Chapter 338 glomerulonephritis

Question 1

Classically Two human kidneys harbor nearly ____ glomerular capillary tufts

Answer 1

1.8 million

Question 2

classically present with hypertension, hema- turia, red blood cell casts, pyuria, and mild to moderate proteinuria.

Answer 2

Acute nephritic syndromes

Question 3

Poststreptococcal glomerulonephritis due to impetigo develops

Answer 3

2–6 weeks after skin infection

1–3 weeks after streptococcal pharyngitis.

Question 4

renal biopsy in poststreptococcal glomerulonephritis demon- strates

Answer 4

1. hypercellularity of mesangial and endothelial cells
2. glomerular infiltrates of polymorphonuclear leukocytes
3. granular subendothelial immune deposits of IgG, IgM, C3, C4, and C5–9
4. subepithelial deposits (which appear as “humps”)

Question 5

In PSGN, Five percent of children and 20% of adults have ___ in the nephrotic range.

Answer 5

proteinuria

Question 6

In PSGN, Positive cultures for streptococcal infection are inconsistently present which can help confirm the diagnosis

Answer 6

Cultures (10–70%)
increased titers of ASO (30%), anti-DNAse, (70%)*
antihyaluronidase antibodies (40%)

Question 7

Complete resolution of the hematuria and proteinuria in the majority of children occurs within _____ of the onset of nephritis

Answer 7

3–6 weeks

Question 8

In acute bacterial endocarditis it takes _____ to develop immune complex–mediated injury, by which time the patient has been treated, often with emergent surgery.

Answer 8

10–14 days

Question 9

kidneys have subcapsular hemorrhages with a “flea-bitten” appearance, and microscopy on renal biopsy reveals focal prolif- eration around foci of necrosis associated with abundant mesangial, subendothelial, and subepithelial immune deposits of IgG, IgM, and C3.

Answer 9

in subacute bacterial endocarditis

Question 10

in subacute bacterial endocarditis

Primary treatment is eradication of the infection with

Answer 10

4–6 weeks of antibiotics,

Question 11

In lupus nephritis, most common clinical sign of renal disease is ,

Answer 11

proteinuria

Question 12

is common in patients with acute lupus nephritis (70–90%) and declining complement levels may herald a flare.

Answer 12

Hypocomplementemia

Question 13

is the only reliable method of identifying the morphologic variants of lupus nephritis

Answer 13

renal biopsy

Question 14

designates mesangial immune complexes with mesangial proliferation

Answer 14

Class II lupus nephritis

Question 15

describes focal lesions with proliferation or scarring, often involving only a segment of the glomerulus
The most varied course

Answer 15

Class III FOCAL NEPHRITIS.

Question 16

describes global, diffuse proliferative lesions involving the vast majority of glomeruli.

have high anti- DNA antibody titers, low serum complement, hematuria, red blood cell casts, proteinuria, hypertension, and decreased renal function;

50% of patients have nephrotic-range proteinuria.

Answer 16

Class IV DIFFUSE NEPHRITIS

Question 17

Without treatment, this aggressive lesion has the worst renal prognosis.

Answer 17

Class IV DIFFUSE NEPHRITIS

Question 18

—defined as a return to near-normal renal function and proteinuria ≤330 mg/dL per day—is achieved with treatment, renal outcomes are excellent

Answer 18

remission

Question 19

describes subepithelial immune deposits produc- ing a membranous pattern;

Answer 19

class V lesion  
Membranous nephritis

Question 20

subcategory of class V lesions is associated with proliferative lesions and is sometimes called

Answer 20

mixed membranous and proliferative disease

Question 21

predisposed to renal-vein thrombosis and other thrombotic complications.

Answer 21

class V lesion  
Membranous nephritis

Question 22

90% sclerotic glomeruli and end-stage renal disease with interstitial fibrosis.

Answer 22

Class 6 sclerotic nephritis

Question 23

% of patients with lupus nephri- tis will reach end-stage disease, requiring dialysis or transplantation.

