Focal segmental glomerulosclerosis natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Several clinical and pathological factors are associated with poor renal outcome. The most important predictor of spontaneous remission or progression of FSGS into ESRD is the quantification of proteinuria. 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.
Complications
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).[1]
Natural History & Prognosis
The most important poor prognostic factors in focal segmental glomerulosclerosis (FSGS) are[2]:
- 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.[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 less than 6% of patients, the degree of proteinuria may predict the probability of spontaneous remission.[1]
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.[3] 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) - ↑ 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.