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Questions and Answers
In the context of nephritic syndrome, what is the primary mechanism that leads to the disruption of the glomerular filtration process?
In the context of nephritic syndrome, what is the primary mechanism that leads to the disruption of the glomerular filtration process?
- Inflammation-induced damage to the glomerular basement membrane, resulting in impaired filtration and leakage of red blood cells and protein. (correct)
- Vasoconstriction of the afferent arteriole, leading to reduced glomerular blood flow and subsequent reduction in the glomerular filtration rate (GFR).
- Deposition of immune complexes in the tubular interstitium, leading to tubular damage and impaired reabsorption.
- Direct injury to the podocytes, causing increased permeability and massive proteinuria characteristic of nephrotic syndrome.
Which of the following conditions associated with nephritic syndrome involves the deposition of IgA in the mesangium, often following an upper respiratory or gastrointestinal infection?
Which of the following conditions associated with nephritic syndrome involves the deposition of IgA in the mesangium, often following an upper respiratory or gastrointestinal infection?
- ANCA-associated vasculitis (AAV)
- Anti-glomerular basement membrane (anti-GBM) disease
- Henoch-Schönlein Purpura (HSP) (correct)
- Post-infectious glomerulonephritis
A patient presents with nephritic syndrome and rapidly declining renal function. Which of the following patterns of immunofluorescence findings would be most consistent with anti-GBM disease?
A patient presents with nephritic syndrome and rapidly declining renal function. Which of the following patterns of immunofluorescence findings would be most consistent with anti-GBM disease?
- Absence of staining
- Mesangial deposition
- Diffuse granular pattern
- Linear pattern (correct)
What is the primary pathological process that characterizes rapidly progressive glomerulonephritis (RPGN)?
What is the primary pathological process that characterizes rapidly progressive glomerulonephritis (RPGN)?
A patient presents with nephritic syndrome, pulmonary hemorrhage, and antibodies against the glomerular basement membrane. Which type of collagen is targeted by these antibodies?
A patient presents with nephritic syndrome, pulmonary hemorrhage, and antibodies against the glomerular basement membrane. Which type of collagen is targeted by these antibodies?
Which of the following clinical findings is most indicative of ANCA-associated vasculitis causing nephritic syndrome?
Which of the following clinical findings is most indicative of ANCA-associated vasculitis causing nephritic syndrome?
Which laboratory finding is most specific for post-streptococcal glomerulonephritis (PSGN) in the context of nephritic syndrome?
Which laboratory finding is most specific for post-streptococcal glomerulonephritis (PSGN) in the context of nephritic syndrome?
In a patient with membranoproliferative glomerulonephritis (MPGN), what histological finding is most characteristic?
In a patient with membranoproliferative glomerulonephritis (MPGN), what histological finding is most characteristic?
What is true regarding the symptoms of Nephritic Syndrome?
What is true regarding the symptoms of Nephritic Syndrome?
A patient with nephritic syndrome also exhibits features of Henoch-Schönlein purpura. What additional signs or symptoms would support this diagnosis?
A patient with nephritic syndrome also exhibits features of Henoch-Schönlein purpura. What additional signs or symptoms would support this diagnosis?
In the differential diagnosis of hematuria, what conditions should be considered in addition to nephritic syndrome?
In the differential diagnosis of hematuria, what conditions should be considered in addition to nephritic syndrome?
A patient with nephritic syndrome presents with edema. What other condition should be considered in the differential diagnosis?
A patient with nephritic syndrome presents with edema. What other condition should be considered in the differential diagnosis?
Which of the following is the primary purpose of obtaining a renal biopsy in the evaluation of nephritic syndrome?
Which of the following is the primary purpose of obtaining a renal biopsy in the evaluation of nephritic syndrome?
What is the general target blood pressure for managing patients with nephritic syndrome?
What is the general target blood pressure for managing patients with nephritic syndrome?
