Podcast
Questions and Answers
In the context of glomerular disease, how does the presence of red cell casts in urine specifically contribute to the understanding of the origin and nature of haematuria?
In the context of glomerular disease, how does the presence of red cell casts in urine specifically contribute to the understanding of the origin and nature of haematuria?
- They point towards bleeding from the kidney, but do not provide specific information on the location or cause.
- They indicate bleeding from the lower urinary tract, such as the bladder or urethra.
- They confirm glomerular bleeding, suggesting damage or inflammation within the glomeruli. (correct)
- They suggest bleeding from the renal tubules due to ischemic injury.
Which of the following systemic conditions is least likely to directly cause haematuria through non-renal mechanisms?
Which of the following systemic conditions is least likely to directly cause haematuria through non-renal mechanisms?
- Erythrocyte Sedimentation Rate (ESR) (correct)
- Vitamin K Deficiency
- Severe Thrombocytopenia
- Disseminated Intravascular Coagulation (DIC)
In the evaluation of a patient presenting with haematuria, what is the most critical distinction to make in order to guide initial diagnostic and therapeutic strategies?
In the evaluation of a patient presenting with haematuria, what is the most critical distinction to make in order to guide initial diagnostic and therapeutic strategies?
- Determining if the haematuria is painful or painless. (correct)
- Determining the precise number of red blood cells present per high-power field.
- Differentiating between microscopic and macroscopic haematuria to assess disease severity.
- Identifying the specific type of haemoglobin present in urine.
A patient presents with haematuria, proteinuria, and hypertension. Renal biopsy shows mesangial IgA deposits. Which of the following underlying mechanisms is the least likely contributor to the observed hypertension?
A patient presents with haematuria, proteinuria, and hypertension. Renal biopsy shows mesangial IgA deposits. Which of the following underlying mechanisms is the least likely contributor to the observed hypertension?
A 25-year-old male presents with recurrent episodes of macroscopic haematuria following upper respiratory tract infections. Renal biopsy reveals mesangial IgA deposits. Which of the following is the least likely long-term complication associated with this condition?
A 25-year-old male presents with recurrent episodes of macroscopic haematuria following upper respiratory tract infections. Renal biopsy reveals mesangial IgA deposits. Which of the following is the least likely long-term complication associated with this condition?
A patient diagnosed with IgA nephropathy based on renal biopsy showing mesangial deposits is being evaluated for prognosis. Which of the following histological findings, as per the Oxford classification, would least likely indicate a poor long-term renal outcome?
A patient diagnosed with IgA nephropathy based on renal biopsy showing mesangial deposits is being evaluated for prognosis. Which of the following histological findings, as per the Oxford classification, would least likely indicate a poor long-term renal outcome?
What is the underlying genetic basis and inheritance pattern of Alport syndrome in most cases?
What is the underlying genetic basis and inheritance pattern of Alport syndrome in most cases?
In the context of haematuria, what is a key difference between thin basement membrane nephropathy (TBMN) and Alport syndrome?
In the context of haematuria, what is a key difference between thin basement membrane nephropathy (TBMN) and Alport syndrome?
What primary immunological mechanism differentiates ANCA-associated vasculitis from Goodpasture's disease in the context of glomerulonephritis?
What primary immunological mechanism differentiates ANCA-associated vasculitis from Goodpasture's disease in the context of glomerulonephritis?
In the diagnostic workup of a patient with glomerulonephritis and suspected vasculitis, which of the following is the least helpful in distinguishing between ANCA-associated vasculitis and anti-GBM (Goodpasture's) disease?
In the diagnostic workup of a patient with glomerulonephritis and suspected vasculitis, which of the following is the least helpful in distinguishing between ANCA-associated vasculitis and anti-GBM (Goodpasture's) disease?
What shared pathogenic mechanism is observed in both ANCA-associated glomerulonephritis and Goodpasture's disease that directly leads to glomerular damage?
What shared pathogenic mechanism is observed in both ANCA-associated glomerulonephritis and Goodpasture's disease that directly leads to glomerular damage?
Which of the following is least likely to cause haematuria related to the ureter?
Which of the following is least likely to cause haematuria related to the ureter?
A patient presents with haematuria and is subsequently diagnosed with bladder cancer. Which specific type of cancer is the underlying cause the majority of the time?
A patient presents with haematuria and is subsequently diagnosed with bladder cancer. Which specific type of cancer is the underlying cause the majority of the time?
