Renal Pathology 2: Hematuria and Nephrotic Syndrome

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Questions and Answers

In a patient presenting with macroscopic hematuria without associated pain, which of the following potential etiologies should be prioritized in the initial diagnostic workup?

  • Benign prostatic hyperplasia
  • Acute urinary tract infection
  • Ureteral calculi
  • Urothelial carcinoma of the bladder (correct)

A microscopic finding of red cell casts in urine sediment strongly suggests what location of hemorrhage?

  • Ureter
  • Renal vasculature excluding glomeruli
  • Lower urinary tract
  • Glomerular (correct)

A 62-year-old male presents with hematuria, lower back pain, and a palpable abdominal mass. Imaging reveals a large staghorn calculus. Which of the following best describes the calculus and its complications?

  • Composed of calcium oxalate, associated with high dietary oxalate intake, rarely obstructs
  • Composed of uric acid, typically causing intermittent hematuria
  • Composed of struvite, associated with recurrent UTIs, and may cause obstruction (correct)
  • Composed of cystine, associated with cystinuria, typically causing flank pain only

What systemic condition would be most likely to present with hematuria due to its direct impact on the integrity of the glomerular filtration barrier?

<p>Systemic vasculitis (C)</p> Signup and view all the answers

Which of the following etiological classifications of IgA glomerulonephritis best describes the condition's primary pathophysiology regarding IgA deposition?

<p>Type III Hypersensitivity Reaction (D)</p> Signup and view all the answers

If a renal biopsy of a patient with IgA glomerulonephritis reveals a significant increase in mesangial cellularity and endocapillary inflammation, according to the Oxford classification, what would these features primarily influence regarding patient management?

<p>Selection of immunosuppressive therapy intensity (A)</p> Signup and view all the answers

A 25-year-old male with Alport's syndrome is considering fatherhood. Given the inheritance pattern, what is the most accurate genetic counseling information you can provide?

<p>Each daughter has a 50% chance of being a carrier, and each son has a 50% chance of being affected. (B)</p> Signup and view all the answers

Which of the following electron microscopy findings is most consistent with Alport's Syndrome?

<p>Lamellation of the glomerular basement membrane (D)</p> Signup and view all the answers

In ANCA-associated vasculitis, which pathogenic mechanism directly leads to glomerular injury and subsequent hematuria?

<p>Neutrophil activation and release of lytic enzymes. (B)</p> Signup and view all the answers

An individual with ANCA-associated vasculitis presents with both hemoptysis and hematuria. Which diagnosis is most likely?

<p>Goodpasture syndrome (B)</p> Signup and view all the answers

What is the primary target antigen in Goodpasture syndrome?

<p>Type IV collagen in the glomerular basement membrane (C)</p> Signup and view all the answers

A patient with hematuria and pulmonary hemorrhage is suspected of having Goodpasture syndrome. Which of the following laboratory findings would best support this diagnosis?

<p>Positive anti-glomerular basement membrane (anti-GBM) antibodies (C)</p> Signup and view all the answers

A patient presents with nephrotic syndrome, including significant proteinuria, edema, and hyperlipidemia. This clinical presentation is most directly caused by damage to what?

<p>Visceral epithelial cells (A)</p> Signup and view all the answers

Which of the following clinical findings in a patient with nephrotic syndrome would most strongly suggest the presence of an underlying glomerular inflammation?

<p>Elevated creatinine levels with blood in urine (B)</p> Signup and view all the answers

A 6-year-old child presents with nephrotic syndrome following an upper respiratory infection. Initial investigations, including light microscopy, show normal glomeruli. What is the most likely diagnosis?

<p>Minimal change disease (A)</p> Signup and view all the answers

What specific ultrastructural finding confirms the diagnosis for minimal change disease?

<p>Diffuse effacement (flattening) of podocyte foot processes (B)</p> Signup and view all the answers

A kidney biopsy from a patient with nephrotic syndrome shows focal and segmental glomerulosclerosis (FSGS). Which clinical feature is most critical in determining the subtype and guiding subsequent treatment?

