Glomerular Disease / Nephrotic / Nephritic Flashcards
Proteinuria (>3.5g), Lipiduria, Fatty Casts, Edema, and HTN are all presentation of ______ syndrome
Nephrotic
Hematuria, RBC Casts, HTN, and Edema are all presentations of ______ syndrome.
Nephritic
When working up glomerulonephritis or nephrotic/nephritic syndromes…….
What would ANCA test for?
Anti-GBM?
ASO Titers?
SPEP/UPEP?
ANCA: Vasculitis, Wegner’s
Anti-GBM: Goodpasture’s
ASO: Strep
SPEP/UPEP: Multiple Myeloma
___ ________ is the most common lesion that causes primary glomerulonephritis.
This typically occurs 2-4 days after a URI of GI infection that results in the overproduction of an immune complex
IgA Nephropathy (Berger Disease)
Is IgA Nephropathy a nephritic or nephrotic syndrome?
Proliferative or non-proliferative glomerulonephritis?
Nephritic
Proliferative
What is seen on histology in IgA Nephropathy?
This is also diagnostic
Large, Globular IgA deposits in the mesangium
What is the classic presentation of a patient with IgA Nephropathy?
Hematuria following a recent URI
Could microscopic hematuria and mild proteinuria be seen in IgA Nephropathy?
Yes
If a patient with IgA Nephropathy was becoming HTN, which anti-hypertensive medications would you recommend?
ACEi
ARB
If a patient with CVD and IgA Nephropathy developed HLD, what medication class would you recommend?
Statin
_________ glomerulonephritis is the cause of 1/3rd of all non-DM associated nephrotic syndromes and is commonly idiopathic.
However, it is also associated with Hep B, SLE, Malignancy, and NSAIDs.
Membranous Glomerulonephritis (MGN)
Is Membranous Glomerulonephritis (MGN) a nephritic or nephrotic syndrome?
Proliferative or non-proliferative glomerulonephritis?
Nephrotic
Non-Proliferative
What is commonly seen on histology in a patient with MGN?
Diffuse thickening of the glomerular basement membrane
What may been seen on histology in a patient with immune mediated MGN?
Diffuse, Granular IgG deposition in the capillary walls
If you were to preform a silver stain on a patient with MGN, what may you see that would be diagnostic?
Spike Appearance
T:F: MGN is mostly idiopathic so it is NOT important to rule out secondary causes?
False
What transmembrane receptor expressed in the podocytes has been identified as a major antigen in idiopathic MGN?
Phospholipase A2 receptor
The M-type phospholipase A2 receptor (PLA2R)
How is MGN treated in patients who are most at risk for developing progressive disease?
What are these risk factors?
Immunosupressants
- Older age at presentation
- Male
- Nephrotic Proteinuria (>8-10 g/day)
- Increased sCr at presentation
What percentage of patients with MGN will develop ESRD?
1/3rd
On histology…..
A lobular Appearance Increased Glomerular Cellularity Mesangial Expansion Narrow Capillary Lumens An a thickening of glomerular capillary walls
Would all indicate _____________ glomerulonephritis
Membranoproliferative
Is membranoproliferative glomerulonephritis a nephritic or nephrotic syndrome?
Proliferative or non-proliferative glomerulonephritis
Nephritic
Proliferative
T/F: Membranoproliferative glomerulonephritis is classifed as being mediated by immune complexes and complex dysregulation that ultimately leads to endothelial injuries
True
When working up membranoproliferative glomerulonephritis what is important to rule out?
Hep B
Hep C
What is the most common cause of acute nephritis in children?
Particularly what a result of what?
Post-Infectious Glomerulonephritis
Group A, Beta-Hemolytic Streptoccocus
(Often referred to as PSGN)
Is post-infectious glomerulonephritis a nephritic or nephrotic syndrome?
Proliferative or non-proliferative glomerulonephritis
Nephritic
Proliferative
How soon after a strep infection would you expect to see PSGN to manifest?
10-14 days
PSGN histology appears as diffuse proliferative disease that is often describe as a “______ _____” appearance.
Starry Night
T/F: PSGN can present as asymptomatic microscopic hematuria?
True
PSGN can present in a full blown nephritic syndrome…
What are the symptoms of this?
Red-Brown Urine Proteinuria (up to nephrotic levels) Edema HTN AKI
What labs are most diagnostic for PSGN?
ASO Titers and Low Serum Compliment
How is PSGN treated and managed?
Supportive Care
Abx (Considered)
Rapidly Progressive GN is diagnosed by a _______ formation on biopsy.
What are TWO examples RPGN?
Crescentric
Goodpasture’s
Henoch-Schonlein Purpura
RPGN on histology can present as a cellular crescent or fibrotic crescent appearance.
Which has the worse prognosis?
Fibrotic
_________ _______ is a RPGN that effects Type IV collagen of the kidney and is commonly associated with manifestations in the lungs
Goodpasture’s Disease
Other than a crescentric appearance, what would be seen on histology in a patient with Goodpasture’s?
Linear Deposition of IgG in Anti-GBM antibodies
What is the treatment of choice for Goodpasture’s Disease?
What medications are often given in combination with this?
Plasmaphoresis
PO Prednisone
Cyclophophamide (Immunosupression)
T/F: If left untreated, Acute GN due to Goodpasture’s has a good prognosis
False
Very poor prognosis if left untreated
Patients with Goodasture’s Disease often present with proteinuria, does this reach nephrotic range?
