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Diagnosis: Collapsing glomerulopathy
(Focal and segmental glomerulosclerosis collapsing variant)

Collapsing glomerulopathy is a morphologic variant of focal segmental glomerulosclerosis (FSGS) characterized by segmental or global collapse of the glomerular capillaries, marked hypertrophy and hyperplasia of podocytes, and severe tubulointerstitial disease. The cause of this disorder is unknown, but nearly identical pathologic findings are present in idiopathic collapsing glomerulopathy and human immunodeficiency virus (HIV)-associated nephropathy, and collapsing glomerulopathy has been associated with parvovirus B19 infection and treatment with pamidronate. Collapsing glomerulopathy can also develop in association with lymphoproliferative disorders, systemic lupus erythematosus-like and other autoimmune diseases, other immune deficiency syndromes and viral infections, and in the context of immunosuppressive therapy.

It is not entirely clear the pathogenesis of collapsing glomerulopathy, but this involves visceral epithelial cell injury leading to cell cycle dysregulation and a proliferative phenotype. Clinically, collapsing glomerulopathy is characterized by black racial predominance, a high incidence of nephrotic syndrome, and rapidly progressive renal failure. It is possible that Hispanic patients have a bigger incidence of this glomerulopathy (study in preparation). Collapsing glomerulopathy also may recur after renal transplantation or present de novo, often leading to loss of the allograft. The optimal treatment for collapsing glomerulopathy is unknown. Treatments may include steroids or cyclosporine in addition to aggressive blood pressure control, angiotensin converting enzyme inhibitors and/or angiotensin II receptor blockers, and lipid lowering agents. The role of other immunosuppressive agents in the treatment of collapsing FSGS remains to be defined.

See the chapter [Focal segmental glomerulosclerosis] (only in spanish).

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Bibliography

• Srivastava T, Garola RE, Singh HK. Cell-cycle regulatory proteins in the podocyte in collapsing glomerulopathy in children. Kidney Int. 2006, 70:529-35. [PubMed link]
• Schwimmer JA, Markowitz GS, Valeri A, Appel GB. Collapsing glomerulopathy. Semin Nephrol. 2003, 23:209-18. [PubMed link]
• Laurinavicius A, Rennke HG. Collapsing glomerulopathy--a new pattern of renal injury. Semin Diagn Pathol. 2002, 19:106-15. [PubMed link]
• Singh HK, Baldree LA, McKenney DW, Hogan SL, Jennette JC. Idiopathic collapsing glomerulopathy in children. Pediatr Nephrol. 2000, 14:132-7. [PubMed link]
• Laurinavicius A, Hurwitz S, Rennke HG. Collapsing glomerulopathy in HIV and non-HIV patients: a clinicopathological and follow-up study. Kidney Int. 1999, 56:2203-13. [PubMed link]
• Stokes MB, Davis CL, Alpers CE. Collapsing glomerulopathy in renal allografts: a morphological pattern with diverse clinicopathologic associations. Am J Kidney Dis. 1999, 33:658-66. [PubMed link]
• Grcevska L, Polenakovik M. Collapsing glomerulopathy: clinical characteristics and follow-up. Am J Kidney Dis. 1999, 33:652-7. [PubMed link]
• Detwiler RK, Falk RJ, Hogan SL, Jennette JC. Collapsing glomerulopathy: a clinically and pathologically distinct variant of focal segmental glomerulosclerosis. Kidney Int. 1994, ;45:1416-24. [PubMed link]
  
  

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