Glomerular Ds Flashcards
Absolute indication for hemodialysis
Other indication
Uremia
Others ecg change metabolic acidosis fluid overload unresponsive to med mgt
Clinical features of rpgn
Clinical features of rpgn
Progressive facial puffiness pedal edema high colored urine that progress to oliguria htn
Bp 150/100
Biopsy finding of rpgn
Crescent
Collapse of glomeruli
Parietal epithelial proliferation along with fibrin platelet
No of crescent more than size of crescent
Classification of rpgn is done on basis of
Immunofluorscene
Type 1to5
Most common rogn overall
Type 3 >2 >1
Most common rpgn in 1-20 yrs age grp
Type 2
Immune complex deposit
Good pasture syndrome is type of
Type 1 rpgn Linear igG +c3 deposit along capillary wall
SLE MPGN ADULT HSP ADULT PIGN are type of
Type 2 RPGN immune complex deposit
IgG IgA or full house
Type 3 ie pauci immune is found in
Anca vasculitis
Good pasture syndrome consist of
Rpgn
Diffuse alveolar hemorrhage
What is good pasture disease
Rpgn
Dah
Ab against NC1 domain of alpha 3 chain of type 4 collagen
Only glomerular disease which has smoking as risk factor
RPGN- anti GBM disease
Confirmation test for dah (rpgn)
Bal sputum :hemosiderin laden macrophage
Pft increased diffusing capacity of lungs for carbon monoxide
Bronchoscopy blood in air spaces
Most sensitive test for diffuse alveolar hemorrhage
Pft increased dlco
Most specific test for diffuse alveolar hemorrhage
Bal sputum
Hemosiderin laden macrophage
Treatment for good pasture disease
Plasma exchange
Steroids and cyclophosphamide for 3months
Anti gbm disease consists of
Rpgn
DAH
Anti gbm antibody
Type 4 rpgn i e double positive means
Type 1 with anti gbm and anca +
How to detect inheritance of alport syndrome from skin IF
If skin IF Abnormal - defect in alpha 5 i e x linked (80%)
If skin IF normal -defect in alpha 3&4 ie autosomal recessive or dominant
Pathogenesis of alport
Type 4 collagen alpha 1 23 class switch to
Alpha 345 - in glomerlus cochlea ocular bm
Alpha 556 epidermal bm
Amyloid deposit in amyloid kidney is most likely to be
Lambda > kappa
Staining characteristics to comfirm amyloid deposit
Extracellular hyaline amorphous nodule
Weakly pas positive
Congo red positive
Apple green birefringence on polarised microscopy
Polyclonal nodules can be found in
MPGN
Organized mono clonal ig deposit( lambda)
Suspect?
Amyloidosis
Secondary amyloidosis clinical feature are
Nephrotic syndrome and massive proteinuria Autonomic neuropathy Hepatomegaly Peripheral neuropathy Macroglossia
Prognosis of X linked inheritance
Autosomal recessive
Autosomal dominant
X linked in male progresses to esrd
AR severe esrd in both sexes
AD mild ds in both sexes
Manifestation of alport at the age of 5-10 yrs
Proteinuria htn
Start on ace
Manifestation of alport at the age of 5yrs and
At 10 yrs
Microscopic hematuria
Sr Creatinine increased
High or Low frequency sn hearing loss occurs at what age in alport
15 yrs
What are opthalmic manifestation of alport and at what age they appear
Most common dot and fleck retinopathy
Pathognomic anterior lenticonus
At the age of 20 yrs
Can ophthalmoscope be used to find opthalmic manifestation of alport syndrome
Oil drop appearance on fundoscopy
Thin gbm disease
Characteristics
Uniform thinning of GBM
No risk of Ckd
Microhematuria
No extrarenal feature
Basket weaven appearance
Alternating thickening and thinning of gbm
Is seen in
Alports syndrome
Prognosis after transplant in a pt of alports
Zero percent post transplant recurrence
5% post transplant good pasture disease in graft
Most common primary glomerulonephritis
IgA nephropathy
Aka bergers disease henoch scholein nephritis
Innocent bystander theory is wrt which disease
IgA nephropathy
Pathogenesis of IgA nephropathy
Defective galactosylation of polymeroc IgA1 produced by malt Not cleared by liver Antiglycan ab Immune complex Deposit in mesangium Kidney is innocent bystander
Other causes where increased polymeric IgA mucosal production leads to IGA nephropathy
Celiac disease
Whipples
IBS (UC>CD)
Other causes where defective uptake by liver lead to IgA nephropathy
Cirrhosis
Alcohol
NASH
Most common glomerular ds acc with hep B
Membranous nephropathy
1/3rd rule. Of Iga nephropathy
One third spontaneously resolve
“ Stable course with maintained gfr
“ CKD even with t/t
MESTC Score is seen in which glomerular ds
IgA nephropathy
Mesangial hypercellularity Endocapillary hypercellularity Segmental sclerosis Tubular atrophy Crescent formation
Can IgA nephropathy present as nephrotic syndrome or ckd
Less than 5% present as Nephrotic Syndrome (MCD)
Less than 5% as CKD (B/L small kidney)
Prognostic factor of IgA nephropathy
Do serum IgA level and complement levels have no role in prognosis.?