<p>Response to steroid therapy (B)</p> Signup and view all the answers

A patient diagnosed with steroid-resistant FSGS is found to have a mutation affecting nephrin. Which mechanism is most likely responsible for the patient's condition?

<p>Abnormality/absence of foot process protein (A)</p> Signup and view all the answers

What is the primary antigen targeted in most cases of membranous glomerulonephritis?

<p>Phospholipase A2 receptor (PLA2R) (C)</p> Signup and view all the answers

A patient with nephrotic syndrome has a renal biopsy that shows thickened capillary loops with 'spikes' on silver stain. Immunofluorescence reveals granular IgG deposits. What is the likely diagnosis?

<p>Membranous glomerulonephritis (B)</p> Signup and view all the answers

Where do fibrils primarily deposit in the kidney in cases associated with amyloidosis?

<p>Mesangium, capillary loops, arterioles, and interstitium (A)</p> Signup and view all the answers

Which underlying condition is most commonly associated with amyloidosis causing nephrotic syndrome?

<p>Multiple myeloma/plasma cell dyscrasia (A)</p> Signup and view all the answers

What microscopic finding on a kidney biopsy would most strongly suggest a diagnosis of amyloidosis?

<p>Extracellular eosinophilic deposits with apple-green birefringence under polarized light after Congo red staining (B)</p> Signup and view all the answers

A patient with IgA nephropathy develops rapidly progressive glomerulonephritis (RPGN). What additional pathological finding would you expect to see?

<p>Crescents (D)</p> Signup and view all the answers

The presence of crescents in a kidney biopsy from a patient is indicative of?

<p>ANCA-associated vasculitis (C)</p> Signup and view all the answers

Segmental sclerosis observed in a glomerulus indicates which pathological condition?

<p>Focal Segmental Glomerulosclerosis (A)</p> Signup and view all the answers

In a patient with pulmonary-renal syndrome, which of the following antibodies are most specific to Goodpasture syndrome?

<p>Anti-glomerular basement membrane (GBM) antibodies (C)</p> Signup and view all the answers

Which of the following conditions commonly presents with a combination of lung hemorrhage and glomerulonephritis due to antibody-mediated injury?

<p>Goodpasture syndrome (C)</p> Signup and view all the answers

Anti-GBM disease results in the activation of PMN (polymorphonuclear neutrophils) leading to glomerular injury and subsequent hematuria. What is the process that contributes to activation of PMNs?

<p>Complement activation and release of chemotactic factors (A)</p> Signup and view all the answers

In minimal change disease, why is the kidney biopsy often normal when viewed under light microscopy?

<p>The glomerular damage is subtle and not detectable by light microscopy. (B)</p> Signup and view all the answers

In which of the following glomerular diseases are 'spikes' observed on silver staining due to new basement membrane formation between subepithelial deposits?

<p>Membranous glomerulonephritis (C)</p> Signup and view all the answers

A patient presents with nephrotic syndrome and is diagnosed with FSGS. Which of the following best describes the long-term prognosis for patients with focal segmental glomerulosclerosis (FSGS)?

<p>FSGS recurs in the absence of treatment and leads to ESRD inevitability but this can sometimes be circumvented via transplant (B)</p> Signup and view all the answers

IgA nephropathy is characterized by the deposition of IgA in the mesangium. What diagnostic method is best suited to visualize these deposits?

<p>Direct immunofluorescence (C)</p> Signup and view all the answers

In a patient with Alport's syndrome, which of the following findings is most likely to be present in addition to hematuria?

<p>Nerve deafness and eye abnormalities (D)</p> Signup and view all the answers

In the context of glomerular diseases causing hematuria, what role do neutrophils play in ANCA-associated vasculitis?

