SM_209a: Nephritis Flashcards
Describe characteristics of nephritic syndromes
Nephritic syndromes
- Key feature: hematuria
- Other features: oliguria, azotemia, hypertension
- Common histomorphologic feature: proliferation of cells within the glomerulus and damage to GBM
- Result: RBC escape into urine
- Three most common entities: acute post-streptococcal glomerulonephritis, crescentic GN (rapidly progressive GN) types 1/2/3, IgA nephropathy
Key feature of nephritic syndromes is ______
Key feature of nephritic syndromes is hematuria
Describe acute post-streptococcal glomerulonephritis
Acute post-streptococcal nephritis
- Kids
- 1-6 weeks after a Group A (beta-hemolytic) strep infection
- Cola colored urine and low serum compliment compliment levels
- less severe cases self resolve and may not be biopsied
- LM: neutrophils and lymphocytes in glomerulus, fibrin thrombi
- FM: granular IgG and compliment in starry-sky pattern
- EM: dense subepithelial humps
_____ occurs 1-6 weeks after a Group A (beta-hemolytic) strep infection
Acute post-streptococcal glomerulonephritis occurs 1-6 weeks after a Group A (beta-hemolytic) strep infection
Acute post-streptococcal glomerulonephritis is characterized by _____ urine and low _____ levels
Acute post-streptococcal glomerulonephritis is characterized by Cola-colored urine and low serum compliment levels
On FM, acute post-streptococcocal glomerulonephritis manifests as _____
On FM, acute post-streptococcocal glomerulonephritis manifests as granular IgG and compliment in starry-sky pattern
On EM, acute post-streptococcocal glomerulonephritis manifests as ______
On EM, acute post-streptococcocal glomerulonephritis manifests as dense subepithelial humps
Describe rapidly progressive glomerulonephritis / crescentic glomerulonephritis
Rapidly progressive glomerulonephritis / crescentic glomerulonephritis
- Adults
- Rapid loss of renal function, rapidly proceeding to need for dialysis
- RPGN: clinical syndrome
- Crescentic GN: most common pathologic picture
- LM: crescents in most glomeruli - proliferation of parietal eptihelial cells of Bowman’s capsule
- FM and EM for further classification
- 3 types
RPGN is a ______, while crescentic GN is ______
RPGN is a clinical syndrome, while crescentic GN is the most common pathologic picture
RPGN manifests as ______ on LM due to ______
RPGN manifests as crescents in most glomeruli on LM due to proliferation of parietal epithelial cells of Bowman’s capsule
Describe RPGN type I
RPGN type I
- Anti-GBM disease: antibody recognizes collagen IV
- FM: linear IgG deposits with or without C3
- EM: no distinct deposits, antibody evenly distributed throughout GBM
- In many patients IgG also recognizes pulmonary basement membrane (Goodpasture syndrome): hematuria, hemoptysis
RPGN type I manifests as _____ on FM
RPGN type I manifests as linear IgG deposits with or without C3
RPGN type 1 in which the IgG also recognizes the pulmonary basement membrane is ______
RPGN type 1 in which the IgG also recognizes the pulmonary basement membrane is Goodpasture syndrome
Describe RPGN type II
RPGN type II
- Immune complex disease
- Complication of any autoimmune disorders which generate immune complexes (SLE, post-streptococcal glomerulonephritis, Henoch Schonlein purpura)
- FM: granular immune complex deposits with C3 and IgG or IgA
- EM: lumpy-bumpy appearance
RPGN type II manifests as ______ on FM
RPGN type II manifests as granular deposits of C3 with IgG or IgA on FM
Describe RPGN type III
RPGN type III (pauci-immune)
- 50% of crescentic GN cases
- FM: negative
- EM: no deposits
- 80% have ANCAs: anti-neutrophil cytoplasmic antibodies: myeloperoxidase (MPO, p-ANCA), proteinase-3 (PR3, c-ANCA), atypical ANCA (X-ANCA)
Describe RPGN type III with ANCA
RPGN type III with ANCA
- ANCA seen in vasculitides
- Systemic manifestations: purpura, ulcers, pulmonary hemorrhage, peripheral neuropathies, may have fever
- MPA (microscopic polyangiitis): vasculitis with neutrophols
- GPA (granulomatosis with polyangiitis): formerly called Wegener’s, vasculitis with neutrophils and granulomas
- EGPA (eosinophilic granulomatosis with polyangiitis): formerly called Churg-Strauss, vasculitis with neutrophils and granulomas with addition of asthma and eosinophilia
RPGN III is a _____ involving _____, _____, and _____
RPGN III is a necrotizing vasculitis involving fibrinoid necrosis, neutrophils, and thrombi
(scarred so will not regain function)
Describe IgA nephropathy (Berger disease)
IgA nephropathy (Berger disease)
- Kids and young adults, recurrent episodes of hematuri, first episode occurs after infection
- Abnormal IgA deposits at GBM
- Serum IgA increased, including immune complexes
- Pathogenesis: immune complexes lodge in kidney, activate complement, damage GBM, and cause RBC leakage into filtrate
- LM: mesangial proliferation, mesangial deposits, or crescents
- FM: granular IgA and C3 deposts in mesangium
- EM: mesangial deposits
IgA nephropathy (Berger disease) is characterized by ______
IgA nephropathy (Berger disease) is characterized by abnormal IgA deposits at GBM
IgA nephropathy manifests as ______ on FM
IgA nephropathy manifests as granular IgA and C3 deposits in mesangium on FM
Describe lupus nephritis
Describe lupus nephritis
- Classified into 6 classes which directly affect treatment: minimal changes -> active changes -> chronic changes with somewhat parallel increases in severity
- Full house on FM (including IgG, IgM, and IgA with C3 and C1q)
- Active lesions show hypercellularity, endocapillary fibrin, and apoptotic bodies
- Chronic changes: sclerosis (scarring) and crescents
Amyloid is _____
Amyloid is deposit of certain proteins capable of forming beta-pleated sheet polymers resistant to degradation that interferes with normal functions of the organ
Lupus nephritis manifests as ______ on FM
Lupus nephritis manifests as full house (IgG, IgM, IgA and C3 and C1q) on FM
Describe the classification system of lupus nephritis
Lupus nephritis classification system
Describe amyloidosis
Amyloidosis
- Clinical syndrome occurring as a result of amyloid (proteins capable of forming beta-pleated sheet polymers resistant to degradation) in various organ systems
- Deposition interferes with normal function of organ - filtration (glomerulus), reabsorption (GI), vascular compliance (vasculature), barrier function (bladder, skin)
- Currently classified according to protein type
Amyloid protein AA stands for _____ and occurs in cases of _____
Amyloid protein AA stands for Amyloid of Serum Amyloid A (SAA) and occurs in cases of chronic inflammation
(infectious or autoimmune)
Amyloid protein AL stands for _____ and usually occurs in _____
Amyloid protein AL stands for amyloid of light chains and usually occurs in lymphoid neoplasms
Amyloid protein AH stands for ______ and usually occurs in ______
Amyloid protein AH stands for amyloid of heavy chains and usually occurs in lymphoid neoplasms
Amyloid protein Abeta2M stands for ______ and is seen in ______ patients
Amyloid protein Abeta2M stands for amyloid of beta2-microglobulin and is seen in chronic hemodialysis patients
Amyloid protein ATTR stands for ______, is seen in ______, and has a propensity for the ______
Amyloid protein ATTR stands for amyloid of transthyretin, is seen in elderly or familial if mutated transthyretin, and has a propensity for the heart
Amyloid exhibits _______ on polarized light
Amyloid exhibits apple-green birefringence on polarized light
