gt skin gt lung gt
  endocarditis gt teeth
• 56 complete remission
• 4-17 requiring renal replacement therapy
• Evidence supporting the use of steroid therapy
  for postinfectious crescentic GN is largely
  anecdotal