Which initial treatment is most appropriate for a 45-year-old male, who presents to the clinic with complaints of dark-coloured urine, swelling in his hands and feet, and joint pain. On examination, he has rash on his lower extremities. No past medical history and not on any regular medications. On Examination his temperature is 37.3°C, pulse is 88/min, respiratiosn are 20/min and blood pressure is 150/80 mmHg. He has a vasculitic rash with small, palpable purpuric lesions. His serum creatinine is 194 umol/L, Blood Urea Nitrogen is 15.0 mmol/L, Serum Potassium is 4.0 mmol/L, and Urinalysis shows Blood +3 protein +1 with RBC casts, WBC 10-15/HPF, no bacteria.
Which initial treatment is most appropriate for a 45-year-old male, who presents to the clinic with complaints of dark-coloured urine, swelling in his hands and feet, and joint pain. On examination, he has rash on his lower extremities. No past medical history and not on any regular medications. On Examination his temperature is 37.3°C, pulse is 88/min, respiratiosn are 20/min and blood pressure is 150/80 mmHg. He has a vasculitic rash with small, palpable purpuric lesions. His serum creatinine is 194 umol/L, Blood Urea Nitrogen is 15.0 mmol/L, Serum Potassium is 4.0 mmol/L, and Urinalysis shows Blood +3 protein +1 with RBC casts, WBC 10-15/HPF, no bacteria.
A patient with nephritic syndrome is diagnosed with IgA nephropathy. According to the information, what percentage of patients with IgA nephropathy progress to end-stage kidney disease (ESKD) within 20 years?
A patient with nephritic syndrome is diagnosed with IgA nephropathy. According to the information, what percentage of patients with IgA nephropathy progress to end-stage kidney disease (ESKD) within 20 years?
A patient with HSP results in ESKD in how many years?
A patient with HSP results in ESKD in how many years?
How would you define Nephritic Syndrome?
How would you define Nephritic Syndrome?
What is the most common cause of the Aetiology of Nephritic Syndrome?
What is the most common cause of the Aetiology of Nephritic Syndrome?
What is the overarching principle of management dependent on?
What is the overarching principle of management dependent on?
What are the first line Immunosuppressive medications commonly used?
What are the first line Immunosuppressive medications commonly used?
The treatment for Nephritic Syndrome depends on what main factor?
The treatment for Nephritic Syndrome depends on what main factor?
What are the 4 main components of a complete physical examination in a patient?
What are the 4 main components of a complete physical examination in a patient?
When considering laboratory investigations of urinalysis, what 2 outcomes of the urine dipstick are important?
When considering laboratory investigations of urinalysis, what 2 outcomes of the urine dipstick are important?
When considering labarotory investigations of blood tests, which of these options should be included in the routine test?
When considering labarotory investigations of blood tests, which of these options should be included in the routine test?
When considering laboratory investigations of blood tests, which of these options should be included in the Serological/ Immunological test?
When considering laboratory investigations of blood tests, which of these options should be included in the Serological/ Immunological test?
List the signs and symptoms that should be looked for in the clinical evaluation of nephritic syndrome.
List the signs and symptoms that should be looked for in the clinical evaluation of nephritic syndrome.
A patient with nephritic syndrome has a history of recent pharyngitis and presents with hematuria, hypertension, and edema. Which of the following immune mechanisms is most likely contributing to the glomerular injury in this patient?
A patient with nephritic syndrome has a history of recent pharyngitis and presents with hematuria, hypertension, and edema. Which of the following immune mechanisms is most likely contributing to the glomerular injury in this patient?
A patient with nephritic syndrome is suspected of having ANCA-associated vasculitis. Which of the following autoantibodies is most likely to be elevated in this patient?
A patient with nephritic syndrome is suspected of having ANCA-associated vasculitis. Which of the following autoantibodies is most likely to be elevated in this patient?
A patient with nephritic syndrome is found to have low serum C3 and C4 complement levels. Which of the following glomerular diseases is most likely associated with this finding?
A patient with nephritic syndrome is found to have low serum C3 and C4 complement levels. Which of the following glomerular diseases is most likely associated with this finding?
What are the common signs in an examination with a patient presenting with Nephritic Syndrome?
What are the common signs in an examination with a patient presenting with Nephritic Syndrome?
When describing Rapidly Progressive Glomerulonephritis (RPGN) what is the true definition?