<p>They infiltrate the glomerulus and release enzymes, causing damage. (D)</p> Signup and view all the answers

How does Goodpasture syndrome typically affect the kidneys and lungs?

<p>It results in glomerulonephritis and pulmonary hemorrhage. (C)</p> Signup and view all the answers

Damage to the visceral epithelial cells (podocytes) of the glomerulus leads directly to which of the following?

<p>Proteinuria (C)</p> Signup and view all the answers

A child presents with proteinuria and edema, but the glomerular architecture appears normal on light microscopy. What is the most likely underlying diagnosis?

<p>Minimal change disease (C)</p> Signup and view all the answers

Focal segmental glomerulosclerosis (FSGS) is characterized by sclerosis affecting some glomeruli and parts of each glomerulus, but not all of it. What best characterizes this condition?

<p>Scarring of specific regions within affected glomeruli. (C)</p> Signup and view all the answers

When a patient with proteinuria is diagnosed with Membranous Glomerulonephritis, which receptor is the primary antigen located on Podocytes?

<p>PLA2R (A)</p> Signup and view all the answers

Which immune complex deposition disease presents with severe Nephrotic Syndrome, deposition of fibrils in the mesangium/capillary loops, multiple types, and is commonly due to multiple myeloma/plasma cell dyscrasia?

<p>Amyloid and the kidney (C)</p> Signup and view all the answers

Urothelial carcinoma is a cause of Hematuria associated with the Ureteric, Bladder, and Prostate but NOT Urethral. Which type of carcinoma is specifically likely to be associated with the Ureteric, Bladder and Prostate?

<p>Transitional cell carcinoma (D)</p> Signup and view all the answers

Which cause of Haematuria can be categorized as 'Latrogenic'?

<p>Catheter (B)</p> Signup and view all the answers

The following are causes of haematuria associated with which part of the body; UTI, STD's, Urethral Caruncle?

<p>Urethral (B)</p> Signup and view all the answers

In a patient presenting with both haematuria and proteinuria, which of the following glomerular pathologies is LEAST likely to be associated with a painless presentation?

<p>Alport's syndrome with secondary infections (D)</p> Signup and view all the answers

A 30-year-old male presents with microscopic haematuria detected during a routine medical examination. His renal function is normal, and he has no other symptoms. Given the context alone, which of the following conditions is most likely, needing further investigation?

<p>Thin basement membrane nephropathy (C)</p> Signup and view all the answers

Which of the following conditions is most likely to cause haematuria due to the direct impact on the integrity of the glomerular filtration barrier without significant inflammation?

<p>Thin basement membrane nephropathy (A)</p> Signup and view all the answers

What underlying genetic defect is most likely in a 10-year-old boy presenting with haematuria, sensorineural deafness, and progressive visual impairment?

<p>Mutation in COL4A5 (A)</p> Signup and view all the answers

Which of the following pathological processes is most directly responsible for the glomerular injury observed in ANCA-associated vasculitis?

<p>Antibody-mediated activation of neutrophils causing endothelial damage (B)</p> Signup and view all the answers

A patient with ANCA-associated vasculitis presents with haematuria and rapidly progressive glomerulonephritis (RPGN). What is the most critical next step in managing this patient?

<p>Performing immediate plasma exchange and immunosuppressive therapy (D)</p> Signup and view all the answers

In Goodpasture syndrome, the disease targets a specific component of the glomerular basement membrane (GBM). What is the antigen?

<p>Type IV collagen (B)</p> Signup and view all the answers

A patient with Goodpasture syndrome presents with haematuria and pulmonary haemorrhage. Which treatment approach would be the most effective in directly addressing the underlying pathological mechanism?

<p>Immunosuppression with corticosteroids, cyclophosphamide, and plasma exchange (D)</p> Signup and view all the answers

A 5-year-old child presents with nephrotic syndrome characterized by edema, proteinuria, and hypoalbuminemia, but without haematuria or hypertension. A kidney biopsy is performed. What is the most likely diagnosis?

