Monte Minute‎ > ‎Monte Minute‎ > ‎

MPGN Mai Colvin PGY-3

posted Dec 10, 2014, 6:54 AM by Kevin Hauck

Today’s case presentation at Moses with Dr. Folkert was about a 50yo man with decompensated HCV cirrhosis (failed tx ~15yrs ago?), hx of polysubstance abuse on methadone presenting with worsening LE swelling x 3 wks.   Vitals WNL.  Exam showed diffuse crackles, grade II/VI systolic murmur heard best at the L 4th IC space, distended abdomen with fluid wave, 2+ pitting edema extends to the sacrum, no asterixis, and normal mental status.  Labs notable for Cr 1.9 (0.9 in 12/2013), albumin < 2, U/A with > 1000 protein and large occult blood. Urine sediment showed no cast.  Further work-up revealed C3 40 (low), C4 13 (low), and cryoglobulin neg (x1).   This monte minute will go over how we thought through the case. 

Background:

The term nephrotic syndrome is defined by the presence of heavy proteinuria (protein excretion of > 3-3.5g per day), hypoalbuminemia (<3g/dL), and peripheral edema. 

 

7 most common causes of Nephrotic syndrome (in no specific order):

·        Focal segmental glomerulosclerosis (FSGS)/idiopathic

·        Membranous nephropathy

·        Minimal change disease

o   MCC of nephrotic syndrome in children

·        Diabetic nephropathy

·        Systemic lupus erythematosus (SLE)

·        Amyloidosis

·        HIV associated nephropathy

Note: Aprroximately 30% of adults with the nephrotic syndrome have a systemic disease such as DM, amyloidosis, or SLE.  The remaining cases are usually due to primary disorders including FSGS, minimal change disease, and membranous nephropathy.  Heavy proteinuria without edema or hypoalbuminemia is more likely to be due to secondary FSGS. 

 

When building our differential diagnosis, membranoproliferative glomerulonephritis (MPGN) was added to this list given this patient’s history of Hep C although MPGN more commonly manifests as part of nephritic syndrome.   Also based on patient’s age and history, diabetic nephropathy, SLE, amyloidosis, and HIV associated nephropathy were taken out of our ddx at this time. 

 

We then reviewed the common causes of low serum complement GN

·        SLE

·        Endocarditis

·        MPGN

·        Post-infectious GN

 

A number of serologic studies often are obtained in the evaluation of patients with the nephrotic syndrome, however, in most adults a renal biopsy is required to establish the diagnosis. 

 

This patient’s renal biopsy revealed Membranoproliferative glomerulonephritis.

Patient is a candidate for Rituximab from the renal stand point, however patient with Hep C VL >150000 and hx of HepB exposure (core ab +).  Patient will receive outpatient HCV treatment along and hep B prophylaxis, and once HCV Tx is initiated, pt will receive rituximab. 


Comments