What is the primary pathological mechanism that distinguishes nephrotic syndrome from nephritic syndrome?
What is the primary pathological mechanism that distinguishes nephrotic syndrome from nephritic syndrome?
Which of the following clinical findings is least associated with nephrotic syndrome?
Which of the following clinical findings is least associated with nephrotic syndrome?
A patient with nephrotic syndrome develops a deep vein thrombosis (DVT). What is the primary pathophysiological mechanism contributing to this increased risk of thromboembolism?
A patient with nephrotic syndrome develops a deep vein thrombosis (DVT). What is the primary pathophysiological mechanism contributing to this increased risk of thromboembolism?
Which of the following features is least characteristic of minimal change disease (MCD)?
Which of the following features is least characteristic of minimal change disease (MCD)?
In the context of minimal change disease, what is the most critical finding on electron microscopy that confirms the diagnosis and explains the proteinuria?
In the context of minimal change disease, what is the most critical finding on electron microscopy that confirms the diagnosis and explains the proteinuria?
Which of the following mechanisms contributes the least to the pathogenesis of minimal change disease?
Which of the following mechanisms contributes the least to the pathogenesis of minimal change disease?
What best describes the distribution of glomerular involvement in Focal Segmental Glomerulosclerosis (FSGS)?
What best describes the distribution of glomerular involvement in Focal Segmental Glomerulosclerosis (FSGS)?
What distinguishes steroid-responsive FSGS from steroid-unresponsive FSGS in terms of underlying pathophysiology?
What distinguishes steroid-responsive FSGS from steroid-unresponsive FSGS in terms of underlying pathophysiology?
Which of the following would least likely be a factor in the pathogenesis of steroid-unresponsive FSGS?
Which of the following would least likely be a factor in the pathogenesis of steroid-unresponsive FSGS?
What is the primary target antigen in the majority of cases of primary membranous glomerulonephritis (MGN)?
What is the primary target antigen in the majority of cases of primary membranous glomerulonephritis (MGN)?
What unique histological feature is commonly observed in membranous glomerulonephritis (MGN) using silver stain?
What unique histological feature is commonly observed in membranous glomerulonephritis (MGN) using silver stain?
What is the primary mechanism driving the deposition of amyloid fibrils in the kidney?
What is the primary mechanism driving the deposition of amyloid fibrils in the kidney?
Which of the following conditions is least associated with amyloidosis of the kidney?
Which of the following conditions is least associated with amyloidosis of the kidney?
What is the typical appearance of the kidney in advanced amyloidosis upon gross examination?
What is the typical appearance of the kidney in advanced amyloidosis upon gross examination?
A patient with nephrotic syndrome has a kidney biopsy performed. Light microscopy shows glomeruli with thickened capillary walls, and silver stain reveals 'spikes' on the basement membrane. Immunofluorescence microscopy is most likely to show which of the following?
A patient with nephrotic syndrome has a kidney biopsy performed. Light microscopy shows glomeruli with thickened capillary walls, and silver stain reveals 'spikes' on the basement membrane. Immunofluorescence microscopy is most likely to show which of the following?
A patient with a history of intravenous drug use presents with nephrotic syndrome. Kidney biopsy reveals focal segmental glomerulosclerosis (FSGS). Which of the following is the most likely underlying mechanism in this patient's FSGS?
A patient with a history of intravenous drug use presents with nephrotic syndrome. Kidney biopsy reveals focal segmental glomerulosclerosis (FSGS). Which of the following is the most likely underlying mechanism in this patient's FSGS?
A 65-year-old patient presents with nephrotic syndrome. Further workup reveals monoclonal gammopathy. Kidney biopsy shows amyloid deposits. Electron microscopy of the kidney is most likely to show which of the following?
A 65-year-old patient presents with nephrotic syndrome. Further workup reveals monoclonal gammopathy. Kidney biopsy shows amyloid deposits. Electron microscopy of the kidney is most likely to show which of the following?
A patient with a history of hepatitis B infection presents with nephrotic syndrome. Kidney biopsy reveals membranous glomerulonephritis (MGN). In this case, the MGN is likely to be related to which of the following?
A patient with a history of hepatitis B infection presents with nephrotic syndrome. Kidney biopsy reveals membranous glomerulonephritis (MGN). In this case, the MGN is likely to be related to which of the following?
