FSGS
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Transcript of FSGS
Focal segmental GN
► Most common progressive renal disease in children
► 20 – 25 % in adults► Primary & secondary ► Acute NS presentation.
►Secondary have slowly progressing non nephrotic range proteinuria & renal insufficiency.
►Diffuse foot processes fusion in primary type. focal & limited to sclerotic area in the secondary type.
►Recurrence in 20 – 40 % RXT
Etiological classification
► Primary idiopathic FSGS.► Familial ► Secondary to hereditary
nephropathies► HIV associated nephropathy ► Heroin associated nephropathy► Secondary FSGS
Tip lesion in FSGS
The lesion is towards the proximal tubules pole of the glomerulus with foamy cells in the injured area.
Tip lesion
Secondary FSGS
► Secondary to reduced renal mass
* oligomeganephronia** reflux nephropathy *** renal dysplaia
► Secondary to glomerular adaptation * Diabetic.N** morbid obesity *** sickle cell disease**** pre-eclampsia
► Circulating factor implicated in the pathogenesis 30 – 50 KD
► Glomerular hypertrophy ( Fego et al)
Familial
► Genetic mutation in memb proteins► Podocin , actinin4, CD2 assosciated
protein ,NPHS2 & wilm`s tumor gene.► Steroid resistance ► No recurrence after RXT
Secondary FSGS
► Reduced No or increased stress on nephrons lead to compensatory intraglomerular HTN & hypertrophy in the rest of glomeruli.
► Glom cell proliferation,macrophage, extra cellular matrix components.
► Transforming growth factor TGFb► >50% nephron mass loss is required
Pathology
► Area of solidification in the perihilar region in continuity with the vascular pole
► Collapse & wrinkling of the CW with obliteration of capillary lumens.
► Adjacent podocytes are prominent ► Oesinophylic material with in the
Capillary loop ( hyalinosis lesion)► Foam cells
Sub endothelial hyaline material deposits
Variants ► FSGS other wise unclassified► Cellular type:
More sever & have poor prognosis► Collapsing type :
HIV Parvovirus infection Alendronate.
► Tip lesion :The segmental lesions occur exclusively at the tubular part, suggesting better response to steroids .
Collapsed capillary loops
Crowded cells in the remaining open loops
Widened Bowman’s capsule space
Capillary loops
Intra-endothelial tubuloreticular structure pathgnomonic of HIV infection FSGS
FSGS and proteinuria
Prognosis is rather poor in FSGS in relation to proteinuria presentation
Treatment
► Spontaneous remission is rare.► Prognosis is poor if :
1- not treated2- S.Cr>1.3mg/dl3- histological abnormalities outside the glomeruli4- interstitial fibrosis at presentation
Corticosteroids
► I mg/kg/day for 2 – 3 /12 then taper & continue for 5 – 9 month.
►Other therapies : Calcineurin inhibitor :
** 39% of children & 21% of adults could enter complete remission within 3/12 using cyclosporine.** normal RF,diffuse GS or interstitial F & at adose < 5mg/kg/day
Stop if level of Cr increased by more than 30% for 1/12
Alkylating agents
1. Cyclophosphamide 2 mg/kg/day Or Chlorambucil 0.1 – 0.2 mg/kg/dayfor 2 – 3 month usually with steroids lead to reestablishment of remission in steroids dependent Pt >70%
Purine synth. Inhibitors
► There is insufficient information supporting their beneficial effects.
► Aphoresis - immunoadsorption Found to be effective ► Pefloxacin 200 – 400 twice a day for 4 – 8 /52► Immunomodulator ► Vit E 200 IU twice a day for 3/12