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A prospective study of collapsing focal segmental glomerulosclerosis
Ramachandran Raja1, Ritambhra Nada2, Ashok K Yadav1
1a Department of Nephrology , Postgraduate Institute of Medical Education and Research , Chandigarh , India ;
Renal Failure
|June 9, 2016
Summary
Collapsing focal segmental glomerulosclerosis (cFSGS) shows poor treatment response and rapid progression to end-stage renal disease (ESRD). The long-term outcomes for cFSGS and therapy-resistant FSGS are similar, indicating a grave prognosis for both conditions.
Area of Science:
- Nephrology
- Pathology
- Clinical Medicine
Background:
- Collapsing focal segmental glomerulosclerosis (cFSGS) is a severe form of kidney disease.
- cFSGS is associated with rapid progression to end-stage renal disease (ESRD).
- Understanding the clinicopathological features and prognosis of cFSGS is crucial for patient management.
Purpose of the Study:
- To evaluate the clinicopathological spectrum of cFSGS.
- To compare the clinical behavior of cFSGS with steroid and tacrolimus (TAC)-resistant noncollapsing FSGS.
- To assess the long-term outcomes and progression to ESRD in cFSGS patients.
Main Methods:
- Retrospective analysis of 22 cFSGS patients (>14 years).
- Comparison with a prospectively followed cohort of 19 steroid and TAC-resistant noncollapsing FSGS cases.
- Histological evaluation including podocyte markers (WT1, PAX, KI67) and assessment of tubulointerstitial changes.
Main Results:
- cFSGS patients exhibited higher serum creatinine and more advanced tubulointerstitial damage.
- Complete remission (13.6%), partial remission (4.5%), steroid resistance (27.3%), ESRD progression (36.4%), and death (18.2%) were observed in cFSGS.
- No significant difference in ESRD progression was found between cFSGS and therapy-resistant FSGS at two years.
Conclusions:
- Glomerular collapse in FSGS is linked to poor treatment response and high ESRD rates.
- The long-term prognosis for cFSGS and steroid/TAC-resistant FSGS is comparable.
- Effective therapeutic strategies for cFSGS remain a significant clinical challenge.
