Systemic Lupus
Erythematosus
Zhongshan Hospital
於强
一, Types of lupus
▲ Discoid
▲ Systemic
▲ Drug-induced
二, Characteristic of disease,
▲ Atuoimmune
▲ Target tissue damage,
connection tissue (shin,Joint,
muscles)
▲ Predominantly population,
women
during their reproductive year 90%
▲ morbidity,70 per 100,000
三, Pathogenic factor
▲ Susceptibility gene,HLA-DR3
▲ Environmental Factors,a,ultraviolet light
b,food
c,drugs hydralazine
procainamide
D-penicillamine
▲ Sex hormone
▲ Infections agents
四, pathogenesis
unclear
Susceptibility gene
Environmental Factors Sex hormone
Helper T cell activity
Hyperactivated B cell
Immune complex
Self antibody + self antigens
Multiple system involvement vasculitis
五, Pathology
1,vasculitis
2,lupus nephritis
a,minimal change
b,mesangial proliferative
c,focal segmental lesions
d,diffuse
e,membranous
f,sclerosing
六,Clinical menifestations
of SLE
-----Multiple system
involvement
1,Nonspecific symptom
● fever
● loss of weight
● inertia
2.Skin and mucosa
● skin symptoms in 80% of patients
● specific features,40%
malar rash
discoid rash
● nonspecific features 60%
● photosensitivity 40%
● baldness 40%
● oral ulcer 60%
● Raynaud’s phenomenon
3,Joint and muscle 80%
● arthritis---non erosion
● large and small joints
● myosalgia 40%
● myositis 5%
4,Kidney
▲ renal biopsy can be found
renal involvement is all
▲ present in about 70% of
patients
▲ 25% patients are dead in
cause of renal
involvement
5,Heart
● pericarditis
pericardial effusions
● myocarditis
● endocarditis
cardiac valves
vegetation
6,Lungs
● acute lupus penumonitis 3%
● interstitial pneumonia
pulmonary hypertension
● pleuritis
pleural friction sound
hydrothorax
interstitial pneumonia
7.Neuropsychiatric
involvement
● CNS
unfavourable prognosis
activity
cranial nerves
spinal cord
● peripheral nervous system
● mental disorder
8.Digestive system
● appetite loss vomiting
● abdominal pain
diarrhea
ascites
● ALT\AST
● pancreatitis
● intestinal perforation
obstruction
● mesentery vasculitis
9.Hematologic system
● anemia 60%
hemolytic anemia 10%
● leukopenia 40%
● thrombocytopenia 20%
ITP
● lymphadenectasis
10.eye
11.Overlap CTD
RA
SS
七, Laboratory findings
● ESR
● CRP
Antibody
1,ANA (antinuclear antibody)
screening test
2,Anti double-stranded
DNA antibody
(ds-DNA)
● specific
● activity
● strong association of
lupus nephritis
3.Anti Smith antibody (Sm)
● marker antibody
● non-activity
4.Antiphospholipid antibody
● arterial\venous thrombosis
● pregnancy morbidity
● thrombocytopenia
5,Anti SS-A
SS-B
RNP(ribonucleoprotein)
RF
6,Complement
● depressed C3\C4\CH50
● activity
7,CT
X-ray
ultrasound
八,Diagnosis
The 1982 Revised Criteria for
Classification of SLE
1,Malar rash
2,Discoid rash
3,Photosensitivity
4,Oral ulcers
5,Arthritis
6,Serositis
a) Pleuritis b) Pericarditis
7,Renal disorder
a) Persistent
proteinuria > 0.5 g/d or > 3+
OR
b)may be red cell,hemoglobin,
granular,tubular,or mixed
8,Neurologic disorder
9,Hematologic disorder
a) Hemolytic anemia
OR
b) Leukopenia < 4,000
OR
c) Lyphopenia < 1,500
OR
d)Thrombocytopenia <100,000
10,Immunologic disorder
a) Positive LE cell
OR
b) Anti-DNA
OR
c) Anti-Sm
OR
d) False positive serologic
test for syphilis
11,Antinuclear antibody
a person shall be said to have
SLE if any 4 or more of the 11
criteria are present,serially or
simultaneously,during any
interval of observation
九,Management of SLE
1,remove the cause
drug
food
uv light infection
have rest
2,Discoid
nonsteroidal anti-
inflammatory drugs
（ NSAIDs）
+
antimalarials (chloroquine）
OR
local steroids or
low dose GC
3,SLE
glucocorticoid (GC)
+
cytotoxic drugs
(cyclophosphamide)
● moderate dose GC
1mg/kg/d
4,
● lymphocyte-specific
cytotoxic drug
● intravenous gamma
globulin
● immunoablation with
autologous stem cell
transplantation
十,Prognosis
● has improved
● death is caused most
frequently by infection
or severe nephritis or
diffuse CNS lupus