A 7-year-old child presents with sudden onset edema, proteinuria, and hypoalbuminemia. Initial investigations are unremarkable apart from nephrotic range proteinuria. Which of the following kidney biopsy findings would least support a diagnosis of minimal change disease (MCD)?
A 7-year-old child presents with sudden onset edema, proteinuria, and hypoalbuminemia. Initial investigations are unremarkable apart from nephrotic range proteinuria. Which of the following kidney biopsy findings would least support a diagnosis of minimal change disease (MCD)?
A 45-year-old African American male with a history of hypertension and obesity presents with nephrotic syndrome. Kidney biopsy reveals Focal Segmental Glomerulosclerosis (FSGS). Which of the following is least likely to be an underlying factor in causing FSGS in this patient?
A 45-year-old African American male with a history of hypertension and obesity presents with nephrotic syndrome. Kidney biopsy reveals Focal Segmental Glomerulosclerosis (FSGS). Which of the following is least likely to be an underlying factor in causing FSGS in this patient?
A 60-year-old woman presents with nephrotic syndrome and is found to have membranous glomerulonephritis (MGN). Further testing shows positive anti-PLA2R antibodies. What does the presence of these antibodies suggest about the nature of her disease?
A 60-year-old woman presents with nephrotic syndrome and is found to have membranous glomerulonephritis (MGN). Further testing shows positive anti-PLA2R antibodies. What does the presence of these antibodies suggest about the nature of her disease?
A 55-year-old male presents with nephrotic syndrome. Kidney biopsy reveals amyloid deposits that stain positive with Congo red and show apple-green birefringence under polarized light. Which of the following is the most important next step in evaluating the cause of his amyloidosis?
A 55-year-old male presents with nephrotic syndrome. Kidney biopsy reveals amyloid deposits that stain positive with Congo red and show apple-green birefringence under polarized light. Which of the following is the most important next step in evaluating the cause of his amyloidosis?
A patient is diagnosed with ANCA-associated vasculitis affecting the kidneys. What is the primary mechanism by which ANCA antibodies cause damage to the glomeruli?
A patient is diagnosed with ANCA-associated vasculitis affecting the kidneys. What is the primary mechanism by which ANCA antibodies cause damage to the glomeruli?
A young male presents with haematuria, hearing loss, and progressive renal failure. A kidney biopsy shows glomerular basement membrane abnormalities. Which of the following is the most likely diagnosis?
A young male presents with haematuria, hearing loss, and progressive renal failure. A kidney biopsy shows glomerular basement membrane abnormalities. Which of the following is the most likely diagnosis?
A patient with known IgA nephropathy develops macroscopic haematuria after an upper respiratory infection. What is the most likely mechanism of this haematuria?
A patient with known IgA nephropathy develops macroscopic haematuria after an upper respiratory infection. What is the most likely mechanism of this haematuria?
A patient with nephrotic syndrome is treated with corticosteroids, but the proteinuria persists, and a kidney biopsy is performed. Light microscopy shows segmental areas of sclerosis in some glomeruli, and electron microscopy reveals foot process effacement. Which of the following is most likely the diagnosis?
A patient with nephrotic syndrome is treated with corticosteroids, but the proteinuria persists, and a kidney biopsy is performed. Light microscopy shows segmental areas of sclerosis in some glomeruli, and electron microscopy reveals foot process effacement. Which of the following is most likely the diagnosis?
A patient with a history of Goodpasture's syndrome is being monitored for disease recurrence. Which of the following laboratory findings would be the most specific indicator of recurrent disease activity?
A patient with a history of Goodpasture's syndrome is being monitored for disease recurrence. Which of the following laboratory findings would be the most specific indicator of recurrent disease activity?
Which of the following is least likely to be directly associated with glomerular haematuria?
Which of the following is least likely to be directly associated with glomerular haematuria?
What is the primary clinical significance of identifying red cell casts in a urine sample?
What is the primary clinical significance of identifying red cell casts in a urine sample?
In a patient presenting with haematuria, what aspect of their clinical history is least likely to assist in differentiating between medical and surgical causes?
In a patient presenting with haematuria, what aspect of their clinical history is least likely to assist in differentiating between medical and surgical causes?
What systemic condition is least likely to present with haematuria as a primary manifestation?
What systemic condition is least likely to present with haematuria as a primary manifestation?
A patient presents with haematuria and is found to have low platelets. What pathophysiological change is least likely to contribute to the presentation of haematuria?
