Focal Segmental Glomerulosclerosis Questions & Answers

1. Rosenberg AZ, Kopp JB. Focal Segmental Glomerulosclerosis. Clin J Am Soc Nephrol. 2017 Mar 7. 12 (3):502-517. [QxMD MEDLINE Link]. [Full Text].

2. Freedman BI, Hicks PJ, Bostrom MA, et al. Polymorphisms in the non-muscle myosin heavy chain 9 gene (MYH9) are strongly associated with end-stage renal disease historically attributed to hypertension in African Americans. Kidney Int. 2009 Jan 28. [QxMD MEDLINE Link].

3. Shabaka A, Tato Ribera A, Fernández-Juárez G. Focal Segmental Glomerulosclerosis: State-of-the-Art and Clinical Perspective. Nephron. 2020. 144 (9):413-427. [QxMD MEDLINE Link]. [Full Text].

4. Rao TKS. Renal complications in HIV disease. Med Clin North Am. 1996 Nov. 80(6):1437-51. [QxMD MEDLINE Link].

5. Winston JA, Burns GC, Klotman PE. The human immunodeficiency virus (HIV) epidemic and HIV-associated nephropathy. Semin Nephrol. 1998 Jul. 18(4):373-7. [QxMD MEDLINE Link].

6. D'Agati VD. The spectrum of focal segmental glomerulosclerosis: new insights. Curr Opin Nephrol Hypertens. 2008 May. 17(3):271-81. [QxMD MEDLINE Link].

7. Barisoni L, Schnaper HW, Kopp JB. Advances in the biology and genetics of the podocytopathies: implications for diagnosis and therapy. Arch Pathol Lab Med. 2009 Feb. 133(2):201-16. [QxMD MEDLINE Link].

8. D'Agati VD, Fogo AB, Bruijn JA, Jennette JC. Pathologic classification of focal segmental glomerulosclerosis: a working proposal. Am J Kidney Dis. 2004 Feb. 43 (2):368-82. [QxMD MEDLINE Link]. [Full Text].

9. D'Agati VD, Alster JM, Jennette JC, Thomas DB, Pullman J, Savino DA. Association of histologic variants in FSGS clinical trial with presenting features and outcomes. Clin J Am Soc Nephrol. 2013 Mar. 8(3):399-406. [QxMD MEDLINE Link].

10. Tryggvason K, Patrakka J, Wartiovaara J. Hereditary proteinuria syndromes and mechanisms of proteinuria. N Engl J Med. 2006 Mar 30. 354(13):1387-401. [QxMD MEDLINE Link].

11. Winn MP, Conlon PJ, Lynn KL, et al. A mutation in the TRPC6 cation channel causes familial focal segmental glomerulosclerosis. Science. 2005 Jun 17. 308(5729):1801-4. [QxMD MEDLINE Link].

12. Shankland SJ, Pollak MR. A suPAR circulating factor causes kidney disease. Nat Med. 2011 Aug 4. 17(8):926-7. [QxMD MEDLINE Link].

13. Gribouval O, Boyer O, Knebelmann B, Karras A, Dantal J, Fourrage C, et al. APOL1 risk genotype in European steroid-resistant nephrotic syndrome and/or focal segmental glomerulosclerosis patients of different African ancestries. Nephrol Dial Transplant. 2018 Jul 9. 40 (5):3306-3310. [QxMD MEDLINE Link].

14. Mele C, Iatropoulos P, Donadelli R, et al. MYO1E mutations and childhood familial focal segmental glomerulosclerosis. N Engl J Med. 2011 Jul 28. 365(4):295-306. [QxMD MEDLINE Link].

15. McCarthy ET, Sharma M, Savin VJ. Circulating permeability factors in idiopathic nephrotic syndrome and focal segmental glomerulosclerosis. Clin J Am Soc Nephrol. 2010 Nov. 5(11):2115-21. [QxMD MEDLINE Link].

16. Wei C, El Hindi S, Li J, Fornoni A, et al. Circulating urokinase receptor as a cause of focal segmental glomerulosclerosis. Nat Med. 2011 Jul 31. 17(8):952-60. [QxMD MEDLINE Link].

17. Wei C, Trachtman H, Li J, Dong C, Friedman AL, Gassman JJ. Circulating suPAR in two cohorts of primary FSGS. J Am Soc Nephrol. 2012 Dec. 23(12):2051-9. [QxMD MEDLINE Link].