When describing Rapidly Progressive Glomerulonephritis (RPGN) what is the true definition?
What is the role of a renal ultrasound in the initial diagnostic workup of nephritic syndrome?
What is the role of a renal ultrasound in the initial diagnostic workup of nephritic syndrome?
In managing edema associated with nephritic syndrome, what is the first-line dietary recommendation?
In managing edema associated with nephritic syndrome, what is the first-line dietary recommendation?
Which laboratory parameter is most useful for monitoring the effectiveness of immunosuppressive therapy in a patient with nephritic syndrome?
Which laboratory parameter is most useful for monitoring the effectiveness of immunosuppressive therapy in a patient with nephritic syndrome?
Which of the following management strategies is aimed at correcting fluid volume overload in nephritic syndrome?
Which of the following management strategies is aimed at correcting fluid volume overload in nephritic syndrome?
For IgA Nephropathy, what symptoms would make you consider it?
For IgA Nephropathy, what symptoms would make you consider it?
What would you see on Immunofluorescence regarding IgA Nephropathy?
What would you see on Immunofluorescence regarding IgA Nephropathy?
Post-streptococcal Glomerulonephritis normally occurs how many weeks after a haemo streptococcal infection?
Post-streptococcal Glomerulonephritis normally occurs how many weeks after a haemo streptococcal infection?
What is an important characteristic to note about Membranoproliferative Glomerulonephritis (MPGN)?
What is an important characteristic to note about Membranoproliferative Glomerulonephritis (MPGN)?
Flashcards
Nephritic Syndrome
Nephritic Syndrome
Clinical manifestation of glomerular inflammation, presenting with proteinuria, microscopic hematuria, rise in serum creatinine, and possible hypertension.
Causes of Nephritic Syndrome
Causes of Nephritic Syndrome
ANCA-associated vasculitis; Anti-glomerular basement membrane disease; IgA nephropathy; Lupus nephritis; Post-infectious glomerulonephritis; Membranoproliferative glomerulonephritis
Pathogenesis of Nephritic Syndrome
Pathogenesis of Nephritic Syndrome
Disrupts filtration, leading to glomerular basement membrane destruction. Glomerular injury is often immune-mediated or due to systemic conditions.
Rapidly Progressive Glomerulonephritis
Rapidly Progressive Glomerulonephritis
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ANCA-Associated Vasculitis findings
ANCA-Associated Vasculitis findings
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Anti-GBM Disease findings
Anti-GBM Disease findings
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IgA Nephropathy
IgA Nephropathy
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Henoch-Schonlein Purpura
Henoch-Schonlein Purpura
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Post-Streptococcal GN
Post-Streptococcal GN
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Membranoproliferative GN
Membranoproliferative GN
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Symptoms of Nephritic Syndrome
Symptoms of Nephritic Syndrome
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Differential Diagnosis for Hematuria
Differential Diagnosis for Hematuria
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Differential Diagnosis for Odema
Differential Diagnosis for Odema
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Medical History in Nephritic Syndrome
Medical History in Nephritic Syndrome
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Urinalysis
Urinalysis
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Creatinine ratio
Creatinine ratio
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Routine Blood Tests
Routine Blood Tests
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Immunological Testing
Immunological Testing
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Imaging for Diagnosis
Imaging for Diagnosis
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Renal Biopsy
Renal Biopsy
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Medical management
Medical management
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immunosuppressive drugs and treatment
immunosuppressive drugs and treatment
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Prognosis
Prognosis
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Study Notes
- Nephritic syndrome is a clinical condition due to glomerular inflammation.
- Nephritic syndrome manifests with proteinuria, microscopic hematuria, rising serum creatinine, and hypertension.
Causes of Nephritic Syndrome
- ANCA-associated vasculitis (AAV)
- Anti-glomerular basement membrane disease (anti-GBM disease)
- IgA nephropathy / Henoch-Schonlein Purpura (HSP)
- Lupus nephritis
- Immune complex-mediated glomerulonephritis (GN)
- Post-infectious glomerulonephritis, also known as infection-associated GN
- Membrano-proliferative glomerulonephritis
- Cryoglobulinaemic vasculitis
Pathogenesis
- Glomerular injury disrupts filtration, leading to glomerular basement membrane destruction.
