Nephrotic : Nephritic Syndrome, Lecture notes of Pathology

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PATHOLOGY OF THE NEPHROTIC, NEPHRITIC AND CHRONIC^ LECTURES THREE AND FOUR

DR. AMMAR AL RIKABI, PROFESSOR M.O. ALSOHAIBANI AND DR. HALA KFOURY^ KIDNEY DISEASE^ BY

DEPARTMENT OF PATHOLOGY KING KHALID UNIVERSITY HOSPITAL

Contents: - Evaluation of glomerular disease (Terminology & Techniques).

• Glomerular Diseases:
• Renal Failure. - Azotemia.

1 |^ Nephro'c Syndrome: Minimal Change Disease. (^2) Golmerulosclerosis| Focal Segmental 3 | Membranous 4 | 65 (^) | Lupus Nephropathy.^ Gomerulonephri'sDiabe'c Nephropathy.| Renal Amyloidosis.^.

Nephri'c Syndrome: 21 Glomerulonephri's.| Rapidly Progressive| Posstreptococcal 3 | Good Pasture Syndrome.^ Glomerulonephri's.

Other Glomerular Diseases: 2 | Membranoprolifera've (^1) Glomerulonephri's.| IgA nephropathy.

• è è - Important pointsExplanationFemale NotesMale Notes

TYPE A] Disorders manifest by the nephrotic Minimal change disease (lipid nephrosis) syndrome (^) MORPHOLOGIC FINDINGS Focal segmental glomerulosclerosis Membranous glomerulonephritis Diabetic nephropathy Renal amyloidosis Lupus nephropathy

No visible basement membrane changes; foot process No visible basement membrane changes; sclerosis of scattered juxtamedullaryglomeurli.; lipid accumulation in renal tubular cells. fused epithelialsegmental Basement membrane markedly thickened by intramembranous and epimembranous (subepithelial) immune complex deposits; granular immunofluorescence, "spike and dome" appearance. Basement membrane markedly thickened; diffuse or nodular mesangial membrane-like material. Amyloid protein identified by special stains (e.g. Red microscopy "criss-cross" fibrillary pattern.) with birefringence under polarized light, or electron accumulations of basement Congo B] Disorders manifest by the nephritic syndrome^ Immune complex deposition in subendothelial location^ may manifest as membranous glomerulonephritis. Post-streptococcal glomerulonephritis rapidly progressive (crescentic) glomerulonephritis Goodpasture syndrome Alport syndrome

Subepithelial electron-dense "humps"; lumpy-bumpy" immunofluorescence, crescents formation, antineutrophil cytoplasmic antibody (ANCA) - negative forms with immune complexes or antiglomerular basement membrane antibodies; ANCA-positive (pauci-immune) form with Wegener granulomatosis. Linear immunofluorescence antibody deposition caused by antiglomerular basement membrane antibodies. Split basement membrane due to hereditary nephritis. C] Other glomerular disorders IgA nephropathy (Berger disease) Membranoproliferative glomerulonephritis Mesangial IgA deposits. Tram-track appearance; deposits of C3 and dense deposits in one variant.

Prognosis: - - NotMay azotemia. good.develop

• Renal Failure

è^ General Informations: (^) 1) 2) 3) (^3) LightElectronImmunofluorescence. kinds dealing Microscopy. of Microscopy. Investigations with glomerular should disease: be done when^ LIGHT MICROSCOPE

• Minimal 1. !!! “lipid 2. LipoidMinimal(Sometimes vacuole”.Change Nephrosis. Glomerulosclerosis. theDisease renal is tubule also called:appear vaculated
• MCD 1. 2. Most &Gotis young a (^) an (^) commonvery excellentadult. important cause prognosis.for disease nephrosis for in 2 reasons:children!!
• • BeeProteinuria: !!!!!!!!!!! - - Selectivenon-selective stings could è Albuminleadè Albumin to loss.MCD. & Globulin loss. change disease but occurs in somewhat older patients. It is characterized by juxtamedullary glomeruli with^ ocal segmental glomerulosclerosis sclerosis within capillary tufts of the deep focal or segmental^ is clinically similar to minimal distribution. è^ F 1.^ 2.^ Causes:Focal distribution is involvement of some, but not all of the^ glomeruli.Segmental distribution is involvement of only a part of the^ glomerulus.

