SM_209a: Nephritis Flashcards
(32 cards)
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