- Glomerular injury can be immune-mediated, infectious, or non-inflammatory due to systemic conditions.
- Immune-mediated nephritic syndromes can be classified using immunofluorescence findings.
- Injury to the Glomerulus results in Proteinuria, Hypertension, Cola-colored urine, and Oliguria
Rapidly Progressive Glomerulonephritis (RPGN)
- RPGN involves aggressive glomerulonephritis rapidly worsening to cause renal failure.
- RPGN is characterized by the proliferation of parietal epithelial cells and macrophages in Bowman's capsule.
- Small vessel/ANCA Vasculitis can cause RPGN
- Lupus Nephritis can cause RPGN
- Anti-GBM Disease can cause RPGN
- Other glomerulonephritis such as IgA Nephropathy and Membranoproliferative can transform to RPGN
ANCA-Associated Vasculitis
- May be associated with anti-PR3 or anti-MPO antibodies.
- ANCA-Associated Vasculitis has seasonal variations in incidence
- Leads to nephritic syndrome, constitutional symptoms, and ocular/ENT symptoms.
- Evidence may show small vessel vasculitis with rash or pulmonary hemorrhage, and pulmonary symptoms such as hemoptysis, cough, and dyspnea.
Anti-Glomerular Basement Membrane Disease
- Relatively uncommon.
- Clinical findings may include nephritic syndrome.
- Urine output decrease within days to weeks which can possibly lead to anuria.
- Patients experience fatigue.
- Evidence may show small vessel vasculitis with pulmonary symptoms such as hemoptysis, cough, and dyspnea.
- Antibodies to Type IV Collagen in the glomerular basement membrane (anti-GBM Abs) are present
- Can rapidly progress to ESKD (End-Stage Kidney Disease)
IgA Nephropathy
- Most common primary GN in high income countries.
- Occurs classically as gross haematuria after an Upper Respiratory Tract Infection (URTI), Gastrointestinal (GI) infection, or strenuous exercise.
- Characterized by IgA deposition in mesangium, leading to local inflammatory response.
- Immunofluorescence will show IgA, C3, and mesangial proliferation.
- The systemic form of IgA vasculitis is called Henoch-Schonlein purpura.
- Henoch-Schonlein purpura results in a purpuric rash in the extensors, polyarthritis, scrotal pain, GI bleeds, and nephritis.
Post-Streptococcal Glomerulonephritis
- Occurs 2-4 weeks after Group A beta haemolytic streptococcal infections such as pharyngitis and tonsillitis.
- Lead to rapidly progressive glomerulonephritis (RPGN).
- Diagnosed by measuring a positive anti-streptolysin - O titre (ASOT).
- Streptococcal antigen deposits in the glomerulus leads to immune complex formation and inflammation.
Membranoproliferative Glomerulonephritis
- It is a histological lesion rather than a specific disease entity.
- Involves immune complex deposition and complement system dysregulation.
- The above leads to inflammation and thickening of the glomerular basement membrane.
- Biopsy shows large glomeruli and mesangial proliferation.
- Microscopy of the membrane will show a "Tramline" double-basement membrane
Signs and Symptoms
- Presentation depends on the underlying cause.
- Symptoms include fatigue, malaise, and haematuria
- Other symptoms are decreased urine output (due to glomerular destruction) and a low-grade fever (infectious/autoimmune).
- Symptoms include features of vasculitis (purpuric rash and haemoptysis).
- Symptoms related to underlying conditions (e.g., Systemic Lupus Erythematousus).
- Frothy urine (proteinuria) may be present in those with advanced symptoms.
- Facial oedema/lower limb swelling may be present in those with advanced symptoms.
- Weight gain (oedema) may be present in those with advanced symptoms.
- Orthopnoea/Paroxysmal Nocturnal Dyspnea (pulmonary oedema) may be present in those with advanced symptoms.
- Facial oedema can occur, but not typically as severe as in nephrotic syndrome.