A patient presents with haematuria and is found to have low platelets. What pathophysiological change is least likely to contribute to the presentation of haematuria?
Which of the following characteristics is least indicative of pain associated with haematuria?
Which of the following characteristics is least indicative of pain associated with haematuria?
In the context of IgA nephropathy, what is the primary mechanism by which upper respiratory tract infections are associated with episodes of macroscopic haematuria?
In the context of IgA nephropathy, what is the primary mechanism by which upper respiratory tract infections are associated with episodes of macroscopic haematuria?
Which element of the Oxford classification of IgA nephropathy is least indicative of a poor prognosis?
Which element of the Oxford classification of IgA nephropathy is least indicative of a poor prognosis?
What clinical feature is least likely to be present in a female carrier of Alport syndrome?
What clinical feature is least likely to be present in a female carrier of Alport syndrome?
What pathological finding is most indicative of Alport syndrome rather than thin basement membrane nephropathy (TBMN)?
What pathological finding is most indicative of Alport syndrome rather than thin basement membrane nephropathy (TBMN)?
In ANCA-associated vasculitis, what is the principal mechanism by which neutrophils contribute to glomerular injury?
In ANCA-associated vasculitis, what is the principal mechanism by which neutrophils contribute to glomerular injury?
How does the immunofluorescence pattern typically differ between anti-GBM disease (Goodpasture's) and ANCA-associated vasculitis?
How does the immunofluorescence pattern typically differ between anti-GBM disease (Goodpasture's) and ANCA-associated vasculitis?
In the context of haematuria, what is least likely to be primarily associated with ureteral factors?
In the context of haematuria, what is least likely to be primarily associated with ureteral factors?
How does damage to visceral epithelial cells directly contribute to the development of nephrotic syndrome?
How does damage to visceral epithelial cells directly contribute to the development of nephrotic syndrome?
Which of the following is a less common feature of nephrotic syndrome?
Which of the following is a less common feature of nephrotic syndrome?
Which mechanism contributes the least to hypercoagulability in nephrotic syndrome?
Which mechanism contributes the least to hypercoagulability in nephrotic syndrome?
What is the primary mechanism underlying the pathogenesis of proteinuria in minimal change disease (MCD)?
What is the primary mechanism underlying the pathogenesis of proteinuria in minimal change disease (MCD)?
In Minimal Change Disease, other than diffuse foot process effacement, what other electron microscopy findings are expected?
In Minimal Change Disease, other than diffuse foot process effacement, what other electron microscopy findings are expected?
Which of the following is least associated with the development of minimal change disease?
Which of the following is least associated with the development of minimal change disease?
In focal segmental glomerulosclerosis (FSGS), which of the following patterns of glomerular involvement is least likely to be observed?
In focal segmental glomerulosclerosis (FSGS), which of the following patterns of glomerular involvement is least likely to be observed?
What characteristic is generally associated with steroid-responsive FSGS but not typically observed in steroid-resistant FSGS?
What characteristic is generally associated with steroid-responsive FSGS but not typically observed in steroid-resistant FSGS?
Which factor is least likely to contribute to the development of acquired or secondary forms of focal segmental glomerulosclerosis (FSGS)?
Which factor is least likely to contribute to the development of acquired or secondary forms of focal segmental glomerulosclerosis (FSGS)?
What is the most significant difference in clinical outcome between primary and secondary membranous glomerulonephritis (MGN)?
What is the most significant difference in clinical outcome between primary and secondary membranous glomerulonephritis (MGN)?
In membranous glomerulonephritis (MGN), what is the primary significance of the 'spike' formation observed on silver staining of the glomerular basement membrane?
In membranous glomerulonephritis (MGN), what is the primary significance of the 'spike' formation observed on silver staining of the glomerular basement membrane?
Which of the following mechanisms is the least likely contributor to the pathogenesis of amyloid deposition in the kidney?
Which of the following mechanisms is the least likely contributor to the pathogenesis of amyloid deposition in the kidney?
Which of the following characteristics is least associated with renal amyloidosis?
Which of the following characteristics is least associated with renal amyloidosis?
What is the least expected presentation of the kidney in advanced stages of amyloidosis?
What is the least expected presentation of the kidney in advanced stages of amyloidosis?
A patient's kidney biopsy shows glomeruli infiltrated by Congo red-positive material displaying apple-green birefringence under polarized light. This indicates what?
