Podcast
Questions and Answers
What is the primary method for diagnosing amyloid in affected individuals?
What is the primary method for diagnosing amyloid in affected individuals?
Which treatment is considered reasonable for younger patients with predominantly renal involvement due to AL amyloid?
Which treatment is considered reasonable for younger patients with predominantly renal involvement due to AL amyloid?
In the context of AA amyloid, which treatment focuses on managing the underlying inflammatory disease process?
In the context of AA amyloid, which treatment focuses on managing the underlying inflammatory disease process?
What condition is characterized by the precipitation of filtered light chains within the tubule lumen in myeloma?
What condition is characterized by the precipitation of filtered light chains within the tubule lumen in myeloma?
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What supportive care measure might be recommended for patients with nephrotic syndrome due to amyloid?
What supportive care measure might be recommended for patients with nephrotic syndrome due to amyloid?
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What is the percentage range of unselected lupus patients that have clinical renal disease at disease onset?
What is the percentage range of unselected lupus patients that have clinical renal disease at disease onset?
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Which autoantibody is considered more specific but less sensitive for systemic lupus erythematosus?
Which autoantibody is considered more specific but less sensitive for systemic lupus erythematosus?
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What is the diagnostic definition of renal involvement in systemic lupus erythematosus?
What is the diagnostic definition of renal involvement in systemic lupus erythematosus?
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What major factor besides immune complex deposition may exacerbate renal damage in lupus nephritis?
What major factor besides immune complex deposition may exacerbate renal damage in lupus nephritis?
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According to the most recent WHO classification, how many categories are used to classify lupus kidney biopsies?
According to the most recent WHO classification, how many categories are used to classify lupus kidney biopsies?
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What is the hallmark of systemic lupus erythematosus?
What is the hallmark of systemic lupus erythematosus?
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Why does the ANA titer not correlate well with renal involvement severity in SLE?
Why does the ANA titer not correlate well with renal involvement severity in SLE?
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What is a common pathway of pathophysiology in thrombotic microangiopathy (TMA)?
What is a common pathway of pathophysiology in thrombotic microangiopathy (TMA)?
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Which of the following laboratory features is essential for diagnosing HUS/TTP?
Which of the following laboratory features is essential for diagnosing HUS/TTP?
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What disease complicating infection with shiga toxin-producing Escherichia coli (STEC) is a significant cause of acute renal failure in children?
What disease complicating infection with shiga toxin-producing Escherichia coli (STEC) is a significant cause of acute renal failure in children?
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ADAMTS13 is linked to which condition in thrombotic microangiopathy?
ADAMTS13 is linked to which condition in thrombotic microangiopathy?
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What is a prominent neurologic symptom associated with HUS/TTP?
What is a prominent neurologic symptom associated with HUS/TTP?
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Which clinical finding is NOT typically considered in diagnosing HUS/TTP?
Which clinical finding is NOT typically considered in diagnosing HUS/TTP?
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What laboratory feature is common in patients with thrombocytic microangiopathy?
What laboratory feature is common in patients with thrombocytic microangiopathy?
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Which pathway is often dysregulated in aHUS?
Which pathway is often dysregulated in aHUS?
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What type of anemia is primarily associated with microangiopathic hemolytic anemia?
What type of anemia is primarily associated with microangiopathic hemolytic anemia?
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Which of the following is a common symptom seen in patients presenting with HUS/TTP?
Which of the following is a common symptom seen in patients presenting with HUS/TTP?
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What factor significantly increases the risk of worsening renal function in pregnant patients with lupus nephritis?
What factor significantly increases the risk of worsening renal function in pregnant patients with lupus nephritis?
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Which medication is contraindicated in pregnant patients with lupus nephritis?
Which medication is contraindicated in pregnant patients with lupus nephritis?
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What percentage of patients with lupus nephritis progress to end-stage renal disease (ESRD)?
What percentage of patients with lupus nephritis progress to end-stage renal disease (ESRD)?
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What unique serologic feature is typically found in patients with mixed connective tissue disease (MCTD)?
What unique serologic feature is typically found in patients with mixed connective tissue disease (MCTD)?
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Which of the following is true regarding allograft survival rates in patients with ESRD due to lupus nephritis?
Which of the following is true regarding allograft survival rates in patients with ESRD due to lupus nephritis?
