Chapter 338 glomerulonephritis Flashcards
Classically Two human kidneys harbor nearly ____ glomerular capillary tufts
1.8 million
classically present with hypertension, hema- turia, red blood cell casts, pyuria, and mild to moderate proteinuria.
Acute nephritic syndromes
Poststreptococcal glomerulonephritis due to impetigo develops
2–6 weeks after skin infection
1–3 weeks after streptococcal pharyngitis.
renal biopsy in poststreptococcal glomerulonephritis demon- strates
- hypercellularity of mesangial and endothelial cells
- glomerular infiltrates of polymorphonuclear leukocytes
- granular subendothelial immune deposits of IgG, IgM, C3, C4, and C5–9
- subepithelial deposits (which appear as “humps”)
In PSGN, Five percent of children and 20% of adults have ___ in the nephrotic range.
proteinuria
In PSGN, Positive cultures for streptococcal infection are inconsistently present which can help confirm the diagnosis
Cultures (10–70%)
increased titers of ASO (30%), anti-DNAse, (70%)*
antihyaluronidase antibodies (40%)
Complete resolution of the hematuria and proteinuria in the majority of children occurs within _____ of the onset of nephritis
3–6 weeks
In acute bacterial endocarditis it takes _____ to develop immune complex–mediated injury, by which time the patient has been treated, often with emergent surgery.
10–14 days
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.
in subacute bacterial endocarditis
in subacute bacterial endocarditis
Primary treatment is eradication of the infection with
4–6 weeks of antibiotics,
In lupus nephritis, most common clinical sign of renal disease is ,
proteinuria
is common in patients with acute lupus nephritis (70–90%) and declining complement levels may herald a flare.
Hypocomplementemia
is the only reliable method of identifying the morphologic variants of lupus nephritis
renal biopsy
designates mesangial immune complexes with mesangial proliferation
Class II lupus nephritis
describes focal lesions with proliferation or scarring, often involving only a segment of the glomerulus
The most varied course
Class III FOCAL NEPHRITIS.
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.
Class IV DIFFUSE NEPHRITIS
Without treatment, this aggressive lesion has the worst renal prognosis.
Class IV DIFFUSE NEPHRITIS
—defined as a return to near-normal renal function and proteinuria ≤330 mg/dL per day—is achieved with treatment, renal outcomes are excellent
remission
describes subepithelial immune deposits produc- ing a membranous pattern;
class V lesion Membranous nephritis
subcategory of class V lesions is associated with proliferative lesions and is sometimes called
mixed membranous and proliferative disease
predisposed to renal-vein thrombosis and other thrombotic complications.
class V lesion Membranous nephritis
90% sclerotic glomeruli and end-stage renal disease with interstitial fibrosis.
Class 6 sclerotic nephritis
% of patients with lupus nephri- tis will reach end-stage disease, requiring dialysis or transplantation.
20%
Patients who develop autoantibodies directed against glomerular basement antigens
Anti-GBM disease
Anti-GBM present with lung hemorrhage and glomerulonephritis, they have a pulmonary-renal syndrome called
Goodpasture’s syndrome.
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
Goodpasture’s syndrome.
presence of anti-GBM antibodies and complement is recognized on biopsy by
linear immunofluorescent staining for IgG (rarely IgA)
In goodpastures syndrome or Anti-GBM Disease, Prognosis at presentation is worse if there are
> 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.
It is classically characterized by episodic hematuria asso- ciated with the deposition of IgA in the mesangium.
IgA nephropathy
The two most common presentations of IgA nephropathy are
recurrent episodes of macroscopic hematuria during or immediately following an upper respiratory infection often accompanied by proteinuria or
persistent asymptomatic microscopic hematuria
group of patients with small-vessel vasculitis (arterioles, capillar- ies, and venules; rarely small arteries) and glomerulonephritis have serum ANCA;
ANCA SMALL-VESSEL VASCuLITIS
ANCA antibodies are of two types,
anti-proteinase 3 (PR3)
anti-myeloperoxidase (MPO)
anti- bodies have also been reported experimentally as potentially patho- genic in ANCA
Lamp-2
they are ANCA-positive and have a pauci-immune glomerulonephritis with few immune complexes in small vessels and glomerular capillaries.
Granulomatosis with polyangiitis
microscopic polyangiitis
Churg-Strauss syndrome
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;
Granulomatosis with Polyangiitis
The disease is more common in patients exposed to silica dust and those with α1-antitrypsin deficiency, which is an inhibitor of PR3.
Granulomatosis with Polyangiitis
Biopsy of involved tissue will show a small- vessel vasculitis and adjacent noncaseating granulomas
Granulomatosis with Polyangiitis
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.
Microscopic Polyangiitis
When small-vessel vasculitis is associated with peripheral eosinophilia, cutaneous purpura, mononeuritis, asthma, and allergic rhinitis
Churg-Strauss Syndrome
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.
MEMBRANOPROLIFERATIVE GLOMERuLONEPHRITIS
is commonly associated with persistent hepatitis C infections, autoimmune diseases like lupus or cryoglobulinemia, or neoplastic diseases
Type I MPGN
characterized by expansion of the mesangium, sometimes associated with mesangial hyper- cellularity; thin, single contoured capillary walls; and mesangial immune deposits.
MESANGIOPROLIFERATIVE GLOMERuLONEPHRITIS
classically presents with heavy proteinuria, minimal hematuria, hypoalbuminemia, hypercholesterolemia, edema, and hypertension.
Nephrotic syndrome
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.
Minimal change disease (MCD),
sometimes known as nil lesion,
Minimal change disease (MCD),
presents clinically with the abrupt onset of edema and nephrotic syndrome accompanied by acellular urinary sediment.
Minimal change disease (MCD),
In MCD, Average urine protein excretion reported in 24 h is ____ with severe hypoalbuminemia.
10 grams
In MCD; Ninety to 95% of children will develop a complete remission after
8 weeks of steroid therapy
In MCD, 80–85% of adults will achieve complete remission, but only after a longer course of
20–24 weeks.
refers to a pattern of renal injury characterized by segmental glomerular scars that involve some but not all glomeruli
FSGS
FOCAL SEGMENTAL GLOMERULOSCLEROSIS
most common cause of nephrotic syndrome in the elderly
Membranous glomerulonephritis (MGN) Aka membranous nephropathy
Uniform thickening of the basement membrane along the peripheral capillary loops is seen by light microscopy on renal biopsy
Membranous glomerulonephritis (MGN) Aka membranous nephropathy
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.
ALPORTS SYNDROME
Some patients develop lenticonus of the anterior lens capsule, “dot and fleck” retinopathy, and rarely, mental retardation or leiomyomatosis.
ALPORTS SYNDROME
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.
Alport’s syndrome
characterized by persistent or recurrent hematuria is not typically associated with proteinuria, hypertension, or loss of renal function or extrarenal disease.
THIN BASEMENT MEMBRANE DISEASE
Another name for THIN BASEMENT MEMBRANE DISEASE
benign familial hematuria
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.
Nail patella syndrome
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
Nail patella syndrome
Provides 90% diagnostic in renal amyloidosis
Biopsy of liver or kidney
Aka secondary amyloidosis
AA AMYLOIDOSIS
Systemic AL AMYLOIDOSIS
Primary amyloidosis
is an X-linked inborn error of globotriaosylceramide metabolism secondary to deficient lysosomal α-galactosidase A activity, resulting in excessive intracellular storage of globotriaosylceramide
Fabry’s disease
