Radhakrishnan Addl Professor Dept Of Pathology The main clinical renal syndromes Acute Nephritic syndrome Nephrotic syndrome Asymptomatic hematuria proteinuria or both Acute renal failure ID: 1038889
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1. Kidney diseasesDr.Manoj RadhakrishnanAddl ProfessorDept. Of Pathology
2. The main clinical renal syndromesAcute Nephritic syndromeNephrotic syndromeAsymptomatic hematuria, proteinuria, or bothAcute renal failureChronic renal failureRenal tubular defectsUrinary infectionsObstructive uropathyUrinary stonestumors
3. Principal laboratory findings in Uremia(end-stage kidney disease)Azotemia(incrsd. BUN blood urea nitrogen, & serum creatinine)Electrolyte abnormalities(retention of sodium, potassium and phosphate with secondary changes in calcium)AcidosisAnemiaProlonged bleeding time
4. Acute nephritic syndromeTypically presents 1-2 weeks after an upper respiratory tract infection caused by streptococciOliguria : due to reduced GFRHematuria : due to damaged GBM, brownish- red urine, RBCs in urine sedimentProtienuria : increased permeability of GBM, maybe mild or severeGeneralized edema: “ puffy eyes” due to hypo albuminemiaHypertension : reduced GFR leads to secretion of reninLow complement levels in blood: immune complexes bind the complementAzotemia :high bld urea & serum creatinine
5. Nephrotic syndromeProtienuria : massive > 3.5 g/dayThere is hypoalbuminemiaGeneralized edema : due to reduced oncotic pressure (hypoalbuminemia)Hyperlipidemia(increased LDL, lipid casts in urineAffected patients are prone to infection & thrombotic events because of increased urinary loss of serum protiens
6. Derangements of urine volumeAnuria : reduced urine output (< 100 ml urine per day ) reflecting renal injuryOliguria : reduced urine output below 400 ml / day sign of renal failurePolyuria: increased volume of urine ( > 3 L of urine per day) may result from excessive fluid intake, osmotic diuresis( eg Diabetes insipidus) or impaired tubular concentration (eg tubular necrosis)
7. Features of Acute renal failureIs characterised most often b reversible detrioration of renal functionOliguriaAzotemia ( increased Blood Urea, Serum Creatinine)Electrolyte disturbancesMost patients recover without dialysis
8. Chronic renal failureDevelops insidiously in stagesDiminished renal reserve : develop high urea, creatinine levels during inter-current illnessRenal insufficiency : GFR ( 20% - 50% of normal)azotemia anemia, polyuriaRenal failure : (<20% of normal GFR) & edema metabolic acidosis hypocalcemia, and multi-sstem signs of uremiaEnd-stage renal failure : <5% GFR , clinical signs of uremia
9. Features of urinary tract InfectionCharcterised by bacteriuria & pyuria>100,000 bacteria/ml of cultured urine> 10 wbcs /HPF in urine sampleLeucocytes are counted in urinary sediment Signs of urinary irritation- urgency, pain
10. Antibody associated glomerular InjuryAntibodies to endogenous antigens of the GBM : This mechanism accounts for the renal inury in Goodpasture syndrome a disease caused by antibodies to collagen type IVAntibodies to non-glomerular antigens : Antigen-antibody complexes found in glomeruli may result from two pathogenetic mechanisms.In situ immune complex formation results from the binding of circulating antibodies– seen in PSGN ( post streptococcal GN) where streptococcal antigens are implantedin the GBM during the infectionCirculating immune complexes formed from soluble antigens and corresponding Ab – eg seen in SLE
11. PSGNGroup A beta- hemolytic streptococci ( streptococcus pyogenes) account for 90% of all GN casesTypically occurs 1-4 weeks after a strep throat infection or skin Infection(Impetigo)Occasionally same clinical-pathological findings may follow staphlococcal infection or even some viral diseases –HBV, HCV or HIV Infection.
12. PSGNThe increase in cellularity is due to increasi in epithelial endothelial and mesangial cellsAs well as neutrophils in and around capillary loops.
