Background/Purpose:

Background:

Collapsing Glomerulopathy (CG) is an uncommon podocytopathy with distinct clinical and pathological characteristics. It is usually associated with HIV disease or parvovirus B19 infection. There are a few case reports of CG in association with well defined connective tissue diseases mainly SLE. The rheumatological literature does not describe patients with CG and marked serological abnormalities who do not have sufficient clinical findings to diagnose definite collagen vascular disease. 

Objective:

Our objective is to expand the spectrum of rheumatological disease that may accompany CG to include patients with marked serological abnormalities, but minimal clinical findings that do not meet criteria for definite collagen vascular disease.  These patients all appear to have a very similar mode of presentation and course. Rheumatologists should be aware of this condition and its association with marked serological abnormalities.

Methods:

A review of all kidney biopsies performed at Regions Hospital from 2004 till 2012 revealed four cases of CG not associated with other diseases. We conducted a chart review of these patients. These four cases were then compared with the sixteen cases of collagen vascular-like disease in the renal literature to construct a profile of patients with collagen vascular-like disease and CG.

Results:

Four patients (all non-Caucasian) were identified. Ages ranged from 29 to 51 years. All patients presented with massive proteinuria (approximately 20 grams / 24 hours) and renal insufficiency. None of the renal biopsies showed evidence of lupus nephritis, immune complex deposition or vasculitis. All had evidence of CG. All testing for HIV, parvovirus B19, and other known causes of CG were negative.

 

Age	Sex	Ethnicity	Clinical	Cr (mg/dl)	Proteinuria (gr/24 hr)	ANA	Other	Rx	Outcome
38	M	AA*	Arthralgias Dry mouth	3.7	21	1:160	High titer Anti-RNP, IgG ACLA*	Prednisone, MMF*	Improved at 6 mo f/u
51	M	Cambodian	None	3.4	20	1:80	Low titer ds-DNA	Prednisone	On PD* after 3 mo
25	F	African	Alopecia	2.4	12	1:1280	High titer Anti-RNP, IgA/IgM ACLA*, ds-DNA	Prednisone, Cyclosporine, and Hydroxychloroquine	On HD* after 3 years
29	F	Hispanic	Headaches	7.6	Not done	1:640	High titer Anti MPO, low C3	Prednisone	On HD* at onset

* ACLA=Anti-cardiolipin Antibodies, PD= Peritoneal Dialysis, HD= Hemodialysis, AA= African American, MMF=Mycophenolate Mofetil

In comparison, the few cases of collagen vascular-like disease and CG in the renal literature also had marked proteinuria, renal insufficiency, and were treated with prednisone +/- immunomodulatory therapy like MMF. Most patients became dialysis dependent within two years of diagnosis (some sooner) despite initial response to steroids. 

Conclusion:

Rheumatologists may be asked to see patients with collagen vascular-like disease (minimal symptoms and marked serological abnormalities) with severe proteinuria and renal insufficiency due to CG. The presence of high titer antibodies suggests that immune activation may play a role in the podocytopathy of some patients with CG. Treatment with steroids and the agents described to date does not seem to be effective in the long term. Further definition of these patients and treatment trials are necessary.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/collapsing-glomerulopathy-in-collagen-vascular-like-disease/