L3. Glomerular diseases I: Glomerulopathies Flashcards
MINIMAL CHANGE GLOMERULOPATHY
Age range
Pathology
Presnetation
Treatment
MINIMAL CHANGE GLOMERULOPATHY
Age range: 4-8 yo. Occurs also in adults
Pathology: LM and IF normal. EM –> podocyte foot process effacement
Presnetation: Edema but normal renal function. Protein loss –> small MW = albumin mainly. Associated w/certain drugs (NSAID) and tumors (Hodgkin lymphoma)
Treatment: Respond to steroids. Relapse are common
FOCAL SEGMENTAL GLOMERULOSCLEROSIS (FSGS)
Age grp
Ethnic grp
Pathology
Presentation
Pathogenesis
Prognosis
FOCAL SEGMENTAL GLOMERULOSCLEROSIS (FSGS)
Age grp: all grp
Ethnic grp: AA
Pathology: LM diagnosis. Distinct variant histologic lesions
Presentation: hypertension, mild hematuria, elevated creatinine
Pathogenesis: Podocyte disease –> Mutations of genes in the slit diaphragm. Podocyte loss and synechial ahdesion –> develops into scar –>capillary collapse, hyalinosis and sclerosis
Prognosis: progressive disease may respond to steroid and cytokine
Most common cause of nephrotic syndrome in all age grp of African Americans
FSGS
Explain the cause of tubule atrophy and interstitial fibrosis
Damage loops –> glomerular filtrate goes into paraglomerular space –> tubule degeneration and interstitial fibrosis –> tubule atrophy and interstitial fibrosis
Which is the worst prognosis of the FSGS variants and describe its epidemiology
Collapsing variant
Black racial predominance
Younger adults
Typical in HIV nephropathy
May occur w/ drugs
MEMBRANOUS GLOMERULOPATHY
Age grp
Ethnicity
Presentation
Forms
Pathology
Pathogenesis
Prognosis
Age grp: 4th - 5th decade
Ethnicity: caucasian
Presentation: insidious onset of nephrotic syndrome, normal renal fucntion and blood pressure
Forms: Primary –> most common. Secondary –> lupus, carcinomas, hepatitis B and gold/penicillin therapy
Pathology: LM–> thickened capillary wall w/ spikes. IF –> granular capillary wall IgG and C3. EM–> Subepithelial deposits
Pathogenesis: Intrinsic podocyte Ag (PLA2R) –> immune complex
Prognosis: 10 yrs 1/3 remission, 1/3 persistent, 1/3 ESRD
Explain the progression of EM alteration in GMB of membranous glomerulopathy
I : Focal loss of foot prcesse + subepithelial deposists
II : Remodeling of GBM pretrude btwn deposites
III: GMB becomes thickened and incorporates the deposits
IV: Thicken GBM but abnormally permeable
How do we treat membranous glomerulopathy. How does it work
Rituximab (Anti-B cell monoclonal Ab) –> depletion of anti-PLA2R Ab
ALPORT SYNDROME
Age grp:
Genetic forms:
Presentation:
Pathology:
Pathogenesis:
Prognosis:
Age grp: Childhood, male
Genetic forms: X-linked (80-85%), Auto. dominant (5%) and Auto. recessive (15%)
Presentation: defness, ocular abnormalities, anterior lenticonus
Pathology: LM –> non-specific w/ progressive glomerulosclerosis. IF –> negative. EM –> GBM thinning. Thickening and lamellation of GBM
Pathogenesis: X-linked –> mutation in alpha5 (IV) chain of collagen. AR –> alpha-3 and alpha-4
Prognosis: ESRD
Describe the reactivity of Goodpasture syndrome plasma w. normal kiney and Alport syndrom kidney. And its significance
Plasma of patients with Goodpasture syndrome contain anti- GBM antibodies that bind the GBM of normal kidneys but fail to react with the GBM of Alport syndrome kidneys.
Describe the molecular meshwork of GBM
collagen type IV protomers interact with laminin (Lm), entactin (En) and the heparan sulate proteoglycans agrin and perlecan (Perl).
What is the IF pattern of X-linked females in Alport syndrome
They show a mosaic expression of alpha-3, -4, -5