Any deposit other than IgA
IgA deposition in capillary wall
Endocapillary proliferation
No, no role in diagnosis/prognosis
Crescent formation in IgA nephropathy present as?
Less than 1% pt present as RPGN TYPE2
Adult Hsp
Any sign which is responsible for good prognosis in IgA nephropathy
Macrohematuria
Treatment of IgA nephropathy with respect to different presentation
ACEI/ARB
PROTEINURIA<500 mg
Limit salt intake
Steroids-
Acc to clinical presentation steroid intake in pt differs
T/T in nephrotic syndrome
RPGN
Proteinuria more than 1g/day and pt on conservative therapy
A -treat like MCD
B- Steroid+cyclophosphamide
C- Steroid
Causative agent of psgn in childhood and types
Group A Beta hemolytic strep
Type 1 3 4 12 mainly
Type 49 mainly
Incidence of psgn is common in
Immunocompetent children boyrs
2-7 yrs of age
Urinalysis of psgn will show
WBC casts
Detection of toxin in psgn
Name
Streptococcal pyogenic exotoxin B
AntiDNase B titre
Nephritis ass plasminogen receptor (NAPL2R)
Glomerulonephritis ass with MRSA seen in immunocompromised / diabetic foot, cellulitus pt
PIGN -Post infectious
Or
IRGN-Infection related GN
In children presentation of PSGN is mostly
Treatment?
Acute nephritic syndrome
Benzathine penicillin 12 lakh/units IM
Steroid not indicated
Importance of C3 levels in psgn in children
As psgn activate alternate complement pathway C3 levels are high
But after treatment follow up after 8 weeks C3 levels if remain high that means diagnosis was wrong
And biopsy is indicated
In adults PIGN presents as
What is treatment and prognosis
Rapidly progressive renal failure
Treatment dialysis
Prognosis poor as mrsa but vancomycin cannot be given nephrotoxic and steroids or immunosuppressent also cannot be used
Characteristics LM finding of PSGN
DPGN
endocapillary hypercellularity mesangial hypercellularity with neutrophil infiltration
In children DPGN
In adults DPGN +crescent
Electron microscopic finding of psgn
Sub Epithelial camel hump deposit
Lumpy bumpy deposit
Mesangiocapillary glomerulonephritis is other name for
MPGN
What is podocytopathy
Podocyte injury resulting in proteinuria
Seen in MCD FSGS MN
Lipoid nephrosis is other name for
Minimal change disease
Primary causes of MPGN
None it is always secondary
Glomerular ds with extremely high chance for recurrence post transplant
Almost 100% in type 2 MPGN
Types of MPGN
Type 1 classical MPGN
TYPE 2 C3 GN
Most important causes of type 1 MPGN
Infection- HCV, Leprosy, P. Malariae, IE
Malignancies monoclonal Immunoglobulin (plasma cell dyscrasia)
Autoimmune -cryoglobulinemia, sle3&4 , scleroderma, sjogrens syndrome
Complement levels in TYPE 1&2 MPGN
Type 1 C3 &4 levels low– classical pathway
Type 2 C3 low C4 normal – alternate pathway
Type 2 C3 MPGN Characteristic
Partial lipodystrophy of face
Drusen on retina
Aka dense deposit disease intramembranous deposit
Most important manifestation of scleroderma in kidney
Small vessel ds(TMA)
Most important manifestation of sjogrens in kidney
RTA type 1
Light microscopy finding of MPGN
Mesangial Hypercellularity> endocapillary Hypercellularity
Thickening of capillary wall
Lobular capillary tuft
Uniform small nodules
Double contour/Tram track appearance is seen in
Type 1MPGN
Subendothelial deposit
Hyaline thrombi in MPGN is specific to which secondary cause
Cryoglobulinemia
Only glomerular ds which can present as nephrotic and nephritic syndrome
MPGN
35% each