<p>Minimal change disease (B)</p> Signup and view all the answers

A patient with minimal change disease is treated with corticosteroids and shows significant improvement in their proteinuria. Which of the following mechanisms is most likely responsible for the therapeutic effect of corticosteroids in this disease?

<p>Modulating T-cell function to reduce podocyte damage (C)</p> Signup and view all the answers

A patient with nephrotic syndrome has a kidney biopsy that shows focal and segmental glomerulosclerosis (FSGS). Which of the following clinical findings would suggest a primary rather than a secondary cause of FSGS?

<p>Rapid onset of nephrotic-range proteinuria (D)</p> Signup and view all the answers

A child with steroid-resistant FSGS is found to have a mutation affecting podocin. Which of the following mechanisms is most likely responsible for the patient's condition?

<p>Disruption of the structural integrity of the slit diaphragm (C)</p> Signup and view all the answers

What is the most commonly implicated antigen targeted by autoantibodies in primary (idiopathic) membranous glomerulonephritis (MGN)?

<p>Phospholipase A2 receptor (PLA2R) (D)</p> Signup and view all the answers

A 55-year-old patient presents with nephrotic syndrome. A kidney biopsy reveals thickened glomerular basement membranes with subepithelial deposits and 'spikes' on silver stain. Immunofluorescence shows granular IgG and C3 deposits. What is the next diagnostic step to evaluate whether this MGN may be secondary to other causes?

<p>Serum protein electrophoresis to exclude a monoclonal gammopathy (C)</p> Signup and view all the answers

In patients with amyloidosis-related nephrotic syndrome, where are the amyloid fibrils primarily deposited within the kidney?

<p>Mesangium and capillary loops (B)</p> Signup and view all the answers

What is the most common underlying systemic condition associated with amyloidosis that leads to nephrotic syndrome?

<p>Multiple myeloma (C)</p> Signup and view all the answers

A patient presents with nephrotic syndrome, and a kidney biopsy shows evidence of amyloid deposition. Which of the following staining techniques is most reliable for confirming the presence and type of amyloid?

<p>Congo red stain with apple-green birefringence under polarized light (D)</p> Signup and view all the answers

A patient with IgA nephropathy develops rapidly progressive glomerulonephritis (RPGN). Which of the following pathological findings would be most indicative of this progression on a kidney biopsy?

<p>Formation of crescents in Bowman's capsule (A)</p> Signup and view all the answers

In a patient with pulmonary-renal syndrome, which of the following autoantibodies is most specifically associated with Goodpasture syndrome alone?

<p>Anti-glomerular basement membrane (anti-GBM) antibody (A)</p> Signup and view all the answers

Which process directly contributes to glomerular injury in Anti-GBM disease ultimately leading to haematuria?

<p>PMN (polymorphonuclear neutrophils) activation (B)</p> Signup and view all the answers

Why are light microscopy results typically normal in cases of minimal change disease?

<p>The abnormalities are so subtle that they are beyond the resolution of light microscopy. (B)</p> Signup and view all the answers

Which immunological complexes result in new basement membrane formation between subepithelial deposits, observed as 'spikes' on silver staining?

<p>Membranous glomerulonephritis (B)</p> Signup and view all the answers

A patient is diagnosed with FSGS. Is the following statement true or false? 'Patients with FSGS typically have a good long-term prognosis, especially if they are steroid-sensitive'.

<p>False, steroid-resistant FSGS often indicates a worse prognosis. (A)</p> Signup and view all the answers

In IgA nephropathy, which diagnostic method is best suited to visualize the IgA in the mesangium?

<p>Direct immunofluorescence microscopy (B)</p> Signup and view all the answers

A young male patient with Alport's syndrome is undergoing genetic counseling. What associated finding is important?

<p>Sensorineural hearing loss (B)</p> Signup and view all the answers

In the setting of glomerular diseases causing haematuria, what is the primary role of neutrophils in ANCA-associated vasculitis?

