Wednesday, February 1, 2012

CONSULT ROUNDS: Minimal Change Disease and lupus

Sudden onset proteinuria, lower extremity swelling, low albumin and biopsy showing complete foot process effacement with a some mesangial fullness and serologies positive for ANA and dsDNA and low complements.  Hmm!!  Minimal Change disease(MCD) with SLE?

The estimated prevalence of MCD in biopsy-proven lupus nephritis is 2.3% in childhood and 1.1% in adults. Now there are a few cases in the literature.  Most patients presented with nephrotic syndrome and not an active sediment. The patient described in the literature have  responded promptly to high-dose glucocorticoids but four (31%) had relapse of proteinuria during their course of SLE. MCD is an uncommon histological class of lupus nephritis. The prognosis of MCN in SLE appears to be good because of its rapid response to glucocorticoids. 
In 2002, another series was reported by Columbia group that had 7 patients.  All 7 patients presented with full nephrotic syndrome including peripheral edema, nephrotic range proteinuria.  In all cases, renal biopsy revealed diffuse foot process effacement in the absence of significant peripheral capillary wall immune deposits, findings consistent with minimal-change disease. In addition, 5 cases displayed mesangial electron-dense deposits, with or without associated mesangial proliferation, consistent with underlying lupus nephritis class II. It is interesting as it might be usually co existing with Class II lupus and sometimes read as possibly atypical lupus presentation.
If and individual is destined is to have lupus\, the phenotype that they present with might be different based on the individual genetic make up. It is unclear why minimal change is not a pre dominant variant of lupus, perhaps in children?

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