No
Would you expect to see dysmorphic RBCs and WBCs in Goodpasture’s Disease?
Yes
_______-_______ _______ is an IgA vasculitis which is the most common form of vasculitis in children?
Henoch-Schonlein Purpura (HSP)
What is the most common presentation in a patient with Henoch-Schonlein Pupura (HSP)?
What else may be present?
Palpable Purpura (WITHOUT thrombocytopenia or coagulopathy)
Arthritis
Abdominal Pain
Renal Disease
T/F: Leukocytoclastic vasculitis with IgA Immune complexes is charateristic of HSP
True
Treatment of HSP can be breakdown into two categories (Supportive and Symptomatic)….
What is involved in each?
Supportive:
IVF
Rest
Symptomatic:
Treat joint pains with NSAIDs and Acetaminophen
_____ ______ _______ results in 90% of nephrotic disease in children and is an idiopathic disease of the podocytes.
Minimal Change Disease
Should you expect to see immune deposits on histology in a patient with minimal change disease?
No
Would you expect to see diffuse effacement of the foot processes in minimal change disease?
Yes
Although minimal change disease is primarily idiopathic…..
What medications are associated with minimal change disease?
NSAIDs
COX2
Lithium
Bisphosphinates
What CA is commonly seen with minimal change disease?
Hodgkin’s Lymphoma
If minimal change disease were a result of infection….
How would this be treated?
Vaccinations
How does minimal change disease clinically present?
What would you see on UA?
Abrupt onset of drastic edema and weight gain
UA: High levels of proteinuria
Is a biopsy warranted to work up minimal change disease?
When would it be warranted?
No, it is primarily diagnosed clinically
If a patient was unresponsive to therapy
How is minimal change disease treated without immunosupressants?
What is used as immunosupression therapy?
Without immunosupression…..
Low NA+ diet
Diuretics
ACEi
Immunosupression….
Prednisone
______ ________ __________ is a disease of the ppdocytes that is classified as non specific trapping of immunoglobulin C3 and IgM not thought to be pathogenic.
It is associated with circulating factor: Soluble Urokinase Receptor (SuPAR)
Focal Segmental Glomerulosclerosis (FSGS)
In African Americans, FSGS involves what gene?
Which chromosome is this gene found on?
APOL1 Gene
Chromosome 22
How does FSGS commonly present/manifest?
Proteinuria
Microscopic Hematuria
HTN
Renal Insufficiency
What is the most determinate factor in the prognosis of FSGS?
Response to therapy
Is FSGS treated with immunospressive therapy?
What is primarily used?
No
Glucocorticoids
T/F: Kidney transplant is a curative treatment option for FSGS
False
FSGS returns to the transplanted kidney up to 40% of the time
What are FOUR causes of Secondary Glomerulonephritis?
DM
SLE
HIV
Amyloidosis
Would you expect to see diabetic nephropathy in Type 1 or 2 DM?
Type 1 DM
What is the classic clinical manifestation of diabetic nephropathy?
Microalbuminuria (30-300 mg/d) n the setting of CKD
If a patient developed macroalbuminuria (>300 mg), what would they be more at risk of developing?
ESRD
What are unique features of diabetic nephropathy of histology?
What lesion is unique to diabetic nephropathy?
Mesangial Expansion
Glomerular Basement Membrane thickening
Glomerular Sclerosis
Kimmelstiel-Wilson Lesion
How is diabetic nephropathy manged/treated?
- BG Control
- BP Control (ACEi, ARB, Diuretics)
- Avoid NSAIDs
- Low Na+ Diet
_______ _______ ________ is a cause of secondary glomerulonephritis that is a chronic inflammatory disease of unknown cause that can affect the skin, joints, kidneys, lungs, nervous system, serous membranes,and/orother organs of the body
Systemic Lupus Erythematosis (SLE)
SLE results in renal disease when Anti-____ imune complexes form and deposit in the ______ and _________ space.
Anti-DNA
Mesangium
Subendothelial Space
How would SLE renal disease present on histology
Renal Artery Hyalinosis
Increased mesangial matrix
Cellular proliferation
Thickening of glomerular capillary walls
How is SLE and renal disease treated?
Glucocorticoids in combination with…..
Mycophenolate Mofetil (MMF)
or
Cyclophosphamide
If a patient with SLE nephritis relapses, what could be used?
Prednisone
+
MMF or Azathioprine
___-_______ ______ is an infection of the kidney epithelial cells by HIV and expression of the HIV gene within the cells.
HIV-associated nephropathy (HIVAN)
In a patient with HIVAN….
What is the CD4 count often?
Would they have a nephritic or nephrotic range of proteinuria?
CD4 count was often less than 200 (64%)
Nephrotic Range (~4.1 g/day)
T/F: HAART is used in HIVAN treatment
True
Although no randomized trials have specifically examined this
If a patient with HIVAN also had HTN, what would you recommend as treatment?
ACEi
ARB
________ is a cause of secondary glomerulonephritis that is characterized by extracellular deposition of beta-sheet fibrils that can lead to organ dysfunction and death
Amyloidosis
If the following was seen on histology what would it indicate….
Nodular amorphous material extending into the mesangium and into the capillaries
Renal Amyloid
T/F: In primary renal amyloid, the degree of proteinuria can be > 20g
True
What is used to treat secondary renal amyloid?
Colchicine
Tx underlying Inflammatory Process