A patient's kidney biopsy shows glomeruli infiltrated by Congo red-positive material displaying apple-green birefringence under polarized light. This indicates what?
A young adult with a history of intravenous drug use presents with nephrotic syndrome. A kidney biopsy reveals focal segmental glomerulosclerosis (FSGS). The pathogenesis of FSGS in this patient least likely directly involves:
A young adult with a history of intravenous drug use presents with nephrotic syndrome. A kidney biopsy reveals focal segmental glomerulosclerosis (FSGS). The pathogenesis of FSGS in this patient least likely directly involves:
A 60-year-old presents with nephrotic syndrome, and further workup reveals monoclonal gammopathy. A kidney biopsy shows amyloid deposits. What Electron microscopy finding is least likely?
A 60-year-old presents with nephrotic syndrome, and further workup reveals monoclonal gammopathy. A kidney biopsy shows amyloid deposits. What Electron microscopy finding is least likely?
A patient with hepatitis B infection presents with nephrotic syndrome. Kidney biopsy reveals membranous glomerulonephritis (MGN). In this case, the MGN is most likely related to:
A patient with hepatitis B infection presents with nephrotic syndrome. Kidney biopsy reveals membranous glomerulonephritis (MGN). In this case, the MGN is most likely related to:
A child presents with nephrotic syndrome, and initial investigations reveal nephrotic-range proteinuria, but all other findings are unremarkable. Which kidney biopsy feature would least support a diagnosis of minimal change disease
A child presents with nephrotic syndrome, and initial investigations reveal nephrotic-range proteinuria, but all other findings are unremarkable. Which kidney biopsy feature would least support a diagnosis of minimal change disease
An obese African American male with hypertension presents with nephrotic syndrome. Kidney biopsy reveals FSGS. Which factor is least likely to cause FSGS in this patient?
An obese African American male with hypertension presents with nephrotic syndrome. Kidney biopsy reveals FSGS. Which factor is least likely to cause FSGS in this patient?
A patient presents with nephrotic syndrome and membranous glomerulonephritis (MGN). Further testing shows positive anti-PLA2R antibodies. What does the presence of these antibodies primarily suggest?
A patient presents with nephrotic syndrome and membranous glomerulonephritis (MGN). Further testing shows positive anti-PLA2R antibodies. What does the presence of these antibodies primarily suggest?
A patient presents with nephrotic syndrome. Kidney biopsy reveals amyloid deposits. Which step is most important in evaluating cause of amyloidosis?
A patient presents with nephrotic syndrome. Kidney biopsy reveals amyloid deposits. Which step is most important in evaluating cause of amyloidosis?
What is the least likely mechanism by which ANCA antibodies directly contribute to glomerular damage?
What is the least likely mechanism by which ANCA antibodies directly contribute to glomerular damage?
What is the least clinical manifestation to be expected in a patient with Alport syndrome?
What is the least clinical manifestation to be expected in a patient with Alport syndrome?
Flashcards
Haematuria
Haematuria
Blood in the urine, can be microscopic or macroscopic.
Haematuria: Painless vs Painful
Haematuria: Painless vs Painful
Painless is associated with malignancy, painful is associated with UTI stones
Causes of Haematuria
Causes of Haematuria
Systemic Clotting disorders, Kidney, Ureter, Bladder and Urethra
Kidney-related causes of haematuria
Kidney-related causes of haematuria
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Ureteric causes of Haematuria
Ureteric causes of Haematuria
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Bladder causes of Haematuria
Bladder causes of Haematuria
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Urethral causes of Haematuria
Urethral causes of Haematuria
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Prostatic causes of Haematuria
Prostatic causes of Haematuria
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Nephrosis vs Nephritis.
Nephrosis vs Nephritis.
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Clinical Features of Nephrotic Syndrome
Clinical Features of Nephrotic Syndrome
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Main Causes for Nephrotic Syndrome
Main Causes for Nephrotic Syndrome
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IgA Glomerulonephritis
IgA Glomerulonephritis
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Glomeruli changes with IgA
Glomeruli changes with IgA
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IgA Glomerulonephritis Classification: Oxford System
IgA Glomerulonephritis Classification: Oxford System
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Thin Membrane Neuropathy
Thin Membrane Neuropathy
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Alport's Syndrome
Alport's Syndrome
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ANCA Vasculitis
ANCA Vasculitis
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Pathophysiology process of ANCA Vasculitis
Pathophysiology process of ANCA Vasculitis
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Pulmonary-renal syndromes
Pulmonary-renal syndromes
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Goodpasture's Syndrome
Goodpasture's Syndrome
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Anti-Glomerular basement membrane disease.
