SM 209 Nephritis Flashcards
What are the 3 Nephritic syndromes?
Acute Post Streptococcal Glomerulonephritis (PSGN)
Rapidly Progressive Glomerulonephritis (RPGN)
IgA Nephropathy
What is the key feature of a Nephritic syndrome?
Hematuria, both micro or macro
What other features besides Hematuria are found in Nephritic syndromes?
Oliguria, HTN, Azotemia
Why is HTN a response in Nephritic syndromes?
Kidney senses poor blood flow due to inflammation in the Glomerulus, activating RAAS to compensate and resulting in HTN
What is the common histomorphographic feature of Nephritic syndromes?
Proliferation of cells in the glomerulus and damage to the GBM
Why are RBC’s in the urine in Nephritic syndromes damaged?
The urine environment is electrolytically harsh and causes lysis
How many types of Rapidly Progressive Glomerulonephritis are there?
3: Type I, II, and III
Who gets affected by PSGN?
Kids 2 weeks after a Group A Beta-hemolytic Strep infection
What does PSGN urine look like?
Cola-colored urine
How does PSGN effect serum complement levels?
Low serum complement
A child presents with cola-colored urine and low serum complement levels. Diagnosis?
Post Streptococcal Glomerulonephritis
How does PSGN present on fluorescent microscopy?
Starry-Sky with IgG and Complement
What disease presents with IgG and Complement in a starry-sky pattern on fluorescent microscopy?
PSGN
What disease presents with subepithelial humps on electron microscopy?
PSGN
How does PSGN appear on electron microscopy?
Subepithelial humps
Who gets RPGN?
Adults
How does RPGN present?
Rapid loss of renal function that needs dialysis
Is RPGN a disease?
RPGN is a clinical syndrome
What is seen on slides with RPGN?
Lots of Crescents
What causes anti-GBM disease?
An antibody against Collagen Type IV
What does RPGN look like on EM?
No distinct deposits because the antibody is evenly distributed throughout the GBM
How does RPGN Type I appear on FM?
Linear IgG deposits +/- C3
What is Goodpasture syndrome?
IgG in RPGN Type I binds to both the glomerular basement membrane as well as the pulmonary basement membrane
How does Goodpasture syndrome present?
Hematuria and Hemoptysis
RPGN Type I
Which type of RPGN causes Goodpasture syndrome?
Type I RPGN
What is RPGN Type II?
An immune complex disease
Which type of RPGN arises as a complication of autoimmune disorders that make immune complexes?
RPGN Type II
How is RPGN Type II described on EM?
Lumpy Bumpy appearance
What disease appears as Lumpy Bumpy on EM?
RPGN Type II
What types of immune deposits are in RPGN Type II?
C3 + IgG or IgA
Which type of RPGN is Pauci-immune?
RPGN Type III
What is the most common type of RPGN?
RPGN Type III
What type of RPGN has ANCA’s?
ANCA’s = anti-neutrophil cytoplasmic antibodies
RPGN Type III
What is RPGN Type III?
A type of RPGN without deposits on EM and negative on FM
What are the subtypes of RPGN Type III?
MPA - Vasculitis w/ Neutrophils
GPA - Vasculitis w/ Neutrophils + Granulomas
EGPA - Vasculitis w/ Neutrophils + Granulomas + Eosinophilia
What is MPA?
A subtype of RPGN Type III that presents as Vasculitis w/ Neutrophils
What is GPA?
A subtype of RPGN Type III that presents as Vasculitis w/ Neutrophils + Granulomas
What is EGPA?
A subtype of RPGN Type III that presents as Vasculitis w/ Neutrophils + Granulomas + Eosinophilia
In which disease are ANCA seen?
ANCA are seen in vasculitis
What type of vasculitis is RPGN Type III?
RPGN is a necrotizing vasculitis that involves a Glomeruli that involves fibrinoid rings forming around blood vessels
Who is affected by IgA Nephropathy?
Kids and young adults
How does IgA Nephropathy present?
Recurrent episodes of Hematuria, typically beginning after an infection
What characterizes IgA deposits in IgA Nephropathy?
IgA deposits at the GBM
How do serum IgA levels change in IgA Nephropathy?
Serum IgA levels rise
How does IgA in IgA Nephropathy damage the kidney?
IgA lodges in the Kidney and activate complement, damaging the GBM and causing RBC leakage into urine
How does IgA Nephropathy affect the appearance of glomerulus on slides?
The Tree of the Glomerulus expands
What is Systemic Lupus Erythematous?
An autoimmune disease of unknown etiology
Which organ systems does SLE effect?
Inflammation of multiple organ systems is characteristic of SLE
How is Lupus Nephritis classified?
It is classified by the ISN system into 6 classes
What sets the treatment of Lupus Nephritis?
The class of the Lupus Nephritis sets the treatment
How do the changes caused by Lupus Nephritis effect severity and treatment?
At higher classes, Lupus has more drastic changes which have more severe symptoms and need more aggressive treatment
How does Lupus Nephritis present on fluorescent microscopy?
A “full house” of IgG, IgM, IgA, C3, C1q
What are active lesions in Lupus Nephritis?
Active lesions are hypercellularity and endocapillary fibrin deposits
What are chronic changes in Lupus Nephritis?
Old injures that lead to scarring and crescents
Which stage of Lupus cause “spike and dome” appearance on EM?
Class V Lupus Nephritis
Which class of Lupus Nephritis is chronic?
Class VI Lupus Nephritis
What is Amyloidosis?
A clinical syndrome that occurs due to deposition of Amyloid in various organ systems
What is Amyloid?
Deposits of proteins that are capable of forming beta-pleated sheet polymers that resist degradation
How does deposition of Amyloid effect normal physiology?
Deposition of Amyloid leads to interference with the normal function of the organ
How is Amyloid classified?
Amyloid is classified by protein type
What is AA Amyloid?
Amyloid of Serum Amyloid A; occurs in chronic inflammation both infectious and autoimmune
What is AL Amyloid?
Amyloid of light chains (from IgG) that occurs in Lymphoid neoplasms
What is AH Amyloid?
Amyloid of heavy chains (from IgG) that occurs in Lymphoid neoplasms
What is Ab2M Amyloid?
Amyloid of beta2-microglobulin, seen in chronic hemodialysis patients because it’s not filtered
What is ATTR Amyloid?
Amyloid of Transthyretin, seen in elderly or familial with a propensity for the heart
Which type of Amyloid proteins are associated with Lymphoid neoplasms?
AH and AL are associated with Lymphoid neoplasms (both are part of the Ig)
Which type of Amyloid proteins are associated with Lupus and Hep B/C?
Lupus and Hep B/C are sources of chronic inflammation which predispose AA
How is Amyloid stained?
Apple-green appearance on Congo Rod stain
