1. more common in developed countries 2. deposition of AMYLOID A PROTEIN 3. develops secondary to chronic inflammatory states (RA, TB, IBD, FMF, Lymphoma) 4. multiorgan failure

1. Autosomal dominant disorder (deformities of TRANSTHYRETIN) 2. Disorders of fibrinogen A alpha chain

Glomerular Nephritis Flashcards by John Fang

What are the most common causes of Secondary Nephrotic Syndrome

Diabetic Nephropathy
Renal Amyloidosis
Multiple Myeloma
HIV associated Nephropathy
Hepatitis B associated kidney disease

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What are clinical features of Diabetic Nephropathy

Decline in kidney fxn
HTN
retinopathy
peripheral neuropathy

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What is the pathophysiology of Renal Amyloidosis

Deposition of abnormal fibrillary structures of proteins

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What is the epidemiology of Renal Amyloidosis

Dx in 60s

slight predominance in males

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describe AL Amyloidosis

Most common systemic amyloidosis in USA

deposition of Ig LIGHT CHAIN

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describe AA Amyloid

more common in developed countries
deposition of AMYLOID A PROTEIN
develops secondary to chronic inflammatory states (RA, TB, IBD, FMF, Lymphoma)
multiorgan failure

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Describe AF Amyloid

Autosomal dominant disorder (deformities of TRANSTHYRETIN)
Disorders of fibrinogen A alpha chain

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AB2m

Hemodialysis associated amyloidosis

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What are the most common features of Renal Amyloidosis

Fatigue
Weight loss
purpura
Hepatosplenomegaly
Lymphadenopathy
Macroglossia
Urine LIGHT CHAIN
Increased Plasma cells

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What is Dx of Renal Amyloidosis

SERUM & URINE FREE LIGHT CHAINS

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What is the Light microscopy appearance of Renal Amyloidosis

CONGO RED positivity

crystal violet stains amyloid deposits in glomeruli and vessels

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What is the Immunostaining difference between amyloid AL and AA?

Amyloid AL: Anti-Ig Light chains (kappa, lambda)

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What is the electronmicroscopy of Renal amyloidosis

nonbranching irregular fibrils deposited in glomeruli, vessel walls, and extracellular spaces

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What is the treatment for AL Amyloid?

Chemo: high dose MELPHALAN, peripheral stem cell transplantation

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What is the treatment for AA Amyloid?

control chronic infections
Colchicine
Liver transplant

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What is the pathophysiology of Multiple Myeloma?

proliferation of plasma cells:

Coppt of IG LIGHT CHAINS + TAMM-HORSFALL Proteins = cast formation in DCT
tubularreabsorption of light chaings = INFLAMMATION + FIBROSIS

How does MM cause renal injury?

Nephrotic syndrome (Cast, Ig, FSGS nephropathy)
ATN

What are the clinical manifestation of MM?

Pain in lower back, Generalized malaise, Bruising

-minimal proteinuria

What is the Dx of MM?

(need 1 major and 1 minor or 3 minor)
major:
Bx: plasmacytoma; Bone marrow: 30% PLASMA CELLS; elevated monoclonal Ig in blood/urine
minor:
marrow - 10%-30%, minor Ig, Osteopenia/lytic lesions, Abnormally low Ab levels

What is the most important determinant of survival in MM?

kidney fxn

What are the clinical manifestions of HIV associated nephropathy?

Nephrotic Syndrome
Kidney failure (ESRD)
No HTN - Sodium wasting in PCT
Severity of HIVAN correlates with CD4 cell count

What is the Bx of HIVAN?

Collapsing glomerulopathy (prominent visceral epithelial cells/podocytes)

Microscystic dilation of tubules (PATHOGNOMONIC)
Tubulo-reticular bodies on EM

What are 4 common causes for Nephritis

Lupus nephritis
MembranoProliferative GN
IgA Nephropathy/HSP
Pulmonary-Renal Syndromes

What are some Sx for SLE?

Constitutional
Arthralgia, arthropathy
Dermatologic (malar rash)
AKI, CKD, Nephritic disease, Nephrotic syndrome
Seizure, psychosis

What happens to the glomerulus in Membranoproliferative GN?

1. Mesangial hypercellularity | 2. Endocapillary proliferation

What are the clinical features of Membranoproliferative?

1. Nephrotic Syndrome 2. Acute Nephritic syndrome 3. Asymptomatic proteinuria 4. Hematuria 5. ESRD in 10 years

What is the histological difference between Type I, II, III MPGN?

Type I: Immunocomplexes in subendothelial space, mesangium Type II: Dense Deposit Disease, deposits ain the mesangium, GBM, TBM and Bowman's capsule Type III: marked disruption of GBM w/ subendothelial and subepithelial electro-dense deposits

What are the two categories of MPGN based on etiology/pathogenesis?

1. Immune Complex-associated MPGN (ICM-MPGN) 2. Complement mediated MPGN-C3N, DDD - --2 = complement dysregulation

What are characteristics of CM-MPGN?

- LOW C3, Normal C4 - Acquired cause: circulating Auto-antibodies - Genetic cause: abnormalities in activating/regulating factors in CM

What are the autobodies directed against in CM-MPGN?

complement regulating factors: Factor H, Factor I, Factor B, convertase auto-Ab: C3NeF

What auto-Ab is predominantly found in patients affected by DDD (80%)

C3NeF (less frequently in C3GN, absent in CFHR5)

What is the activity of C3NeF

binds to C3 convertase prolonging its survival | C3NeF can stabilize C5 convertase

What does renal Bx look like in MPGN patients?

clear C3 staining with few/no immunoglobulin deposition

what cause C3 GN?

dysregulation and excessive activation of the ALT complement (low C3 and positive serum C3NeF)

What is the main cause of MPGN in the Middle East, South America, and Africa?

Chronic bacterial, viral, parasitic infections

What are are serum characteristics of ICM-MPGN?

circulating immune complexes seen in chronic infections (Hep B, C, parasites) Automimmune disease (SLE, RA)

What enzyme activity is decreased in patients with IgAN?

Leukocyte-b-1,3-galactosyl transferase

What is the clinical triad of Goodpasture's syndrome

(ANCA-negative vasculitis) 1. Pulmonary hemorrhage 2. glomerulonephritis 3. circulating Anti-GBM Abs