IgG deposit +C3 in Mesangiocapillary wall can be seen in
mpgn
MCD is seen most commonly in
Boys 2-7 yrs
90% of nephrotic syndrome in children are MCD
3yr old boy with proteinuria no hematuria or htn or raised creat in serum
MC diagnosis
MCD
Drugs responsible for MCD
NSAIDs
Interferon alpha
Gold can cause which renal disorder
Membranous nephropathy
Most common malignancy ass c mcd
Hodgkin’s lymphoma
Why is it called MCD
Because LM and IF show no change ie normal
Which type of MCD can show some changes in LM
IgM nephropathy can cause mild mesangial expansion and it can progresa to ckd
EM finding in MCD
Why MCD never progress to CKD
Effacement of foot process of podocyte
Because no of podocyte do not decrease in MCD
Podocyte injury in MCD can be due to
Increased CD80 expression in podocyte
Increase angiopoietin like 4 expression
Specific Mgt of MCD
Oral prednisolone in children and adults
2mg/kg/day children
1mg/kg/day adula
Full dose in morning for 6 wks then taper for another 6 wks
Total duration 6wks
Growth monitoring and urine monitoring
When do we say child with minimal change disease has gone into remission
Urine albumin is nil for 3 consecutive days
10% children are steroid resistant
How to define it
Proteinuria persisiting despite full dose of steroids fo 4 wks in child and 4 month in adult
Treatment for steroid resistant mcd
Calcineurin inhibitors
Tacrolimus or cyclosporin
Leavmisole
Out of 90% responding 2/3 rd relapse -
What is relapse in mcd
Proteinuria reappearing after mini 4 wks of remission
What can be the cause of relapse in minimal change disease
Frequent relapse -more than 2 relapse in 6 months
Steroid dependant nephrotic syndrome
How to treat relapse in minimal change disease
Steroid then urine albumin become nil continue steroid for 2 more days
And start tapering
DOC in frequent relapse in minimal change disease
Cyclophosphamide 2mg/kg for 12 wks
Define steroid dependant nephrotic syndrome
Treatment
And last choice of drug
2 or more episode of proteinuria within 14 days of stopping steroid or while tapering
T/T calcineurin inhibitors
Last choice of drug rituximab
Prognosis of fsgs
2/3rd pt progress to ckd even after treatment
Patho of fsgs
Decrease in podocyte
Synechiae formation
Loss of bowman space
Sclerosis
Types of FSGS with cause
Type 1 primary
Soluble urolinase plasminogen activator receptor (SUPAR) elevated
Type 2 Secondary
Drugs IFNalpha, pamidronate, sirolimus, heroin
Infection parvovirus hiv cmv ebv
Genetic
Characteristics of secondary FSGS
No nephrotic syndrome
Nephrotic range proteinuria and systemic htn
LM fags
EM podocyte effacement<50%
What is adaptive /perihilar fsgs
Causes
When one kidney is diseased and other kidney develops hyperfiltration injury
RAS, REFLUX NEPHROPATHY MALIGNANCY
OBESITY SICKLE CELL ANEMIA
Characteristics of primary fsgs
75% nephrotic syndrome 25% microscopic hematuria proteinuria better prognosis Biopsy IF focal IgM +-C3 EM more than 50% podocyte effaced
Variant of FSGS
Which variant has better prognosis why
Tip variant best prognosis
Similar to MCD
Collapsing variant worst prognosis
CAUSED by hiv heroin pamidronate parvovirus
Mgt of FSGS
Adult with nephrotic syndrome- (75%)
Steroids
1mg/kg/day given for 6-8 months
Adult with asymptomatic presentation(25%)
Acei arb and salt restriction
How to treat resistance in fsgs
Resistance no response after 4 months
Treatment cyclosporin tacrolimus
