GLOMERULONEPHRITIS

IN SYSTEMIC LUPUS
ERYTHEMATOSUS
YOHANES SATRYA WIBAWA
030.09.275
INTRODUCTION
Systemic Lupus Erythemstosus (SLE) is a
chronic systemic inflammatory disease that
follows a course of alternating exacerbations or
remissions. SLE is autoimmune disorder that
affects multiple organ systems including the
skin, kidneys, and brain. The exact cause of
SLE is unknown but genetic factors, ethnic
origin, environmental factors, and medications
may all be involved in its development.

EPIDEMIOLOGY
o Predominantly affects women of childbearing
age (20 40 years)
o Female to male ratio of 9:1 to 15:1.
o Approximately 8% to 15% of SLE cases occurs
in children.
o Genetic and racial factors African-American
women have a 3 to 4 times higher risk
prevalence of SLE than Caucasian women.
ETIOLOGY
A complex abnormal immune process (unknown)
Dysregulation of B- and T-lymphocytes production of
autoantibodies formation of immune complexes
Cytokines key role
Environmental factors

PATHOPHYSIOLOGY
CLINICAL MANIFESTATION
Acute - insidious
Non spesific fever, weight loss, severe fatique, and
lymphadenopathy
Every organ system may become involved
Common skin manifestations malar or butterfly rash,
with sun-induced macules or papules occurring on the
face
90% joint inflammation
Renal complication (glomerulonephritis and
microvascular thrombosis) common
Modified capillary network that delivers an unfiltrate of plasma to Bowmans
space
1.6 million glomeruli are present in two mature kidneys
GLOMERULUS
Filtration of plasma protein and blood cells
glomerular filtration barrier, composed of :
fenestrated glomerular endothelium;
basement membrane;
foot processes and slit diaphragms of visceral epithelial
cells (podocytes).
Glomerular injury impairment of glomerular
filtration and/or inappropriate appearance of plasma
proteins and blood cells in the urine.

GLOMERULONEPHRITIS
Glomerular injury
Inflammation such as leukocyte infiltration,
antibody deposition, and complement activation
Glomerular disease :
Primary : the pathology is confined to the kidney and
any systemic features are a direct consequence of
glomerular dysfunction; usually idiopathic.
Secondary : part of a multisystem disorder.

GLOMERULONEPHRITIS IN
SLE
GLOMERULONEPHRITIS IN
SLE
Commonest and most serious
75% found at autopsy
Autoimmune disease the immune system cannot tell
the difference between harmful substances and healthy
ones attacks healthy cells and tissue
Damage different parts of the kidney interstitial
nephritis, nephritic syndrome, and membranous
glomerulonephritis.
May rapidly worsen to kidney failure.
WHO CLASSIFICATION of LUPUS
NEPHRITIS
1) WHO class I nephritis : no histologic abnormalities are detected
2) WHO class II nephritis (mesangial lupus nephritis)
3) WHO class III nephritis (focal segmental lupus glomerulonephritis)
4) WHO class IV nephritis (diffuse proliferative lupus nephritis)
5) WHO class V nephritis (membranous lupus nephritis)

CLINICAL FINDING
Hematuria
Proteinuria
Reduced renal function
In rare patients with proliverative
glomerulonephritis, the urinarylis may be
completely normal.

DIAGNOSIS
Detection of circulating antinuclear antibodies(ANA)
Confirmation : demonstrating antibodies that react with
native double-stranded DNA
Depressed C3 and C4 levels
Renal biopsy

TREATMENT
Medical and psychologic support
Immunosuppressive therapy :
Prednisone 1-2 mg/kg/day for a serologic
remission
6 monthly i.v. infusions of Cyclophosphamide
500-1000mg/m
2
reduce the risk of progressive
renal dysfunction
4,5


PROGNOSIS
Unsatisfactory
Up to 25% end stage renal failure in 10
years

CONCLUSION

Systemic lupus erythematosus (SLE) is an autoimmune
disorder that can affect several organ systems, including
the kidney. Although renal involvement is common in
SLE, most studies showed that kidney disease
presented less commonly in late onset SLE compared
with younger patients. Better and earlier recognition of
SLE and more effective treatments have significantly
improved survival rates. The aim of the treatment is first
to stop disease progression, prevent reccurence, and
minimizing the adverse effects. More specifically with
lupus nephritis, the aims of treatment are to reduce the
risk of end stage renal disease, reduce renal and extra-
renal lupus activity of symptoms, and reduce the
mortality risk.