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==Overview==
==Overview==


==Natural History==
==Natural History, Complications, and Prognosis==
 
==Complications==
Renal complications are the most common and most feared complications of focal segmental glomerulosclerosis (FSGS). Unlike minimal change disease, spontaneous remission is very unlikely in FSGS. The degree of proteinuria correlates with the probability to spontaneous remission and progression of disease into chronic kidney disease and end-stage renal disease (ESRD).<ref name="pmid10382985">{{cite journal| author=Korbet SM| title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue=  | pages= 68-73 | pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985  }} </ref>
 
==Prognosis==
The most important poor prognostic factors in focal segmental glomerulosclerosis (FSGS) are<ref name="FSGS">{{cite web |url=http://cdn.intechopen.com/pdfs/22820/InTech-Focal_segmental_glomerulosclerosis.pdf|title= Focal segmental glomerulosclerosis.|last1= Sohal |first1= DS |last2= Prabhakar |first2= SS|date=November 02, 2011|website= Interchopen |publisher= InTech |accessdate=3 December 2013}}</ref>:
*Massive or nephrotic-range proteinuria
*Level of serum creatinine > 1.3 mg/dL
*Collapsing variant on morphological appearance
*Presence of tubulointerstitial fibrosis
*Absence of remission or partial remission
*Black race


According to a 10-year study in 1999, Approximately 50% of patients with heavy proteinuria in the nephrotic-range progress to end-stage renal disease (ESRD) within 3-8 years.<ref name="pmid10382985">{{cite journal| author=Korbet SM|title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. |journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue= | pages= 68-73 |pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985  }} </ref> In converse, patients with non-nephrotic range proteinuria often have a better prognosis, with a 10-year survival reaching > 80%.<ref name="pmid10382985">{{cite journal| author=Korbet SM| title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue=  | pages= 68-73 | pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985  }} </ref> While spontaneous remission is highly uncommon in FSGS, occurring in less than 6% of patients, the degree of proteinuria may predict the probability of spontaneous remission.<ref name="pmid10382985">{{cite journal| author=Korbet SM| title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue=  | pages= 68-73 | pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985  }} </ref>
===Natural History===
*The symptoms of focal segmental glomerulosclerosis  usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.  
*The symptoms of (disease name) typically develop ___ years after exposure to ___.  
*If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].


Serum creatinine > 1.3 mg/dL and interstitial fibrosis > 20% on pathological analysis have been associated with worse renal outcomes.<ref name="pmid10382985">{{cite journal| author=Korbet SM| title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue=  | pages= 68-73 | pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985  }} </ref> The extent of proteinuria, serum creatinine, and extent of fibrosis on pathological observation have been positively correlated.<ref name="pmid10382985">{{cite journal| author=Korbet SM| title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue=  | pages= 68-73 | pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985  }} </ref>
===Complications===
Common complications of focal segmental glomerulosclerosis include:<ref name="pmid10382985">{{cite journal| author=Korbet SM| title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue=  | pages= 68-73 | pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985  }} </ref>
*End stage renal disease
*Malnutrition
*Infections
*Nephrotic syndrome


While collapsing variant, commonly seen in HIV-induced FSGS, is associated with poorer outcomes, tip variant correlates with better outcomes.<ref name="pmid16518352">{{cite journal| author=Thomas DB, Franceschini N, Hogan SL, Ten Holder S, Jennette CE, Falk RJ et al.| title=Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants. | journal=Kidney Int | year= 2006 | volume= 69 | issue= 5 | pages= 920-6 | pmid=16518352 | doi=10.1038/sj.ki.5000160 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16518352  }} </ref> According to Korbet and colleagues, the remission of proteinuria is negatively associated with progression to ESRD in FSGS.<ref name="pmid10382985">{{cite journal| author=Korbet SM| title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue= | pages= 68-73 | pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985  }} </ref>
===Prognosis===
*There are several clinical and pathological features which predict the outcome.
*Factors which are associated with worse prognosis of focal segmental glomerulosclerosis include:<ref name="urlFocal Segmental Glomerulosclerosis in Nephrotic Adults: Presentation, Prognosis, and Response to Therapy of the Histologic Variants">{{cite web |url=http://jasn.asnjournals.org/content/15/8/2169 |title=Focal Segmental Glomerulosclerosis in Nephrotic Adults: Presentation, Prognosis, and Response to Therapy of the Histologic Variants |format= |work= |accessdate=}}</ref><ref name="FSGS">{{cite web |url=http://cdn.intechopen.com/pdfs/22820/InTech-Focal_segmental_glomerulosclerosis.pdf|title= Focal segmental glomerulosclerosis.|last1= Sohal |first1= DS |last2= Prabhakar |first2= SS|date=November 02, 2011|website= Interchopen |publisher= InTech |accessdate=3 December 2013}}</ref>:
**Black race
**Increased degrees of proteinuria
**renal insufficiency
**Increased severity of interstitial fibrosis
**Tubular atrophy in biopsy specimens


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}


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Latest revision as of 20:09, 4 June 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2] Associate Editor(s)-in-Chief: Olufunmilola Olubukola M.D.[3]

Overview

Natural History, Complications, and Prognosis

Natural History

  • The symptoms of focal segmental glomerulosclerosis usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
  • The symptoms of (disease name) typically develop ___ years after exposure to ___.
  • If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

Complications

Common complications of focal segmental glomerulosclerosis include:[1]

  • End stage renal disease
  • Malnutrition
  • Infections
  • Nephrotic syndrome

Prognosis

  • There are several clinical and pathological features which predict the outcome.
  • Factors which are associated with worse prognosis of focal segmental glomerulosclerosis include:[2][3]:
    • Black race
    • Increased degrees of proteinuria
    • renal insufficiency
    • Increased severity of interstitial fibrosis
    • Tubular atrophy in biopsy specimens

References

  1. ↑ Korbet SM (1999). "Clinical picture and outcome of primary focal segmental glomerulosclerosis". Nephrol Dial Transplant. 14 Suppl 3: 68–73. PMID 10382985.
  2. ↑ "Focal Segmental Glomerulosclerosis in Nephrotic Adults: Presentation, Prognosis, and Response to Therapy of the Histologic Variants".
  3. ↑ Sohal, DS; Prabhakar, SS (November 02, 2011). "Focal segmental glomerulosclerosis" (PDF). Interchopen. InTech. Retrieved 3 December 2013. Check date values in: |date= (help)


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