18. Genovese G, Friedman DJ, Ross MD, et al. Association of trypanolytic ApoL1 variants with kidney disease in African Americans. Science. 2010 Aug 13. 329(5993):841-5. [QxMD MEDLINE Link]. [Full Text].

19. Tao J, Mariani L, Eddy S, Maecker H, Kambham N, Mehta K, et al. JAK-STAT signaling is activated in the kidney and peripheral blood cells of patients with focal segmental glomerulosclerosis. Kidney Int. 2018 Oct. 94 (4):795-808. [QxMD MEDLINE Link].

20. Rao TKS, Nicastri AD, Friedman EA. The nephropathies of drug addiction and acquired immunodeficiency syndrome. Renal Pathology. New York, NY: JB Lippincott; 1989. 340-56.

21. Cunningham EE, Brentjens JR, Zielezny MA, et al. Heroin nephropathy. A clinicopathologic and epidemiologic study. Am J Med. 1980 Jan. 68(1):47-53. [QxMD MEDLINE Link].

22. Cunningham EE, Zielezny MA, Venuto RC. Heroin-associated nephropathy. A nationwide problem. JAMA. 1983 Dec 2. 250(21):2935-6. [QxMD MEDLINE Link].

23. Friedman EA, Tao TK. Disappearance of uremia due to heroin-associated nephropathy. Am J Kidney Dis. 1995 May. 25(5):689-93. [QxMD MEDLINE Link].

24. Rao TKS, Nicastri AD, Friedman EA. Natural history of heroin-associated nephropathy. N Engl J Med. 1974 Jan 3. 290(1):19-23. [QxMD MEDLINE Link].

25. Chandra P, Kopp JB. Viruses and collapsing glomerulopathy: a brief critical review. Clin Kidney J. 2013 Feb. 6 (1):1-5. [QxMD MEDLINE Link]. [Full Text].

26. Muehlig AK, Gies S, Huber TB, Braun F. Collapsing Focal Segmental Glomerulosclerosis in Viral Infections. Front Immunol. 2021. 12:800074. [QxMD MEDLINE Link]. [Full Text].

27. Bruggeman LA, Dikman S, Meng C, et al. Nephropathy in human immunodeficiency virus-1 transgenic mice is due to renal transgene expression. J Clin Invest. 1997 Jul 1. 100(1):84-92. [QxMD MEDLINE Link].

28. D''Agati V, Appel GB. HIV infection and the kidney. J Am Soc Nephrol. 1997 Jan. 8(1):138-52. [QxMD MEDLINE Link].

29. Kimmel PL, Bosch JP, Vassalotti JA. Treatment of human immunodeficiency virus (HIV)-associated nephropathy. Semin Nephrol. 1998 Jul. 18(4):446-58. [QxMD MEDLINE Link].

30. Verani RR. Obesity-associated focal segmental glomerulosclerosis: pathological features of the lesion and relationship with cardiomegaly and hyperlipidemia. Am J Kidney Dis. 1992 Dec. 20(6):629-34. [QxMD MEDLINE Link].

31. Kitiyakara C, Eggers P, Kopp JB. Twenty-one-year trend in ESRD due to focal segmental glomerulosclerosis in the United States. Am J Kidney Dis. 2004 Nov. 44(5):815-25. [QxMD MEDLINE Link].

32. McGrogan A, Franssen CF, de Vries CS. The incidence of primary glomerulonephritis worldwide: a systematic review of the literature. Nephrol Dial Transplant. 2011 Feb. 26 (2):414-30. [QxMD MEDLINE Link].

33. Kitiyakara C, Eggers P, Kopp JB. Twenty-one-year trend in ESRD due to focal segmental glomerulosclerosis in the United States. Am J Kidney Dis. 2004 Nov. 44 (5):815-25. [QxMD MEDLINE Link].

34. Kopp JB, Smith MW, Nelson GW, Johnson RC, Freedman BI, Bowden DW. MYH9 is a major-effect risk gene for focal segmental glomerulosclerosis. Nat Genet. 2008 Oct. 40(10):1175-84. [QxMD MEDLINE Link].

35. Kopp JB, Nelson GW, Sampath K, Johnson RC, Genovese G, An P. APOL1 genetic variants in focal segmental glomerulosclerosis and HIV-associated nephropathy. J Am Soc Nephrol. 2011 Nov. 22(11):2129-37. [QxMD MEDLINE Link].

