A CUTE

GLOMERULAR
DISEASES
wvsumc department of pediatrics
chris robinson d. laganao, cc

The Kidney

Anatomy of the Glomerulus

Glomerular Filtration

net result of opposing forces across the

capillary wall
forces that facilitate filtration:

glomerular capillary hydrostatic pressure

forces that oppose filtration:

glomerular capillary oncotic pressure

Bowman space hydrostatic pressure

GFR is estimated by measurement of serum

creatinine

Glomerular Diseases

may be a result of genetic, immunologic, or

coagulation disorders
immunologic injury is the most common
cause of glomerulonephritis

localization of circulating antigen-antibody

immune complexes

interaction of antibody with local antigen in situ

Glomerular Diseases

classified into:

primary vs. secondary

acute vs. persistent

postinfectious vs. noninfectious

Acute Poststreptococcal GN

acute nephritic syndrome

characterized by sudden onset of gross
hematuria, edema, hypertension, and
renal insufficiency

usu. follows an infection of the throat or skin

Group A beta-hemolytic streptococci

M protein subtypes 12 (throat) and 49 (skin)

Acute Poststreptococcal GN

pathologic findings:

enlarged glomeruli with diffuse mesangial cell

proliferation,

presence of PMNs

crescents and interstitial inflammation

mediated by immune complexes

most common among aged 5 12 y/o

phases: oliguric, diuretic, convalescent stages

Acute Poststreptococcal GN

manifestations:

1 to 2 weeks after strep pharyngitis, or

3 to 6 weeks after strep pyoderma

urinary changes: hematuria, oliguria, anuria

edema

hypertension

encephalopathy

malaise, abdominal or flank pain, fever

Acute Poststreptococcal GN

diagnosis:

urinalysis: RBCs, PMNs, RBC casts, proteinuria

mild normochromic anemia

elevated BUN, creatinine levels

low complement level (C3)

high ASO titer (>200 IU/ml)

positive throat culture

positive anti-DNAse B, antihyaluronidase

renal biopsy

Acute Poststreptococcal GN

treatment:

antibiotics
Penicillin V 50-100KU/kg/day x10 days

Erythromycin

sodium and fluid restriction (20cc/kg/day)

diuresis (Furosemide 1 mkdose)

correct hypertension
Nifedipine 0.25-0.5 mkday TID

Captopril 0.5-2.0 mkday TID

Sodium nitroprusside 0.5-1.0 mcg/kg/min

Labetolol 0.2-1.0 mkdose

Hydralazine 0.2-0.4 mkdose

Acute Poststreptococcal GN

complications:

hypertension

acute renal failure

electrolyte abN (K, P, Ca)

heart failure

seizures

Acute Poststreptococcal GN

prognosis:

self limiting in 80% of cases

cure rate >95% if given treatment

recurrences are rare

hematuria may persist 1-2 years after onset

risk factors:
age <2 or >15 years old

rapidly progressive renal dysfunction

presence of crescent formation on biopsy

Nonstreptococcal GN

Viral:

Coxsackie B4, Echovirus 9, Influenza, Mumps,

Rubeola, Rubella, Varicella, Hepatitis B and C,
Herpes simplex
diagnosis is by viral identification and negative
strep tests in the presence of acute nephritic
syndrome
self limited
supportive treatment is sufficient

Nonstreptococcal GN

Bacterial:

Streptococcus viridans (infective endocarditis)

Streptococcus pneumoniae (lobar PN)
Staphylococcus epidermidis (VA shunts in HCP)
Leptospira sp. (Leptospirosis)

Nonstreptococcal GN

Parasitic:

Malaria
Schistosomiasis
Leishmaniasis
Filariasis
Hydatid disease
Trypanosomiasis
Toxoplasmosis

STOP
GO!

IgA Nephropathy

aka Berger Nephropathy

most common chronic glomerular disease

predominance of IgA within mesangial

deposits of the glomerulus in the absence of
systemic diseases

abN in the IgA immune system

linked to genetic abN (6q22-23)

IgA Nephropathy

manifestations:

mild to moderate hypertension

edema

gross or microscopic hematuria

proteinuria

normal complement levels

elevated serum IgA level

IgA Nephropathy

prognosis:

progressive kidney disease develops in 20-30%,

15-20 years after onset

poor prognostic indicators:

persistent hypertension

diminished renal function

heavy or prolonged proteinuria

IgA Nephropathy

treatment:

blood pressure control

immunosuppressants

renal transplantation

Hereditary Nephritis

aka Alport Syndrome

caused by mutations in the genes coding for
type IV collagen
85% are X-linked

Hereditary Nephritis

pathology:

mesangial proliferation and capillary wall

thickening

tubular atrophy, interstitial inflammation and

fibrosis

presence of foam cells

Hereditary Nephritis

manifestations:

asymptomatic microscopic hematuria

progressive proteinuria

extrarenal:
sensorineural hearing loss

visual abnormalities (anterior lenticonus, macular

flecks, recurrent corneal erosions)

leiomyomatosis

Hereditary Nephritis

prognosis:

progressive renal dysfunction occurs in 75%

treatment:

no specific treatment

careful management of hypertension, anemia, and

electrolyte imbalance

dialysis

renal transplantation

Lupus Nephritis

SLE is characterized by fever, weight loss,

rash, hematologic abN, arthritis, involvement
of the heart, lungs, CNS, and kidneys

common among adolescent females

occurs in 30-70% of all cases

WHO classification is based on:

light microscopy
immunofluorescence
electron microscopy

Lupus Nephritis

Class I

Class II (mesangiopathy)

no histologic abN

mesangial widening and hypercellularity

Class III (focal segmental LN)

mesangial deposits in all glomeruli

capillary wall necrosis, crescent formation
sclerosing lesions

Lupus Nephritis

Class IV (diffuse proliferative LN)

most common, most severe form

massive mesangial and subendothelial deposits
crescent formation, sclerosis
necrosis

Class V (membranous LN)

pure membranous GN

Lupus Nephritis

manifestations:

asymptomatic hematuria or proteinuria,

acute nephritic syndrome, or
nephrotic syndrome

Lupus Nephritis

diagnosis:

SLE criteria
high ANA titer
high anti-dsDNA titer
low serum C3 level
renal biopsy

should be performed in all patients with SLE

Lupus Nephritis

treatment:

initial: Oral corticosteroids

Prednisone or Prednisolone 1-2 mkday BID or TID with

gradual tapering for 4 to 6 months

cytotoxic agents:
Cyclophosphamide

Azathioprine

IV corticosteroids (methylprednisolone)
plasmapheresis

Lupus Nephritis

prognosis:

renal failure is the most common cause of death

among patients with SLE

complications

prolonged steroid use (growth retardation, hypertension,

obesity, osteoporosis, diabetes mellitus)

cytotoxic effects (malignancy, infertility)

intercurrent infections
thromboses

The Others

Henoch-Schnlein Purpura Nephritis

aka anaphylactoid purpura

small vessel vasculitis with skin, joint, abdominal
manifestations
renal involvement in 40-60%
immune complex formation
similar clinical findings except ureteritis
no controlled data on treatment protocols

The Others

Polyarteritis nodosa

large vessel vasculitis with rash, arthralgia, and

nephritis
malignant hypertension and weight loss
renal involvement in 40-70%
capillary thrombosis

fibrinoid necrosis

capsular infiltration with crescent formation

treatment:
supportive (steroids, anticoagulants, cytotoxic agents)

antihypertensives

THE E.N.D.