Glomerular Diseases Flashcards
Function of kidney
- barrier: size and charge
- Filters 120-180L/day of plasma water
inflammation of the capillaries
GN (Glomerular Nephritis)
Attract ____ to the tuft and react with antigens/epitopes to produce more cytokines and proteases causing damage
- neutrophils,
- Macs
- T cells
Persistent GN that worsens renal function is always accompanied by
- interstitial nephritis,
- renal fibrosis
- tubular atrophy
Best histo correlate of GN
TIN
- Asymptomatic, +3-5 RBCs/hpf in a spun first voided urine
- Gross - rare except in IgA nephropathy and sickle cell disease
- R/O atomic lesions esp in men; ddx - BPH, IN, papilary necrosis, stones, cysts, vascular
Hematuria
Sustained: more than 1-2 gm/24 hours
- Seen in some inflammatory glomerular disease
- Proteinuria
- pyuria
- 1-2 gm/day proteinuria,
- hematuria with RBC casts,
- pyuria,
- HPN,
- edema,
- decreasedGFR
ACUTE NEPHRITIC
RPGN + lung hemorrhage (GPS, ANCA, LN, cryoglobulinemia)
PULMONARY-RENAL
- heavy proteinuria,
- HPN,
- high chole,
- hypo alb,
- edema/anasarca,
- mxic hematuria
NEPHROTIC SYNDROME
abnormal or injured BM due to autoimmune response + mxic hematuria, mild
to heavy proteinuria + HPN, variable increase in crea
BASEMENT MEMBRANE
vascular injury producing hematuria and moderate proteinuria
GLOMERULAR-VASCULAR
Done in selected patients, clinically stable, adequate blood clotting parameters and willing and able to receive treatment
ULTRASOUND GUIDED BIOPSY
LIGHT MX
- Hand and E:
- PAS:
- Jones methenamine silver stain:
- Congo red:
- Masson’s trichrome:
- Hand and E: cellularity and architecture
- PAS: CHO moieties
- Jones methenamine silver stain: enhance BM structure
- Congo red: amyloid deposits
- Masson’s trichrome: collagen deposits, degree of glomerulosclerosis and interstitial fibrosis
- prototype for acute endocapillary proliferative GN
- Developed countries: elderly M>F; 2-14 y.o.
- Skin and throat infections with M type streptococci
+ Skin 2-6 weeks after impetigo
+ Pharyngitis 1-3 weeks after infection
POST STREP GN
Renal bx of POST STREP GN:
-hypocellularity (mesangial and endothelial cells),
- PMN infiltrates,
- granular sub endothelial
deposits (IgG, IgM, C3, C4, C5-9),
- sub epithelial deposits (“humps”)
Labs of POST STREP GN:
- nephrotic protenuria
- 1st week (90%): CH50 and C3
- RF (30-40%), cryoglobulins and CiCs ANCA against myeloperoxidase
- Confirmatory:
+ strep culture,
+ ASOT,
+ antiDNAase and antihyaluronidase
Labs of POST STREP GN:
- nephrotic protenuria (20% of adults)
- 1st week (90%): low CH50 and C3
- (+) RF (30-40%), cryoglobulins and CiCs (60-70%) ANCA against myeloperoxidase (10%)
- Confirmatory: (+) strep culture, increased ASOT, + antiDNAase and antihyaluronidase
POST STREP GN
° treatment
- control HPN, edema, dialysis
- Anti strep antibiotic treatment for ALL PATIENTS AND COHABITANTS
- NO role for immunotherapy even with crescents
Complication:
+ untreated,
+ (-) blood cultures
+ right sided endocarditis
Biopsy:
+ sub capsular hemorrhages with “flea bitten” appearance (gross)
+ focal
proliferation around a foci of necrosis with abundant mesangial, sub endothelial,
+ sub epithelial immune deposits of IgG, IgM and C3
Associated findings: embolic infarcts or septic abscesses
ENDOCARDITIS ASSOCIATED GN (SBE)
SBE
- primary TX:
- prognosis:
- GN due to
- primary TX: 4-6 weeks of antibiotics
- prognosis: good renal recovery
- GN due to persistent bacteremia
predominant organism in Immunocompromised adults of GN due to persistent bacteremia
Staph
- deposition of ICs and C’ activation leading damage, leukocyte infiltration, activation of procoagulant factors and release of various cytokines
- Most common clinical sign - proteinuria
- Renal biopsy: ONLY reliable method for ID of morphologic variants
LUPUS NEPHRITIS
WHO CLASSIFICATION - defines clinicopatholoic correlations, prognosticates and forms basis for treatment
recommendations
- Class I:
- Class Il:
+ Both I and Il - - Class Ill -
WHO CLASSIFICATION - defines clinicopatholoic correlations, prognosticates and forms basis for treatment
recommendations
- Class I: normal histology or minimal mesangial deposits
- Class Il: mesangial ICs with mesangial proliferation
+ Both I and Il - minimal renal manifestation with normal renal function; limited to the renal
mesangium; excellent prognosis; NO treatment for nephritis - Class Ill - focal lesions with proliferation and scarring ; most varied course
Treatment: same as class IV
- Global, diffuse proliferative lesions
- Increase in anti dsDNA, low complements, hematuria, RBC casts, proteinuria (50% nephrotic range), HPN, decreased renal function(+) crescents: rapid progressive decline in renal function and worst untreated prognosis
- remission (near normal renal function and < 330 mg/dL/day CHONuria) - with treatment excellent renal outcomes
Class IV
Class IV Tx
- Induction: high dose steroids + cyclophosphamide OR MMF x 2-6 months
- maintenance: lower steroid dose + MMF OR AZA
- sub epithelial deposits; membranous (mixed with proliferative)
- CHONuria (60% nephrotic), predisposed to RVT and other thrombotic complications
- Tx same as class IV
Class V
- > 90% sclerotic, ESRD with IF
- SLE becomes quiescent with renal failure (effect of uremia)
- Allograft survival comparable to others
Class VI
- Lung hemorrhage + GN - GPS (pulmonary renal syndrome)
- Target epitope: alpha 3 NC1 domain of collagen IV
- Younger age group: explosive hemoptysis, sudden fall in Hgb, fever, dyspnea, hematuria
- urgent kidney biopsy - confirm and prognosticate
- (+) antiGBM and anti ANCA against MPO
- Poor prognosis: >50% crescents, advanced fibrosis, crea 5-6 mg?dL, (+) oliguria, need foracute dialysis
- Treatment: 8-10 or less plasmapheresis + oral prep and cyclophosphamide (maintain IS until negative titers)
ACUTE NEPHRITIC SYNDROME (ANTI GBM DISEASE)
- Episodic hematuria associated with mesangial IgA deposition
- Male preponderance, peak 2nd and 3rd decade of life, rare familial clustering
- distinguished from HSP by prominent systemic symptoms, younger age, (<20 y.o.), preceding infection and abdominal complaints
- Mesangial IgA deposits
- 2 common presentations:
+ recurrent episodes of macro hematuria during or immediately following an URTIi often accompanied by proteinuria
+ persistent asymptomatic mxic hematuria
ACUTE NEPHRITIC SYNDROME (IgA NEPHROPATHY)
