Friday, July 2, 2010

CLINICAL CASE 19, ANSWER AND SUMMARY

Which of the following is associated with MPGN pattern of injury?


Hepatitis B and C
  21 (87%)
 
Celiac Sprue
  4 (16%)
 
Systemic Lupus
  11 (45%)
 
MGUS
  5 (20%)
 
Chronic TMA
  10 (41%)
 
Paraproteinemias
  10 (41%)
 
Sickle Cell Disease
  8 (33%)
 
Parvovirus B19 infection
  1 (4%)
 


Nice work! Since in this question you could choose multiple answers, most of you got major causes of MPGN pattern of injury.  Besides parvovirus B19( classically collapsing FSGS), rest all of the above diseases cause a MPGN pattern of injury or have been documented cases of MPGN with the diseases.
I think a good article for everyone to read is H.Renke's Kidney International Forum from many years ago.
Whenever you see double contouring and MPGN pattern of injury, THINK OF secondary causes and rule it out before you call it IDIOPATHIC.
The classic four categories that you want to make sure you don't miss are:
Immune complex disease( your MPGN I, II and III and all your autoimmune disease such as SLE)
Chronic Infections( Hep virus,Cryo, malaria, endocarditis)

Chronic Thrombotic Microangiopathies( APLAS, Chronic HUS/TTP, radiation nephropathy, bone marrow transplant nephropathy, sickle cell)
Paraprotein deposition diseases  ( includes MGUS)

References:
http://www.ncbi.nlm.nih.gov/pubmed/7723253

3 comments:

  1. I have a patient with diagnostic of MPGN idiophatic? duoble contouring in pattern of injury we rule out autoinmune disease such as lupus, HVC infection, paraprotein deposition diseases ( MGUS), the patient start treatment with MMF 1 gr/d and corticoteraphy for one year and the proteinuria disapeared but after that the proteinuria start to progress again.Re biopsy now and the biopsy said the same diagnostic. What do you think about treatment? and now what another treatment can put in this patient?
    proteinuria: 7.75 gr, normal renal function.

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    Replies
    1. The new classification of MPGN-
      1. C3 and Ig deposit
      2. C3 deposit only
      3. No Ig and C3 deposit.
      Which criteria does the patient fit, check with the pathologist, and then proceed accordingly with fresh pair of eyes.

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  2. Look for a cause, always..
    How about Light chain deposition disease? which sometimes give you negative SPEP AND negative Light chain in the renal biopsy. Check FLC assay, if positive, check BM biopsy.

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