Answer 23

20%

Question 24

Patients who develop autoantibodies directed against glomerular basement antigens

Answer 24

Anti-GBM disease

Question 25

Anti-GBM present with lung hemorrhage and glomerulonephritis, they have a pulmonary-renal syndrome called

Answer 25

Goodpasture’s syndrome.

Question 26

Renal biopsies typically show focal or segmental necrosis that later, with aggressive destruction of the capillaries by cellular proliferation, leads to crescent formation in Bowman’s space

Answer 26

Goodpasture’s syndrome.

Question 27

presence of anti-GBM antibodies and complement is recognized on biopsy by

Answer 27

linear immunofluorescent staining for IgG (rarely IgA)

Question 28

In goodpastures syndrome or Anti-GBM Disease, Prognosis at presentation is worse if there are

Answer 28

> 50% crescents on renal biopsy with advanced fibrosis

if serum creatinine is >5–6 mg/dL

if oliguria is present

or if there is a need for acute dialysis.

Question 29

It is classically characterized by episodic hematuria asso- ciated with the deposition of IgA in the mesangium.

Answer 29

IgA nephropathy

Question 30

The two most common presentations of IgA nephropathy are

Answer 30

recurrent episodes of macroscopic hematuria during or immediately following an upper respiratory infection often accompanied by proteinuria or

persistent asymptomatic microscopic hematuria

Question 31

group of patients with small-vessel vasculitis (arterioles, capillar- ies, and venules; rarely small arteries) and glomerulonephritis have serum ANCA;

Answer 31

ANCA SMALL-VESSEL VASCuLITIS

Question 32

ANCA antibodies are of two types,

Answer 32

anti-proteinase 3 (PR3)

anti-myeloperoxidase (MPO)

Question 33

anti- bodies have also been reported experimentally as potentially patho- genic in ANCA

Answer 33

Lamp-2

Question 34

they are ANCA-positive and have a pauci-immune glomerulonephritis with few immune complexes in small vessels and glomerular capillaries.

Answer 34

Granulomatosis with polyangiitis

microscopic polyangiitis

Churg-Strauss syndrome

Question 35

Patients with this disease classically present with fever, purulent rhinorrhea, nasal ulcers, sinus pain, poly- arthralgias/arthritis, cough, hemoptysis, shortness of breath, micro- scopic hematuria, and 0.5–1 g/24 h of proteinuria;

Answer 35

Granulomatosis with Polyangiitis

Question 36

The disease is more common in patients exposed to silica dust and those with α1-antitrypsin deficiency, which is an inhibitor of PR3.

Answer 36

Granulomatosis with Polyangiitis

Question 37

Biopsy of involved tissue will show a small- vessel vasculitis and adjacent noncaseating granulomas

Answer 37

Granulomatosis with Polyangiitis

Question 38

distinction is made on biopsy, where the vasculitis in microscopic polyangiitis is without granulomas. Some patients will also have injury limited to the capillaries and venules.

Answer 38

Microscopic Polyangiitis

Question 39

When small-vessel vasculitis is associated with peripheral eosinophilia, cutaneous purpura, mononeuritis, asthma, and allergic rhinitis

Answer 39

Churg-Strauss Syndrome

Question 40

sometimes called mesangiocapillary glomerulonephritis or lobar glomerulonephritis.

It is an immune-mediated glomerulonephritis characterized by thickening of the GBM with mesangioproliferaive changes;

70% of patients have hypocomplementemia.

Answer 40

MEMBRANOPROLIFERATIVE GLOMERuLONEPHRITIS

Question 41

is commonly associated with persistent hepatitis C infections, autoimmune diseases like lupus or cryoglobulinemia, or neoplastic diseases

Answer 41

Type I MPGN

Question 42

characterized by expansion of the mesangium, sometimes associated with mesangial hyper- cellularity; thin, single contoured capillary walls; and mesangial immune deposits.

