Nephritic Syndrome Study Set | U of U PA School Terms & Definitions

What is nephritis?

GLomerular inflammation

Incloudes acute nephritis and glomerulonephritis

What does nephritis cause?

Loss of integrity of glomerular structure and filtration barriers

What does loss of integrity of glomerular structures and filtration barriers lead to? (5)

Hematuria

-Dysmorphic RBCs with RBC casts

Moderate proteinuria

Hypertension

Oliguria (decreased GFR)

AKI

What causes nephritic syndrome?

Antigen antibody complexes activating inflammatory pathways that leads to accumulation of inflammatory cells and cytokines in glomerulus

This damages glomerular endothelium and filtration barriers leading to loss of integrity of glomerular structures and filtration barriers

How are types of nephritic syndrome differentiated?

Primary - kidney alone affected

Secondary - kidney affected as part of a systemic disease

What causes primary nephritic syndrome?

IgA nephropathy

Post infectious glomerulonephritis

Primary membranoproliferative glomerulonephritis

What is the most common cause of primary nephritic syndrome?

IgA nephropathy

What age is IgA nephropathy most common?

20s and 30s

What causes IgA nephropathy?

IgA deposits in mesangium leading to proliferative expansion of mesangium

How can IgA nephropathy present? (5)

Synpharyngitis

-Hematuria during or within 1 week of URI

Microhematuria

Hypertension

AKI

CKD

How is IgA nephropathy diagnosed?

Biopsy shows IgA deposits

Who is most at risk for post infectious glomerulonephritis?

Children 10-14 days after strep pharyngitis or 14-21 days after strep skin infections

How does post infectious glomerulonephritis present? (4)

Begins abruptly with hematuria that is cola colored

Proteinuria

Hypertension

Oliguria/decreased GFR - ranges from asymptomatic to severe oliguria AKI

How does post infectious glomerulonephritis happen?

Antigen antibody complexes and complement deposit in glomeruli causing glomerulonephritis and neutrophils to invade glomeruli

How is post infectious glomerulonephritis diagnosed?

Clinical

ASO titer to check for recent strep infections

Biopsy

What is the treatment for post infectious glomerulonephritis?

Treat symptoms

Self limited

What is membranoproliferative glomerulonephritis?

Glomerulonephritis + thickening of glomerular basement membrane + inflammation of glomerulus

What are the types of membranoproliferative glomerulonephritis?

Primary - no known cause

Secondary to

-Infections - Hep C most common

-Chronic infections - Endocarditis, visceral abscess, shunt nephritis

-Autoimmune

-Malignancy

How does membranoproliferative glomerulonephritis present? (4)

Elevated creatinine

Microscopic hematuria

Proteinuria

Hypertension

How is membranoproliferative glomerulonephritis diagnosed? How is it treated?

Biopsy

Treat underlying cause

-Often need immunosuppression

What are the secondary causes of nephritic syndrome? (4)

Lupus nephritis

Vasculitis

Anti glomerular basement membrane (GBM) nephritis

Infections

What are the symptoms of lupus nephritis? (4)

Photosensitivity (malar) rash

Arthritis

Can have nephritic and/or nephrotic syndrome

Can have acute or chronic renal failure

How does lupus nephritis kidney pathology present?

Mild to severe proliferative glomerulonephritis to membranous nephropathy to end stage scarred kidney

How is the severity of lupus nephritis determined/diagnosed?

Biopsy - to determine severity

Serology - ANA in blood

What type of vasculitis causes glomerulonephritis?

Small vessel

Most common is ANCA associated small vessel vasculitis

What are the types of small vessel vasculitis cause glomerulonephritis?

ANCA associated

-GPA - granulomatosis with polyangiitis - granulomatous necrosis

-MPA - microscopic polyangiitis - non granulomatous necrosis

-EGPA - eosinophilic granulomatosis with polyangiitis

What are ANCA? What does it cause?

Antibodies against enzymes in neutrophil granules

Causes premature diapedesis -> neutrophils release granules attracting cytokines -> causes blood vessel damage and tissue injury/necrosis

What type of ANCA is GPA? MPA?

GPA - c-ANCA

MPA - p-ANCA

What does GPA affect? (4)

Upper respiratory tract - nasal bridge collapse, nasal/throat/ear changes

Lungs - nodules, cavities, hemorrhage

Kidneys - necrotizing glomerulonephritis

Eye - major changes

What does MPA affect? (4)

Lungs - pulm hemmorhage

Kidneys - necrotizing glomerulonephritis

Nerves - nonneuritis multiplex

Skin - palpable purpura

How does ANCA associated small vasculitis present? How is it diagnosed? Treated?

Presentation

-Nephritic syndrome

-Rapidly progressing glomerulonephritis

Diagnosis

-Serology - ANCA testing

-Biopsy

Treatment

-Rapid immunosuppression

What is good pastures syndrome?

Anti GBM nephritis

Auto antibodies to type IV collagen in basement membrane

What do auto antibodies in good pastures bind?

Alveolar glomerular BM causing severe vascular damage

How does good pastures syndrome present?

Lungs - pulmonary hemorrhage

Kidneys - nephritis syndrome with rapidly progressing glomerulonephritis

How do good pasture syndrome and renal limited anti GBM neuritis differ?

Good pasture - lungs and kidney involved

How is good pastures syndrome diagnosed?

Serology - anti glomerular BM antibodies

Biopsy

How is good pastures syndrome treated?

Urgent plasmapheresis

Immunosuppression

What is rapidly progressing glomerulonephritis?

Crescentic glomerulonephritis (because of shape when biopsied)

Nephritic syndrome + rapid loss of kidney function declining over days to weeks

-Creatinine doubles in 6 weeks or less

What can cause rapidly progressing glomerulonephritis?

Any nephritic process if severe enough

ANCA related vasculitis and anti GBM nephritis most common causes

How does acute nephritic syndrome present? (4)

Decreased kidney function

Features of nephritis

-Hematuria

-Proteinuria

-Leukocytouria

What tests can help evaluate acute nephritic syndrome?

BMP

CBC w/ WBC count

What tests can diagnose acute nephritic syndrome? (9)

Serology for

-ASO

-ANA

-ANCA

-Anti GBM

-Hepatitis profile

Complement levels - C3/C4

-Decreased in immune complex disease like lupus or post infectious GN

-Normal in ANCA related GN, anti GBM disease

What are the complications of acute nephritic syndrome? (6)

Acute renal failure becoming chronic renal failure of unfixed

Hypertension

Hyperkalemia

Metabolic acidosis

Volume overload

Anemia

What is the treatment for acute nephritic syndrome?

General - treat consequences of decreasing kidney function and address complications

Treat underlying disease

When should nephritic syndrome be referred?

Always refer unless its a child that 100% has post infectious GN and responding to treatment

Urgent referral if rapidly progressive GN