<p>Causing direct endothelial damage (B)</p> Signup and view all the answers

Damage to the visceral epithelial cells (podocytes) of the glomerulus directly leads to what?

<p>Proteinuria (C)</p> Signup and view all the answers

A person with proteinuria and edema has normal glomerular architecture on light microscopy. What is the most likely diagnosis?

<p>Minimal change disease (D)</p> Signup and view all the answers

Focal segmental glomerulosclerosis (FSGS) is characterised by sclerosis affecting some glomeruli and parts of each glomerulus, but not all of it. What best characterizes FSGS?

<p>Sclerosis affecting some glomeruli and parts of each glomerulus, but not all of it (B)</p> Signup and view all the answers

Which immune complex deposition disease presents with severe Nephrotic Syndrome, deposition of fibrils in the mesangium/capillary loops. What type of amyloid is most frequently found in these cases?

<p>Immunoglobulin light chain (D)</p> Signup and view all the answers

Haematuria can be categorised as 'Latrogenic', which of the examples below is the most relevant?

<p>Catheter (D)</p> Signup and view all the answers

The following can contribute to Haematuria: UTI, STD's, Urethral Caruncle. Where would you typically find these injuries?

<p>Urethra (C)</p> Signup and view all the answers

Flashcards

Haematuria

Blood in the urine, can be microscopic or macroscopic (gross).

Haematuria Classification

A classification method that divides based on the presence or absence of pain and whether it is treated medically or surgically.

Causes of Haematuria

Includes systemic conditions, kidney, ureter, bladder and urethra disorders.

IgA Glomerulonephritis

Glomerulonephritis mostly caused by deposits of IgA in the glomeruli.

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Alport's Syndrome

An inherited condition involving nerve deafness, eye abnormalities, and kidney disease. Commonly X-linked.

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ANCA Vasculitis

A systemic disease caused by anti-neutrophil cytoplasmic autoantibodies. Activates neutrophils, inducing blood vessel wall inflammation.

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Goodpasture Syndrome

A autoimmune disease where autoantibodies attack the glomerular basement membrane, damaging kidneys and lungs..

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ANCA and Anti GBM

This is caused by crossover immunological diseases which include molecular mimicry, induced by Neutrophil activating processes, eg infection or allergy.

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Causes of Haematuria - Ureteric

Urothelial carcinoma, UTI, calculi.

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Causes of Haematuria - Bladder

Urothelial Carcinoma, UTI, Calculi, Latrogenic(Catheter).

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Causes of Haematuria - Urethral

UTI, STD's, Urethral caruncle.

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Causes of Haematuria - Prostatic

BPH, Ca Prostate, Prostatitis.

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Nephrotic Syndrome

A syndrome characterized by proteinuria, oedema, hypoalbuminaemia, and hypercholesterolaemia, without inflammation, raised BP or haematuria.

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Main Causes of Nephrotic Syndrome

Minimal change disease, focal and segmental glomerulosclerosis, membranous glomerulonephritis, and amyloidosis.

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Minimal Change Disease

An inflammatory disease that particularly affects children and elderly where podocyte foot injury can occur. >20g albuminuria/24 hrs. Responds well to steroids, and has full recovery.

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Pathology of Minimal Change Disease

Light microscopy: Normal; F.M: No deposits; Electron Microscopy: Diffuse flattening and simplification of foot processes.

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Focal Segmental Glomerulosclerosis (FSGS)

A kidney disease with nephrotic range proteinuria, and two main types: steroid responsive/dependent and steroid unresponsive,. Some have recirculating factors and some are due to foot process abnormality .

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Membranous Glomerulonephritis

Immune complex deposition disease, caused by antigen phospholipase A2 receptor (PLA2), abundant in foot processes of epithelial cells. Has IgG, and responds well to therapy, and has 3yr recovery.