Answer 42

MESANGIOPROLIFERATIVE GLOMERuLONEPHRITIS

Question 43

classically presents with heavy proteinuria, minimal hematuria, hypoalbuminemia, hypercholesterolemia, edema, and hypertension.

Answer 43

Nephrotic syndrome

Question 44

causes 70–90% of nephrotic syndrome in childhood but only 10–15% of nephrotic syndrome in adults.

Electron microscopy, however, consistently demonstrates an effacement of the foot process supporting the epithelial podocytes with weakening of slit-pore membranes.

Answer 44

Minimal change disease (MCD),

Question 45

sometimes known as nil lesion,

Answer 45

Minimal change disease (MCD),

Question 46

presents clinically with the abrupt onset of edema and nephrotic syndrome accompanied by acellular urinary sediment.

Answer 46

Minimal change disease (MCD),

Question 47

In MCD, Average urine protein excretion reported in 24 h is ____ with severe hypoalbuminemia.

Answer 47

10 grams

Question 48

In MCD; Ninety to 95% of children will develop a complete remission after

Answer 48

8 weeks of steroid therapy

Question 49

In MCD, 80–85% of adults will achieve complete remission, but only after a longer course of

Answer 49

20–24 weeks.

Question 50

refers to a pattern of renal injury characterized by segmental glomerular scars that involve some but not all glomeruli

Answer 50

FSGS

FOCAL SEGMENTAL GLOMERULOSCLEROSIS

Question 51

most common cause of nephrotic syndrome in the elderly

Answer 51

Membranous glomerulonephritis (MGN)
Aka membranous nephropathy

Question 52

Uniform thickening of the basement membrane along the peripheral capillary loops is seen by light microscopy on renal biopsy

Answer 52

Membranous glomerulonephritis (MGN)
Aka membranous nephropathy

Question 53

syndrome develop hematuria, thinning and splitting of the GBMs, mild proteinuria (<1–2 g/24 h), which appears late in the course, followed by chronic glomeru- losclerosis leading to renal failure in association with sensorineural deafness.

Answer 53

ALPORTS SYNDROME

Question 54

Some patients develop lenticonus of the anterior lens capsule, “dot and fleck” retinopathy, and rarely, mental retardation or leiomyomatosis.

Answer 54

ALPORTS SYNDROME

Question 55

typically have thin basement membranes on renal biopsy which thicken over time into multilam- ellations surrounding lucent areas that often contain granules of varying density—the so-called split basement membrane.

Answer 55

Alport’s syndrome

Question 56

characterized by persistent or recurrent hematuria is not typically associated with proteinuria, hypertension, or loss of renal function or extrarenal disease.

Answer 56

THIN BASEMENT MEMBRANE DISEASE

Question 57

Another name for THIN BASEMENT MEMBRANE DISEASE

Answer 57

benign familial hematuria

Question 58

syndrome develop iliac horns on the pelvis and dysplasia of the dorsal limbs involving the patella, elbows, and nails, variably associated with neural-sensory hearing impairment, glaucoma, and abnormalities of the GBM and podocytes, leading to hematuria, proteinuria, and FSGS.

Answer 58

Nail patella syndrome

Question 59

renal biopsy there is lucent damage to the lamina densa of the GBM, an increase in collagen III fibrils along glomerular capillaries and in the mesangium, and damage to the slit- pore membrane, producing heavy proteinuria

Answer 59

Nail patella syndrome

Question 60

Provides 90% diagnostic in renal amyloidosis

Answer 60

Biopsy of liver or kidney

Question 61

Aka secondary amyloidosis

Answer 61

AA AMYLOIDOSIS

Question 62

Systemic AL AMYLOIDOSIS

Answer 62

Primary amyloidosis

Question 63

is an X-linked inborn error of globotriaosylceramide metabolism secondary to deficient lysosomal α-galactosidase A activity, resulting in excessive intracellular storage of globotriaosylceramide

Answer 63

Fabry’s disease