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| ==Overview== | | ==Overview== |
| Several clinical and pathological factors are associated with poor renal outcome. The most important prognostic factor in FSGS is the patient's response to therapy. Proteinuria is an important predictor of spontaneous remission or progression of FSGS into ESRD. Generally, spontaneous remission is an unlikely event in FSGS; and more than 50% of patients with nephrotic-range proteinuria reach ESRD within 3-8 years. Other important prognostic factors include male gender, black race, high level of serum creatinine and collapsing variant on histopathological analysis.
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| ==Natural History, Complications, and Prognosis== | | ==Natural History, Complications, and Prognosis== |
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| ===Natural History=== | | ===Natural History=== |
| *The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___. | | *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 ___. | | *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]. | | *If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3]. |
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| ===Complications=== | | ===Complications=== |
| Common complications of focal segmental glomerulosclerosis include: | | 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 | | *End stage renal disease |
| *Malnutrition | | *Malnutrition |
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| ===Prognosis=== | | ===Prognosis=== |
| *Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%. | | *There are several clinical and pathological features which predict the outcome. |
| *Depending on the extent of the [tumor/disease progression/etc.] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent. | | *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>: |
| *The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
| | **Black race |
| *[Subtype of disease/malignancy] is associated with the most favorable prognosis.
| | **Increased degrees of proteinuria |
| *The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
| | **renal insufficiency |
| | | **Increased severity of interstitial fibrosis |
| ==Complications==
| | **Tubular atrophy in biopsy specimens |
| Renal complications and associated cardiovascular morbidity 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>
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| ==Natural History & Prognosis==
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| 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>:
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| *No adequate response to therapy | |
| *Massive or nephrotic-range proteinuria | |
| *Level of serum creatinine > 1.3 mg/dL | |
| *Collapsing variant on morphological appearance | |
| *Presence of tubulointerstitial fibrosis | |
| *Black race | |
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| Inadequate response to therapy is considered the most important predictor of progression to ESRD.<ref name="pmid18813290">{{cite journal| author=Deegens JK, Dijkman HB, Borm GF, Steenbergen EJ, van den Berg JG, Weening JJ et al.| title=Podocyte foot process effacement as a diagnostic tool in focal segmental glomerulosclerosis. | journal=Kidney Int | year= 2008 | volume= 74 | issue= 12 | pages= 1568-76 | pmid=18813290 | doi=10.1038/ki.2008.413 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18813290 }} </ref> 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 approximately 5-23% 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><ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>
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| 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>
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| 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>
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| ==References== | | ==References== |