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Amyloid and the Kidney

Fibrils deposit in mesangium and capillary loops, as well as arterioles and interstitium. Presents with severe NS and some renal function impairment. Multiple Myeloma/plasma cell dyscrasia

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Study Notes

  • Renal Pathology 2 covers glomerular causes of hematuria and nephrotic syndrome.

Clinical Presentation of Haematuria

  • Blood present in the urine is a sign of haematuria.
  • Microscopic haematuria presents as trace amounts of blood.
  • Macroscopic haematuria presents as gross or frank blood.
  • In microscopic hematuria, the urine may appear normal.
  • Red cell casts in urine indicate glomerular bleeding.
  • Blood in the tubule is another sign of hematuria.

Classification and Causes of Haematuria

  • Haematuria can be classified as painless or painful, and medical or surgical.
  • Systemic causes of haematuria can include clotting disorders, low platelets, and warfarin use.
  • Other causes can include kidney, ureter, bladder, and urethra related issues.
  • In the kidneys, glomerulonephritis, IgA nephropathy, acute post-infectious glomerulonephritis, thin membranes, Alport's syndrome, vasculitis, and neoplasms can cause haematuria.
  • Ureteral obstruction can also cause haematuria.

IgA Glomerulonephritis

  • Direct immunofluorescence for IgA shows IgA deposits in the glomeruli.
  • Histological findings in IgA glomerulonephritis include sclerosed glomeruli, enlarged glomeruli with increased mesangial cellularity and segmental sclerosing lesions.
  • Electron microscopy reveals mesangial deposits.

Oxford Classification for IgA

  • The Oxford system is used for IgA classification and prognosis using the acronym "MEST-C"
  • M stands for Mesangial cellularity increase.
  • E stands for Endocapillary inflammation.
  • S stands for Segmental sclerosis.
  • T stands for Tubulo-Interstitial Fibrosis.
  • C stands for Crescents.

Thin Membrane Nephropathy and Alport's Syndrome

  • Thin membrane nephropathy is also known as benign familial haematuria or March haematuria
  • It typically has a benign course with thin membranes
  • Females may be carriers of the Alport's gene.
  • Alport's Syndrome is associated with nerve deafness and eye abnormalities.
  • It is X-linked in 85% of cases.
  • Males typically develop full disease, with 90% developing end-stage kidney disease by age 40.
  • Females can be carriers with thin membranes.
  • Genetic counseling is available for milder forms of the disease

Vasculitis

  • ANCA vasculitis is a systemic disease
  • It is characterized by anti-neutrophil cytoplasmic autoantibodies
  • Neutrophils are activated, with molecules protruding through the PMN cell wall.
  • Antibodies bind to myeloperoxidase or proteinase 3, causing blood vessel wall inflammation.

ANCA & PMN Activation

  • Activated PMNs can create "holes" in the basement membrane.
  • ANCA can present as cytoplasmic or perinuclear patterns.

Goodpasture Syndrome

  • Goodpasture syndrome is an anti-glomerular basement membrane disease involving autoantibodies against the alpha3 chain of collagen type 4.
  • It affects the kidneys and lungs.
  • It also results in PMN activation, similar to ANCA disease.
  • Linear IgG positivity is observed in anti-GBM disease.
  • Rupture of capillary loops can also occur.

ANCA and Anti-GBM

  • ANCA and Anti-GBM diseases involve crossover immunological responses and molecular mimicry.
  • Neutrophil activating processes, such as infection or allergy, may cause them.
  • Antibodies target neutrophil proteins (ANCA) or collagen proteins (Anti-GBM).

Additional Causes of Haematuria

  • Ureteric causes include urothelial carcinoma, urinary tract infections (UTIs), and calculi.
  • Bladder-related causes include: urothelial carcinoma, UTIs, calculi, and iatrogenic causes such as catheter use.
  • Urethral causes include UTIs, STDs, and urethral caruncles.
  • Prostatic causes include BPH, prostate cancer, and prostatitis.