36. Gipson DS, Trachtman H, Kaskel FJ, et al. Clinical trial of focal segmental glomerulosclerosis in children and young adults. Kidney Int. 2011 Oct. 80(8):868-78. [QxMD MEDLINE Link].

37. Del Rio M, Kaskel F. Evaluation and management of steroid-unresponsive nephrotic syndrome. Curr Opin Pediatr. 2008 Apr. 20(2):151-6. [QxMD MEDLINE Link].

38. Deegens JK, Wetzels JF. Immunosuppressive treatment of focal segmental glomerulosclerosis: lessons from a randomized controlled trial. Kidney Int. 2011 Oct. 80(8):798-801. [QxMD MEDLINE Link].

39. Guruswamy Sangameswaran KD, Baradhi KM. Focal Segmental Glomerulosclerosis. 2022 Jan. [QxMD MEDLINE Link]. [Full Text].

40. Zhang Q, Jiang C, Tang T, Wang H, Xia Y, Shao Q, et al. Clinical Significance of Urinary Biomarkers in Patients With Primary Focal Segmental Glomerulosclerosis. Am J Med Sci. 2018 Apr. 355 (4):314-321. [QxMD MEDLINE Link].

41. Korbet SM. Treatment of primary FSGS in adults. J Am Soc Nephrol. 2012 Nov. 23(11):1769-76. [QxMD MEDLINE Link].

42. Crook ED, Habeeb D, Gowdy O, et al. Effects of steroids in focal segmental glomerulosclerosis in a predominantly African-American population. Am J Med Sci. 2005 Jul. 330(1):19-24. [QxMD MEDLINE Link].

43. [Guideline] Kidney Disease/Improving Global Ooutcomes. KDIGO Clinical Practice Guideline for Glomerulonephritis (GN). Kidney Int. June 2012. 2 suppl 2:[Full Text].

44. Ramachandran R, Kumar V, Rathi M, Nada R, Jha V, Gupta KL, et al. Tacrolimus therapy in adult-onset steroid-resistant nephrotic syndrome due to a focal segmental glomerulosclerosis single-center experience. Nephrol Dial Transplant. 2014 Oct. 29(10):1918-24. [QxMD MEDLINE Link].

45. Laurin LP, Gasim AM, Poulton CJ, Hogan SL, Jennette JC, Falk RJ, et al. Treatment with Glucocorticoids or Calcineurin Inhibitors in Primary FSGS. Clin J Am Soc Nephrol. 2016 Mar 7. 11 (3):386-94. [QxMD MEDLINE Link]. [Full Text].

46. Jordana B. Cohen, Jonathan J Hogan. Rethinking first line immunosuppression for Idiopathic FSGS. Clin J Am Soc Nephrol. 2016. 11:372-373. [QxMD MEDLINE Link]. [Full Text].

47. Garrouste C, Canaud G, Büchler M, Rivalan J, Colosio C, Martinez F, et al. Rituximab for Recurrence of Primary Focal Segmental Glomerulosclerosis After Kidney Transplantation: Clinical Outcomes. Transplantation. 2016 Apr 13. 17 (2):79-82. [QxMD MEDLINE Link].

48. Ruggenenti P, Ruggiero B, Cravedi P, Vivarelli M, Massella L, et al. Rituximab in steroid-dependent or frequently relapsing idiopathic nephrotic syndrome. J Am Soc Nephrol. 2014 Apr. 25 (4):850-63. [QxMD MEDLINE Link]. [Full Text].

49. Kronbichler A, Kerschbaum J, Fernandez-Fresnedo G, Hoxha E, Kurschat CE, Busch M, et al. Rituximab treatment for relapsing minimal change disease and focal segmental glomerulosclerosis: a systematic review. Am J Nephrol. 2014. 39(4):322-30. [QxMD MEDLINE Link].

50. Kang HG, Ha IS, Cheong HI. Recurrence and Treatment after Renal Transplantation in Children with FSGS. Biomed Res Int. 2016. 2016:6832971. [QxMD MEDLINE Link]. [Full Text].

51. Rudnicki M. FSGS Recurrence in Adults after Renal Transplantation. Biomed Res Int. 2016. 2016:3295618. [QxMD MEDLINE Link].