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What clinical manifestation is most indicative of severe hypertension and acute kidney injury in patients with MCTD?
What clinical manifestation is most indicative of severe hypertension and acute kidney injury in patients with MCTD?
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In the treatment of renal involvement in MCTD, what aspect of the therapy is generally emphasized?
In the treatment of renal involvement in MCTD, what aspect of the therapy is generally emphasized?
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Which condition is associated with a risk of fetal loss exceeding 50% in pregnant women with lupus nephritis?
Which condition is associated with a risk of fetal loss exceeding 50% in pregnant women with lupus nephritis?
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What is the primary clinical feature that characterizes glomerular lesions in mixed connective tissue disease?
What is the primary clinical feature that characterizes glomerular lesions in mixed connective tissue disease?
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What is a characteristic feature observed in the peripheral smear of a patient with the laboratory findings described?
What is a characteristic feature observed in the peripheral smear of a patient with the laboratory findings described?
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In primary (AL) amyloidosis, from which component are the deposited fibrils primarily derived?
In primary (AL) amyloidosis, from which component are the deposited fibrils primarily derived?
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Which laboratory finding typically indicates hemolysis in the context provided?
Which laboratory finding typically indicates hemolysis in the context provided?
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What does the presence of para-protein spike in serum or urine indicate in amyloidosis?
What does the presence of para-protein spike in serum or urine indicate in amyloidosis?
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What is one of the most common presenting symptoms of amyloidosis?
What is one of the most common presenting symptoms of amyloidosis?
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Which statement regarding the demographics of amyloidosis is accurate?
Which statement regarding the demographics of amyloidosis is accurate?
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Which of the following is NOT a typical finding in primary amyloidosis?
Which of the following is NOT a typical finding in primary amyloidosis?
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Thrombocytopenia in the described laboratory findings generally indicates which condition?
Thrombocytopenia in the described laboratory findings generally indicates which condition?
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Which organ is the most common site of involvement in primary (AL) amyloidosis?
Which organ is the most common site of involvement in primary (AL) amyloidosis?
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What type of amyloidosis is associated with chronic inflammatory states?
What type of amyloidosis is associated with chronic inflammatory states?
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Study Notes
System Vasculitis and Kidney Damage
- System vasculitis, connective tissue diseases, and kidney damage are interconnected.
- Kidney amyloidosis is a concern.
- Thrombotic microangiopathies, paraproteinemic diseases, and myeloma are also related.
Primary Glomerulopathies
- Acute proliferative glomerulonephritis (post-infectious and other types) are addressed.
- Rapidly progressive glomerulonephritis, membranous glomerulopathy, minimal-change disease, focal segmental glomerulosclerosis, and membranoproliferative glomerulonephritis, IgA nephropathy are all specific types.
- Chronic glomerulonephritis is also mentioned.
Systemic Diseases with Glomerular Involvement
- Systemic lupus erythematosus, diabetes mellitus, and amyloidosis are discussed.
- Goodpasture syndrome, microscopic polyangiitis/polyangiitis, Wegener granulomatosis, Henoch-Schönlein purpura, and bacterial endocarditis are also mentioned.
- Hereditary disorders like Alport syndrome, thin basement membrane disease, and Fabry disease are included.
Lupus Nephritis (LN)
- Lupus nephritis (LN) is a frequent and potentially serious complication of systemic lupus erythematosus (SLE).
- Renal involvement worsens morbidity and mortality rates in lupus.
- Approximately 25% to 50% of unselected lupus patients have clinical renal disease at disease onset, and up to 60% of adults with SLE develop renal disease during their lifetime.
- Renal involvement in SLE is defined by persistent proteinuria exceeding 500 mg daily or the presence of cellular casts.
- Glomerular and vascular damage is potentiated by hypertension and a thrombotic microangiopathy.
Histopathologic Patterns in LN
- Renal biopsies in LN can reveal a number of histopathologic patterns.
- Overlapping patterns and/or histologic progression can occur.
- The World Health Organization (WHO) has a classification system for biopsies.
- Biopsies are classified into six categories based on glomerular changes under light microscopy. (See table 12-1 for details)
Lupus Nephritis (LN) Classification
- The WHO 2003 classification breaks down lupus nephritis biopsies into six classes based on light microscopy findings.