13. PSGNShowing increased number of neutrophils , in post-infectious GN
14. sub-epithelial humps in acute PSGNRepresent epimembranous deposits of immune complexes on the GBM, seen under Electron microscope.Immunofluorescence shows many other immune complexes not dense enough to be seen by EMSmaller immune complexes in mesangium
15. Clinical features of acute PSGN(nephritic syndrome)Childhood diseasePresents with fever, nausea, oliguria,hematuria,RBC casts in urine, mild protienuria(usually less than < 1 g/ day) peri-orbital edema and mild to moderate HTAs immune complex deposition is assoc. with complement activation C3 is the complement consumed in both classical and alternate complement pathway. Hence there will be hypo-complementemia
16. Outcome & long term consequences of acute PSGN CHILDREN90% recover within 2-3 months with conservative therapy aimed at maintaining sodium & water balance.5-8% have persistent GN, in much milder form with abnormal urinary findings for 6-8 months.<1% cases develop Rapidly progressive GN (RPGN)ADULTS60% recover promptly3-5% develop RPGN
17. Rapidly progressive glomerulonephritis is reflective of advaned renal pathologyMostly caused by immunologic mechanismsIs assoc. with 25% mortality & 40 % progression to chronic end- stage kidney disease3 groupsA.N.C.A + Cases(Anti-neutrophil cytoplasmic Ab) , most Adult cases , Wegener’s granulomatosis, P.A.NImmune complex mediated – most common form seen in children & young adults < 20 years – seen in post-infectious GN(PSGN) , SLEAnti-GBM Ab – Good Pasteur's syndrome
18. RPGN or crescentic GNCrescents are found in more than 50 % of glomeruli represent a sin of severe glomerular injuryUsually starts as focal and segmental necrosis – resulting inrupture of the GBM which allows the entry of inlammatory cells and fibrinogen into theurinary space
19. Crescentic GNEarly crescents are composed of fibrin , inflammatory cells exudated into the urinary spaces and proliferated epithelial cells of the bowman’s capsule later these early crescents may get fully fibrotic
20. Nephrotic syndromeProtienuria : massive > 3.5 g/dayThere is hypoalbuminemiaGeneralized edema : due to reduced oncotic pressure (hypoalbuminemia)Hyperlipidemia(increased LDL, lipid casts in urineAffected patients are prone to infection & thrombotic events because of increased urinary loss of serum protiens
21. Differences between Nephrotic syndrome & Nephritic syndromeNephritic syndromeProtienuria ++ to +++Hypoalbuminemia +Edema + to ++Hematuria ++ and RBC castsOliguria ++++No Hyperlipidemia No Lipiduria Hypertension +Nephrotic syndromeProtienuria in nephrotic range(> 3.5 g/dl)Hypoalbuminemia +++Edema + to ++No hematuriaNo OliguriaHyperlipidemiaLipiduriaNo Hypertension
22. Membranous NephropathyIs the most common primary cause of Nephrotic syndrome in AdultsEtiol.: Immune complex deposition in glomeruliPrimary – Idiopathic ,unknownSecondary– prolonged antigenemia- SLE, chronic viral hepatitis B & C, cancer ptsPathology : Diffuse thickening of BM, but no proliferationNo response to steroids Progresses to end stage renal failure over 10-15 years
23. Minimal change disease(Lipoid nephrosis)Most common cause of nephrotic syndrome in children ( 90 % cases < 5, 50 % of < 10 yrs)Unknown etiology related to Type IV hypersensitivityEM scopy shows loss of foot processes of epithelial cellsResponds to steroid therapy
24. How does SLE affect the kidneys?Signs of renal disease are found in 50 % of patients at the time of diagnosis80% will develop some signs of renal disease at some time during the life span.SLE is an immune complex mediated disease, immune complexes are deposited in Glomeruli : most frequently affected part, Immune complexes maybe trapped on the sub-endothelial side, inside the GBM, or on the sub epithelial side & mesangium basement membranes lined with sub-endothelial deposits appear thickened , called wireloopsBlood vesselsTubules & interstitial spaces
25. Classification/ stages of lupus nephritis ( SLE)Class I Class-II-mesangial GNClass III- Focal proliferative GNClass IV- diffuse proliferative GNClass V- membranous GNClass VI- Chronic GNNo depositsMesangial deposists , mild hematuriaMesangial & sub-endothelial moderate GNwidespread deposits,severe GNSubepithelial- nephrotic syndromeChronic GN