Collapsing glomerulopathy in collagen vascular-like disease

Abstract

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.

Collapsing Focal Segmental Glomerulosclerosis in a Patient with Systemic Lupus Erythematosus

“…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].…”

“…Associations with numerous etiologies apart from HIV infection have been reported including other infections (tuberculosis, CMV, parvovirus B19, and hepatitis B and C), drugs (including pamidronate, anabolic steroids, heroin, and interferons), malignancies (multiple myeloma, hemophagocytic syndrome, and acute monoblastic leukemia), and autoimmune diseases like SLE, adult Still's disease, and mixed connective tissue disease, while in the primary form it is seen mostly in patients of African descent [1, 2, 6, 7, 9–14]. It is more common in females compared to males [5].…”

“…Patients with CG usually present with severe renal failure, massive proteinuria and usually have a poor response to steroids and other immunosuppressive therapy [2, 6, 13, 20–22]. They may also present with hypoalbuminemia, hypercholesterolemia, and edema with manifestations being not different compared to noncollapsing focal segmental glomerulosclerosis (FSGS) [15].…”

“…CG has an aggressive course with the mean time from biopsy diagnosis to end-stage renal disease of 13 months compared with 63 months in patients with idiopathic focal segmental glomerulosclerosis [23] suggesting that CG has a more worse prognosis [15, 20, 22]. These patients carry a poor prognosis and most of them end up being dialysis dependent [6], reported to be around 50–100% even with the currently available treatment [3, 24, 25]. …”

“…Retrospective studies have shown good results when steroids are started shortly after diagnosis, although there are no prospective treatment trials [25]. Role of other immunosuppressants including mycophenolate mofetil is yet to be defined [6, 25]. Some studies have shown plasmapheresis as an option for treatment of recurrent FSGS and it has been shown to decrease proteinuria and glomerular injury [27].…”