1. 2. • • (^) FSGS likes to affect the juxtamedullary nephron. (^) HIV.primary (Idiopathic): very common.Secondary: could be due to: - IgA nephropathy.

• 1. 2. Under L.M. Hyalinization and fibrosis to the blood vessels.↑ in the mesangial matrix.

3. 4. Collapse of part of the glomeruli.Adhesions between the affected segment and bowman’s capsule, called “synechiae”.

• • IgM etiology isn’t immunological, but these substances are entrapped. E.M./ Affected foot processes of podocytes.Immunefluorescence/ May find and C₃ deposits, although the

iabetic nephropathy (1) syndrome. (2) thickness of the glomerular basement membrane. Thickening ofOften, this disease is clinically manifested by the nephrotic Electron microscopy demonstrates striking increase in(Can cause nephritic syndrome). vascular basement membranes observable by electron microscopy is one of the earliest morphologic changes in diabetes mellitus (3) morphologic patterns: An increase in (a) Diffuse glomerulosclerosis. mesangial matrix is marked by a diffusely results in two characteristic distributed increase in mesangial matrix. Failure. (b) accumulations of mesangial matrix material (Kimmelstiel- Wilson nodules). Nodular glomerulosclerosis is marked by nodular May develop renal è è 1. Proteinuria. Associated with retinopathy.Clinical Presentation: 2. Tubulo-­‐interstial ?ibrosis.

LIGHT MICROSCOPE

• of 1. 2. Renal (^) Necrotizing Papillitis.Pyelonephritis.diabetes complications militus?

3. Diabetic Nephropathy.

NephropathyDiabetic

D

(1) (2) and mesangial ( syndrome.This condition is another cause of the nephrotic Predominantly subendothelial (^ enal amyloidosis Kidney) amyloid depositsBlood vessels are ) characteristic. (3) of amyloid with special stains [e.g. stain the amyloid in brown, Apple green violet, thioflavin T) and by birefringence under polarized light. It is also demonstrated by a The amyloidosis can be identified by reactivity Congo Red () , crystalwill characteristic criss-cross fibrillary pattern of amyloid by electron microscopy. (4) inflammatory diseases arthritis or plasma cell tumours such as myeloma Most often, there are associations with. , such as rheumatoid multiple chronic

• • Acute Phase Protein? is a protein secreted from the liver, but during chronic inflammation.Light chain of an antibody can cause amyloidosis. upus nephropathy = Lupus nephritis (a) This is the renal component of SLE; the severity of the renal lesion often determines the overall prognosis in patients with SLE. It is often manifest as the nephrotic syndrome but many cases also have major nephritic features.(It can cause nephrotic or nephritic syndrome The pathogenesis of all forms of glomerulonephritis in SLE involves deposition of within the glomeruli. This causes an). DNA and anti DNA complexes inflammatory responses that may cause proliferation of the endothelial, mesangial and/or epithelial glomerular cells and in severe cases necrosis of the glomeruli. The World Health Organization has divided Class one Class two^ SLE glomerular disease into five classes::: immunofluorescence microscopyc.(This is seen in less than 5% ofMesangial lupus glomerulonephritisNormal by light, electron and SLE patients is). seen in 10 to 25% of cases and is

Class three:^ mesangium. Focal proliferative lupus^ immune complex deposits in the^ clinical symptoms and^ associated with mild glomerulonephritis of patients. Here one or two foci within an otherwise normal glomerulus show swelling and proliferation of endothelial and mesangial cells with neutrophilic infiltration or fibrinoid deposits and is seen in 20 to 35% Class four: Is^ capillary thrombi.^ complementemia glomerulonephritis to 60% of SLE patients. The diffuse proliferative^ è and is seen in 35%^ low histological features are similar to the one described in class 3 but are more diffuse. In this condition, immune complexes thickening of the capillary walls which resemble rigid "wire loops" on light deposition create an overall Class five: Is^ microscopy. glomerulonephritis of cases. In class 5, the patients have severe membranous lupus nephrotic syndrome^ (got nephritic syndrome). occurs in 10 to 15% and there is thickening of the capillary walls due to deposition of basement membrane like material as well as immune complexes. develop renal failure). (The worst of all, can

R

L

Wegener’s granulomatosis

Anti-GBM antibody- mediated glomerulonephritis

syndrome^ Alport

disease, affects^ Is an X-­‐linked mostly boys.