- Hypertension is a sign of Nephritic Syndrome
- Oedema, typically not as severe as in nephrotic syndrome occurs in those with Nephritic Syndrome
- Oliguria is a sign of Nephritic Syndrome
Differential Diagnosis
- Hematuria are Urinary tract infections, Renal stones, Renal tract malignancy
- Oedema are Nephrotic syndrome, Congestive cardiac failure, Liver failure
Clinical Evaluation
- Evaluate duration and progression of symptoms in medical history.
- Enquire about recent infections, new medications, and family history.
- Perform complete physical examination.
- Evaluate Vitals, Perform a Renal examination, Cardiac examination, and Respiratory exam.
Laboratory Investigation: Urinalysis
- Urine dipstick tests for hematuria and proteinuria.
- Microscopy & Culture can find:
- Active glomerular injury will present as Acanthocytes/ Red Cell casts.
- Assess for infectious causes.
- Albumin/Creatinine ratio & Protein/Creatinine ratio are measured to see the degree of glomerular damage
Laboratory Investigation: Blood Tests
- FBC - tests for Anaemia, Infection, and Eosinophilia
- U&E/Bone profile – tests GFR and Electrolyte disturbance
- Check albumin levels
- CRP - Active inflammation
Lab Investigation: Serological/Immunological Testing
- Perform ANA, ANCA, Anti-GBM, ANA, ENA, Anti-dsDNA
- Measure complement levels: C3 and C4
- Measure Rheumatoid Factor
- Test for HIV/HBV/HCV and ASOT
- Check Immunoglobulins & serum protein electrophoresis
Imaging
- Chest X-Ray: Pulmonary haemorrhage and Pulmonary oedema
- Renal Ultrasound: Assess size and look for Renal masses/stones/structural abnormality
- CT/MRI/Nuclear imaging: Used in specific cases
- Renal biopsy: Renal biopsy is required to diagnose conditions
- Diagnoses various types of glomerulonephritis.
- Provides prognostic information and influences treatment decisions.
- It shows histopathological information about the glomeruli, tubules, interstitium, and blood vessels
Management
- Supportive management is required
- Blood pressure needs to be controlled
- Target blood pressure should be <130/80.
- Use Diuretics/ACEI/ARB
- Need to Correct volume status
- Restrict water and salt intake.
- May require diuretics.
- Monitor patient with daily weight.
- Treatment: Depends on the underlying etiology and severity of the disease
- Initial goal: Achieve remission (induction) before altering treatment for maintenance.
- Immunosuppressive medications: Corticosteroids (methylprednisolone/prednisolone), Cyclophosphamide and Monoclonal antibody (Rituximab anti-CD20)
- Regular follow-up with Nephrologist is required to monitor the patients
- Blood pressure, Urea & Creatinine, Urine ACR/PCR
Prognosis
- Depends on disease aetiology and stage at diagnosis.
- All glomerular disease can progress to acute or chronic kidney disease.
- Twenty percent of IgA nephropathy cases result in ESKD at 20 years.
- Fifteen percent of HSP cases result in ESKD at 15 years.
- Post-Glomerular Nephritis (PGN) prognosis is excellent
Case Study
- For a 45-year-old male, who presents to the clinic with complaints of dark-colored urine, swelling in their hands and feet, and has joint pain and a rash on their lower extremities, no past medical history or medications, whose temperature is 37.3° C, who pulse is 88/min, respirations are 20/min, and blood pressure is 150/80 mm Hg. He displayed vascultic rash with small, palpable purpuric lesions. He was found to have:
- Serum Creatinine of 194 µmol/L
- Blood Urea Nitrogen (BUN) was 15.0 mmol/L
- Serum Potassium was 4.0 mmol/L
- Blood +3 protein +1 with RBC casts, WBC 10-15/HPF Urinalysis with no bacteria.
- Prednisolone a Corticosteroid is the best treatment option, because the patient has Henoch-Schonlein Purpura
Nephritic syndrome features glomerular destruction with haematuria, low level proteinuria, elevated serum creatinine and hypertension, this is often due to immune complex deposition on the glomerular basement membrane. Renal biopsy is required, it is managed by immunosuppressants, blood pressure control and volume correction and prognosis depends on the underlying cause.
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