Anti-Glomerular basement membrane disease.
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ANCA vs. Anti-GBM
ANCA vs. Anti-GBM
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Clinical presentation 2- Nephrotic Syndrome
Clinical presentation 2- Nephrotic Syndrome
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Nephrotic syndrome indications
Nephrotic syndrome indications
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Minimal change disease
Minimal change disease
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Pathology of minimal change disease
Pathology of minimal change disease
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Focal and Segmental glomerulosclerosis FSGS
Focal and Segmental glomerulosclerosis FSGS
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2 Main types of FSGS
2 Main types of FSGS
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FSGS Steroid responsive/Dependent
FSGS Steroid responsive/Dependent
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FSGS Steroid unresponsive
FSGS Steroid unresponsive
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Membranous Glomerulonephritis
Membranous Glomerulonephritis
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Amyloid tissue structures
Amyloid tissue structures
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Study Notes
- The lecture discusses Glomerular causes of haematuria and nephrotic syndrome.
- Class is in year 2 of a Pathology course.
Clinical Presentation of Haematuria
- Haematuria refers to the presence of blood in the urine.
- Microscopic haematuria involves trace amounts of blood.
- Macroscopic haematuria involves gross or frank blood presence.
- In microscopic haematuria, the urine appears normal.
- Red cell casts can be found in urine.
- Blood observed in the tubule corresponds to haematuria.
Classification and Causes of Haematuria
- Haematuria can be classified as painless or painful.
- It can also be categorized as medical or surgical.
- Causes include systemic conditions. Potential clotting disorders, low platelets, and Warfarin use.
- Haematuria origin points can be located in the kidney, ureter, bladder and urethra.
Causes of Haematuria: Kidney
- Glomerulonephritis, including IgA nephropathy.
- Additionally, Acute GN due to post-infectious causes.
- Thin membranes which are of little clinical concern.
- Alport’s syndrome
- Vascular causes (vasculitis)
- Neoplasms (carcinoma)
IgA Glomerulonephritis
- Direct immunofluorescence for immunoglobulin A (IgA).
- Sclerosed glomeruli are the point where the glomerulus is enlarged with increased mesangial cells
The Oxford System
- The Oxford system is utilized for IgA classification and prognosis.
- M: Mesangial cellularity increase
- E: Endocapillary inflammation
- S: Segmental sclerosis
- T: Tubulo-Interstitial Fibrosis
- C: Crescents
Thin Membrane Nephropathy and Alport Syndrome
- Thin membranes represent the thinner end of the normal distribution.
- Thin membrane nephropathy is also known as Benign familial haematuria/March haematuria.
- It generally has a benign course.
- Females may be carriers of the Alport's gene.
Alport's Syndrome
- Alport's syndrome is associated with nerve deafness and eye abnormalities.
- It is X-linked in 85% of cases.
- Males typically develop the full disease, with 90% progressing to end-stage kidney disease by age 40.
- Females are carriers with thin membranes.
- Other pedigrees with milder disease also occur; genetic counseling is used for severe disease.
Vasculitis
- ANCA Vasculitis is a systemic disease.
- ANCA stands for Anti-Neutrophil Cytoplasmic Autoantibody.
- The activation of neutrophils involves molecules protruding through the PMN (polymorphonuclear neutrophil) cell wall.
- Antibodies target myeloperoxidase or proteinase 3.
- Antibodies bind to MPO/Pr3 and affect of the blood vessel wall by inducing inflammation.
ANCA-Related Conditions
- ANCA can be cytoplasmic or perinuclear.
- Activated PMNs make "holes" in the basement membrane, which result from ANCA.
- Large cellular crescents.
- Pulmonary-renal syndromes, such as pulmonary haemorrhage.
- Lung haemorrhage can occur in ANCA vasculitis.
Goodpasture Syndrome
- Goodpasture syndrome is an anti-glomerular basement membrane disease.
- Autoantibodies target the non-collagenous domain of the alpha3 chain of collagen type 4.
- It affects both kidneys and lungs, leading to pulmonary/renal syndrome.
- Anti-GBM Glomerulonephritis also results in PMN activation similar to ANCA disease.