- Each class has a specific description as to glomerular changes. (See table 12-1 and 12-2 for detailed descriptions.)
Immunopathologic Findings in SLE
- Abnormal autoantibody production is a hallmark of SLE.
- Antinuclear antibodies (ANAs), and especially anti-DNA, are criteria for SLE and help monitor the course of the disease.
- Anti-dsDNA, a more targeted marker for SLE, is more specific, but less sensitive than ANA.
- Anti-Sm, anti-RNP, anti-Ro/serum amyloid A, and anti-La/SSB are other autoantibodies present in lupus but are not as predictive of renal involvement.
Pregnancy and Lupus Nephritis
- Pregnancy in patients with LN has been associated with worsening of renal function.
- Risk factors include active disease in the previous 6 months, hypertension, proteinuria, and baseline renal impairment.
- The risk of fetal loss is elevated, potentially exceeding 50%.
- High doses of corticosteroids and azathioprine are sometimes used, although mycophenolate and cyclophosphamide are contraindicated in pregnancy.
Renal Disease from Lupus Nephritis
- Patients diagnosed with LN, who develop end-stage renal disease have remission of their extrarenal manifestations as well as serological activity.
- Renal transplant programs may allow patients to have a dialysis period (3 to 12 months) to allow the disease to calm down before a transplant to improve the likelihood of the transplant being successful.
Treatment Options for Lupus Nephritis
- Treatment options for lupus nephritis are stratified by International Society of Nephrology classification and phase of therapy.
- Specific induction and maintenance phases of therapy exist.
- Prescribed medications, including but not limited to steroids, mycophenolate mofetil, cyclophosphamide, azathioprine and other immune suppressants are included for different classes of lupus nephritis.
Mixed Connective Tissue Disease (MCTD)
- MCTD shares overlapping characteristics with SLE, scleroderma, and polymyositis.
- A high ANA titer with speckled pattern and specific ribonuclease-sensitive ENA antibodies are often present in MCTD patients.
- Renal involvement is often mild to minimal.
Primary Systemic Vasculitides
- Various types of primary systemic vasculitis are illustrated, including small, medium, and large vessel vasculitides.
- Distinct descriptions of each exist, including clinical characteristics and organ involvement. (Tables 1 and 2 display various forms.)
Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (AAV)
- AAV is a group of autoimmune disorders (GPA, MPA, and EGPA).
- It involves inflammation of small blood vessels.
- Diagnosis relies on ANCA testing, and clinical presentations.
- Treatment includes immunosuppressants like glucocorticoids, cyclophosphamide, and other agents.
Thrombotic Microangiopathy (TMA)
- TMA is a group of disorders characterized by microvascular damage, thrombosis, and hemolysis.
- It includes conditions like HUS and TTP.
- Hallmarks include thrombocytopenia, hemolytic anemia, and schistocytes on blood smears.
- Underlying factors (infections, medications, etc.) can trigger TMA.
Hemolytic Uremic Syndrome (HUS)
- HUS is a type of TMA, often triggered by shiga toxin-producing E.coli (STEC) infection.
- It leads to acute kidney failure, neurologic symptoms, and potentially serious complications.
- Diagnosis requires evaluation of ADAMTS13 levels and testing for STEC to distinguish from atypical HUS.
Amyloidosis
- Amyloidosis involves the abnormal deposition of protein fibrils in various organs.
- Primary (AL) amyloidosis results from immunoglobulin light chain deposition from plasma cells, while secondary (AA) amyloidosis develops in chronic inflammatory conditions.
- Diagnostics frequently involve tissue biopsies.
Waldenstrom Macroglobulinemia
- Waldenstrom macroglobulinemia, a rare blood cancer, features the accumulation of IgM monoclonal proteins.
- It can lead to hyperviscosity syndrome and other symptoms (fatigue, bleeding, neuropathy, etc).
- Treatment focuses on targeting the underlying lymphoproliferative disease and/or addressing hyperviscosity symptoms.
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Description
This quiz covers the interconnections between systemic vasculitis and various forms of kidney damage, including conditions like amyloidosis and glomerulonephritis. It explores both primary glomerulopathies and systemic diseases that affect the kidneys, such as lupus and diabetic nephropathy.
