Focal segmental glomerulosclerosis natural history, complications and prognosis
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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
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.
Natural History, Complications, and Prognosis
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 (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
- Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
- 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.
- The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
- [Subtype of disease/malignancy] is associated with the most favorable prognosis.
- The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
Natural History & Prognosis
The most important poor prognostic factors in focal segmental glomerulosclerosis (FSGS) are[2]:
- 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
Inadequate response to therapy is considered the most important predictor of progression to ESRD.[3] 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.[1] In converse, patients with non-nephrotic range proteinuria often have a better prognosis, with a 10-year survival reaching > 80%.[1] 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.[1][4]
Serum creatinine > 1.3 mg/dL and interstitial fibrosis > 20% on pathological analysis have been associated with worse renal outcomes.[1] The extent of proteinuria, serum creatinine, and extent of fibrosis on pathological observation have been positively correlated.[1]
While collapsing variant, commonly seen in HIV-induced FSGS, is associated with poorer outcomes, tip variant correlates with better outcomes.[5] According to Korbet and colleagues, the remission of proteinuria is negatively associated with progression to ESRD in FSGS.[1]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Korbet SM (1999). "Clinical picture and outcome of primary focal segmental glomerulosclerosis". Nephrol Dial Transplant. 14 Suppl 3: 68–73. PMID 10382985.
- ↑ Sohal, DS; Prabhakar, SS (November 02, 2011). "Focal segmental glomerulosclerosis" (PDF). Interchopen. InTech. Retrieved 3 December 2013. Check date values in:
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(help) - ↑ Deegens JK, Dijkman HB, Borm GF, Steenbergen EJ, van den Berg JG, Weening JJ; et al. (2008). "Podocyte foot process effacement as a diagnostic tool in focal segmental glomerulosclerosis". Kidney Int. 74 (12): 1568–76. doi:10.1038/ki.2008.413. PMID 18813290.
- ↑ Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH; et al. (2013). "KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis". Am J Kidney Dis. 62 (3): 403–41. doi:10.1053/j.ajkd.2013.06.002. PMID 23871408.
- ↑ Thomas DB, Franceschini N, Hogan SL, Ten Holder S, Jennette CE, Falk RJ; et al. (2006). "Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants". Kidney Int. 69 (5): 920–6. doi:10.1038/sj.ki.5000160. PMID 16518352.