52. Garrouste C, Canaud G, Büchler M, Rivalan J, Colosio C, Martinez F, et al. Rituximab for Recurrence of Primary Focal Segmental Glomerulosclerosis After Kidney Transplantation: Clinical Outcomes. Transplantation. 2017 Mar. 101 (3):649-656. [QxMD MEDLINE Link].

53. Uffing A, Hullekes F, Hesselink DA, Mansur JB, Malvezzi P, de Vries APJ, et al. Long-term Apheresis in the Management of Patients With Recurrent Focal Segmental Glomerulosclerosis After Kidney Transplantation. Kidney Int Rep. 2022 Jun. 7 (6):1424-1427. [QxMD MEDLINE Link]. [Full Text].

54. FDA approves new pediatric use for Liposorber Apheresis System [news release]. October 10, 2013. Available at http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm370931.htm. Accessed: October 15, 2013.

55. Han KH, Kim SH. Recent Advances in Treatments of Primary Focal Segmental Glomerulosclerosis in Children. Biomed Res Int. 2016. 2016:3053706. [QxMD MEDLINE Link].

56. Beer A, Mayer G, Kronbichler A. Treatment Strategies of Adult Primary Focal Segmental Glomerulosclerosis: A Systematic Review Focusing on the Last Two Decades. Biomed Res Int. 2016. 2016:4192578. [QxMD MEDLINE Link].

57. Chen L, Wu J, Ying D, Jiang M, Xu Y, Mo Y, et al. Application of adrenocorticotropic hormone in recurrent focal segmental glomerulosclerosis post-transplantation: A case report and literature review. Pediatr Transplant. 2022 Mar. 26 (2):e14184. [QxMD MEDLINE Link].

58. Hogan J, Bomback AS, Mehta K, Canetta PA, Rao MK, Appel GB, et al. Treatment of idiopathic FSGS with adrenocorticotropic hormone gel. Clin J Am Soc Nephrol. 2013 Dec. 8 (12):2072-81. [QxMD MEDLINE Link]. [Full Text].

59. Alhamad T, Dieck JM, Younus U, Matar D, Alasfar S, Vujjini V, et al. ACTH Gel in Resistant Focal Segmental Glomerulosclerosis after Kidney Transplantation. Transplantation. 2018 Jun 12. [QxMD MEDLINE Link].

60. Caster DJ, Magalhaes B, Pennese N, Zaffalon A, Faiella M, Campbell KN, et al. Efficacy and Safety of Immunosuppressive Therapy in Primary Focal Segmental Glomerulosclerosis: A Systematic Review and Meta-analysis. Kidney Med. 2022 Aug. 4 (8):100501. [QxMD MEDLINE Link]. [Full Text].

Sreepada TK Rao, MD, FACP Professor, Department of Medicine, State University of New York Downstate Medical Center

Sreepada TK Rao, MD, FACP is a member of the following medical societies: American Society of Hypertension, International Society of Nephrology, American Society of Nephrology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Eleanor Lederer, MD, FASN Professor of Medicine, Chief, Nephrology Division, Director, Nephrology Training Program, Director, Metabolic Stone Clinic, Kidney Disease Program, University of Louisville School of Medicine; Consulting Staff, Louisville Veterans Affairs Hospital

Eleanor Lederer, MD, FASN is a member of the following medical societies: American Association for the Advancement of Science, American Society for Bone and Mineral Research, American Society of Nephrology, American Society of Transplantation, International Society of Nephrology, Kentucky Medical Association, National Kidney Foundation

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: American Society of Nephrology<br/>Received income in an amount equal to or greater than $250 from: Healthcare Quality Strategies, Inc.

Vecihi Batuman, MD, FASN Huberwald Professor of Medicine, Section of Nephrology-Hypertension, Interim Chair, Deming Department of Medicine, Tulane University School of Medicine

Vecihi Batuman, MD, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, International Society of Nephrology, Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Chike Magnus Nzerue, MD, FACP Professor of Medicine, Associate Dean for Clinical Affairs, Meharry Medical College

Chike Magnus Nzerue, MD, FACP is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Society of Nephrology, National Kidney Foundation

Disclosure: Nothing to disclose.

Anjana S Soman, MD Staff Physician, Department of Pathology, Quest Diagnostics

Anjana S Soman, MD is a member of the following medical societies: American Society for Clinical Pathology and College of American Pathologists

Disclosure: Nothing to disclose.

• Print this section
• Print the entire contents of
• Print the entire contents of article