Clinical Presentation of Nephrotic Syndrome

  • Nephrotic syndrome is different from nephritis, due to nephrosis vs nephritis.
  • Absence of inflammation leads to absence of raised blood pressure and haematuria.
  • Pathogenesis involves damage to visceral epithelial cells.

Normal Kidney Ultra Structure

  • Normal filter membrane ultra structure includes parietal/visceral epithetlial cells, BM (basement membrane), Bowman's space, capillary etc

Nephrotic Syndrome: Clinical Features

  • Key clinical features include:
    • Proteinuria (≥ 3.5g/24hrs)
    • Oedema
    • Hypoalbuminaemia
    • Hypercholesterolaemia
    • Thrombophilia

Main Causes of Nephrotic Syndrome

  • Primary causes of nephrotic syndrome
    • Minimal change disease
    • Focal and segmental glomerulosclerosis
    • Membranous glomerulonephritis
    • Amyloidosis
  • Membranoproliferative glomerulonephritis(MPGN) can present with either nephrotic or acute nephritic syndrome

Nephrotic Syndrome with Normal Creatinine and Raised Creatinine: Signficance

  • Clinical features predict the site of the pathology.
  • If the patient presents with nephrotic syndrome with normal creatinine, it may indicate glomerular damage without inflammation.
  • Raised creatinine along with blood in the urine typically indicates glomerular inflammation.

Minimal Change Disease

  • Minimal change disease affects individuals of all ages, especially children and the elderly
  • It leads to acute onset of florid nephrotic syndrome with albuminuria often exceeding 20g/24 hours.
  • Minimal change disease is self-limiting and responds well to steroids, leading to full recovery.
  • It results from direct injury to foot processes by a short term agent that is currently unknown.

Pathology of Minimal Change Disease

  • On light microscopy (LM), the glomeruli appear normal.
  • Immunofluorescence microscopy (F.M.) shows no deposits
  • Electron microscopy shows diffuse flattening and simplification of foot processes.

Focal and Segmental Glomerulosclerosis (FSGS)

  • FSGS always presents with nephrotic range proteinuria
  • There are 2 main types of FSGS
    • Steroid responsive/dependent
    • Steroid unresponsive
  • Lesions are described as diffuse, global, focal, and segmental.

FSGS: Steroid Responsive/Dependent

  • a circulating factor can cause foot process injury
  • Inevitably leads to end-stage kidney disease over several years or decades
  • Likely to come back in transplants due to the presence of the circulating factor even after the transplant.

FSGS: Steroid Unresponsive

  • The condition occurs due to an abnormality/absence of foot process protein.
  • The most profound form is due to nephrin abnormality or Finnish type congenital NS.
  • There have since been dozens of abnormalities found, for which cytogenetic tests are now available.
  • FSGS does not come back in transplants.

Foot process

  • The foot process has many components, an absence of abnormality of which can cause FSGS usually resulting in chronic renal failure.

Membranous Glomerulonephritis

  • Can be described as an immune complex deposition disease
  • It is also a cross over immunological disease
  • The antigen is Phospholipase A2 receptor, PLA2, which is abundant in foot processes of epithelial cells
  • The antibody that is seen is produced as a reaction to different diseases and/or drugs
  • Responds well to theraphy with a 3 years recovery time

Membranous Glomerulonephritis findings

  • Capillary loops in MGN are thickened
  • "Spikes” are new membrane between deposits
  • An abundance of IgG in MGN
  • Deposits on epithelial side of BM in MGN, with Note no inflammation

Amyloid and the Kidney

  • The kidney is often involved.
  • Fibrils deposit in mesangium and capillary loops, as well as arterioles and interstitium.
  • Presents with severe nephrotic syndrome (NS) and some renal function impairment.
  • All types of amyloid can cause kidney damage
  • The most commonly seen is due to multiple myeloma or plasma cell dyscrasia.
  • Amyloid deposits have a waxy, starch like appearance.

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