Collapsing Glomerulopathy in Collagen Vascular-Like Disease

1Division of Rheumatology, University of Minnesota Medical School, USA.
Lupus (Impact Factor: 2.2). 11/2013; 23(1). DOI: 10.1177/0961203313513509
Source: PubMed


Collapsing glomerulopathy (CG) is a podocytopathy that is usually associated with human immunodeficiency virus (HIV) and parvovirus B19 infections. CG has been reported in association with definite collagen vascular diseases, mainly systemic lupus erythematosus (SLE). There are a few case reports in the nephrology literature of patients with CG and marked serological abnormalities who do not have sufficient clinical findings to diagnose definite collagen vascular disease. We wish to expand the spectrum of rheumatologic disease that accompanies CG. We describe four patients with CG and collagen vascular-like disease and compare these with 14 similar cases reported in the medical literature.Methods
Case reports of four new patients with CG and collagen vascular-like disease are presented. We performed a systematic literature review to find all other cases and construct a profile of patients with CG and collagen vascular-like disease.ResultsAll patients had a similar mode of presentation with severe nephrotic range proteinuria and renal insufficiency resistant to steroids and usual immunomodulatory therapy. All patients had positive antinuclear antibodies (ANA) as well as other marked serological abnormalities but few if any clinical findings that would allow for a definitive diagnosis of a specific collagen vascular disease. Almost all patients became dialysis dependent. Mycophenolate mofetil (MMF) may possibly be a therapeutic option.Conclusion
Rheumatologists may be asked to consult on patients with severe proteinuria and renal insufficiency in the presence of marked serological abnormalities but few clinical symptoms and should be aware of this podocytopathy.

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    • "Collapsing glomerulopathy (CG) has a rapid clinical course with massive proteinuria and relative resistance to standard treatments available. It has become an increasingly recognized pattern of glomerular injury with pathologic appearance characterized by global or segmental collapse of the glomerular capillary tuft, with wrinkling and retraction of the capillary walls overlaid by epithelial cell proliferation in the Bowman space that is frequently accompanied by tubulointerstitial disease [2, 5, 6]. CG shares features with HIV induced nephropathy (HIVAN) but has been shown to be a totally separate entity on its own independent of HIV infection [2, 7, 8]. "
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    ABSTRACT: We present a case of a 36-year-old female from Ghana who presented with atypical chest pain and shortness of breath and was found to have bilateral transudative pleural effusion and trivial pericardial effusion. Further work-up revealed serological markers consistent with active lupus and negative HIV. She developed rapid deterioration of her renal function requiring dialysis. Her renal biopsy showed collapsing focal segmental glomerulosclerosis with diffuse mesangial proliferative glomerulonephritis, consistent with lupus nephritis class II along with tubular degenerative changes. She was started on high dose steroids and later on mycophenolate mofetil. Her renal function slowly recovered to baseline.
    Full-text · Article · Aug 2014 · Case Reports in Medicine