- Anti-GBM disease shows linear IgG positivity.
- Rupture of capillary loops.
ANCA and Anti-GBM Overlap
- ANCA and Anti-GBM are crossover immunological diseases involving molecular mimicry.
- They are likely induced by neutrophil-activating processes, such as infection or allergy.
- Antibodies produced to the original antigen recognize neutrophil proteins (ANCA) or collagen proteins (Anti-GBM) as antigen.
Causes of Haematuria:
- Ureteric sources include urothelial carcinoma, UTI, and calculi.
- Bladder sources include urothelial carcinoma, UTI, calculi, and iatrogenic causes and catheter use.
- Urethral sources include UTI, STD's, and urethral caruncle.
- Prostatic sources include BPH, Ca prostate, and prostatitis.
Clinical Presentation of Nephrotic Syndrome
- Involves nephrosis versus nephritis.
- An absence of inflammation results in the absence of raised BP and haematuria.
- Pathogenesis involves damage to visceral epithelial cells.
Normal Filtration
- Normal filter membrane ultra structure includes parietal and visceral epithelial cells, Bowman's space and membranes, fenestrations and mesangial cells amongst other complex processes.
Clinical Features and Site of Pathology
- Proteinuria (≥ 3.5g/24hrs)
- Oedema
- Hypoalbuminaemia
- Hypercholesterolaemia
- Thrombophilia
Severe lower limb oedema
Main Causes of Nephrotic Syndrome
- Minimal change disease
- Focal and segmental glomerulosclerosis
- Membranous glomerulonephritis
- Amyloidosis
- Membranoproliferative GN (MPGN). It can present with nephrotic syndrome or acute nephritic syndrome
Nephrotic Syndrome: Key Indicators
- Clinical features point to site of pathology.
- Nephrotic syndrome with normal creatinine indicates damage without inflammation.
- Raised creatinine with blood in urine indicates glomerular inflammation.
Minimal Change Disease
- It affects all ages, especially children and the elderly.
- Has an acute onset of florid nephrotic syndrome.
- Albuminuria is often greater than 20g/24 hours.
- It is self-limiting.
- Minimal change disease responds very well to steroids.
- There is full recovery of condition.
- Results from direct injury to foot processes. By short term agent (unknown).
Pathology: Minimal Change Disease
- Light Microscopy (L.M.) appears normal.
- Fluorescence Microscopy (F.M.) shows no deposits.
- Electron microscopy shows diffuse flattening and simplification of foot processes
Focal and Segmental Glomerulosclerosis (FSGS)
- FSGS must have nephrotic range proteinuria.
- There are two main types: Steroid-responsive/steroid-dependent. Steroid-unresponsive
Steroid Responsive FSGS
- Involves a circulating factor causing foot process injury.
- Causes end-stage kidney disease inevitably over several years/decades.
- Recurs in transplants because the circulating factor persists post-transplant.
Steroid Unresponsive FSGS
- Due to an abnormality/absence of foot process protein.
- Most profound form is due to nephrin abnormality (Finnish type).
- Congenital Nephrotic Syndrome (NS).
- Dozens of abnormalities identified; cytogenetic tests are available.
- Does not recur in transplants.
High-Power Schematic
- A high-power schematic view of the foot process highlights components.
- The absence or abnormality of these components which can cause FSGS usually resulting in chronic renal failure.
Membranous Glomerulonephritis
- It is an immune complex deposition disease.
- It is a crossover immunological disease.
- The antigen is Phospholipase A2 receptor (abundant in foot processes of epithelial cells).
- Antibodies are produced in reaction to a variety of diseases/drugs and sees PLA2 as antigen.
- It responds well to therapy, typically with 3 years recovery.
- The thickening of capillary loops, "Spikes”, is a new membrane between deposits.
- Granular positivity of IgG
- Deposits reside on the epithelial side of the basement membrane with no noted inflammation.
Amyloid and the Kidney
- The kidney is often involved.
- Fibrils deposit in the mesangium and capillary loops, as well as arterioles and interstitium.
- It presents with severe nephrotic syndrome and some renal function impairment.
- All types of amyloid can trigger such a response.
- The commonest type seen is due to multiple myeloma/plasma cell dyscrasia.
- Amyloid presents as waxy, starch-like tissue.
- Amyloid is "deposited" throughout the glomerulus observed under Light microscopy/Electron microscopy.
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