ERITEMATOSUS
Sex
Age
Although lupus affects people of all ages, it's most often diagnosed
between the ages of 15 and 40.
Race
Genetics
environ
ment
Abnormal
immune
respons
Genetic Associations
• HLA’s are loci on genes that code for
certain β chain on the MHC complex
• HLA-DR2
• HLA-DR3
• HLA-DQB1 – Involved in mediating
production of antibodies to ds-DNA
Environmental triggers
• UV light
• Gender (estrogen)
• Epstein Barr Virus
• Vitamin D deficiency
• Other organic compounds
– Silica dust, solvent, smoking
• Drugs
– Sulfonamide, hidralazyn, isoniazid,
penicilline,propafenone, procainamide,
interferon, TNF inhibitor
PATHOPHYSIOLOGY
Diagnostic Criteria
1997 Update of the 1982 American College of Rheumatology Revised Criteria for
Classification
of Systemic Lupus Erythematosus
SLEDAI (Systemic lupus Erythematous Disease
Activity Index) is one of the major scoring
systems to evaluate the activity of lupus in
clinical studies. The evaluation of the disease
could be used as the reference for the treatment.
Disease Severity Classification
Mild SLE:
a)Clinically calm
b)No signs or symptoms are life-threatening.
c)The functions of organs are stable , which is: kidney, lung, heart,
gastrointestinal, central nervous system, joints, hematology and skin.
The example is SLE with arthritis and skin manifestations.
Moderate SLE:
a)Mild to moderate nephritis (Lupus nephritis class I and II).
b)Thrombocytopenia (thrombocyte 20-50x103 / mm3)
c)Serositis major
Severe SLE:
a)Heart: Libman-Sacks endocarditis, coronary artery vasculitis, myocarditis,
cardiac tamponade, malignant hypertension
b)Lung: pulmonary hypertension, pulmonary bleeding, pneumonitis,
pulmonary embolism, Pulmonary infarction, interstitial fibrosis, shrinking lung
c)Gastrointestinal: pancreatitis, mesenteric vasculitis.
d)Kidney: proliferative and or membranous nephritis.
e)Skin: severe vasculitis, diffuse rash accompanied by ulcers or blisters.
f)Neurology: seizures, acute confusional state, coma, stroke, transverse
myelopathy, mononeuritis, polineuritis, optic neuritis, psychosis,
demyelinating syndrome.
g)Hematology: hemolytic anemia, neutropenia (leukocytes
<1,000/mm3),Thrombocytopenia <20.000 / mm3, thrombotic
thrombocytopenia purpura, thrombosis of veins or arteries.
Lupus Nephritis Classification
Treatment
Rest
Education/counseling
Physical
exercise
Rehabilitation Therapy
Therapeutic
modalities
Orthotic
OAINS
Antimalaria
Pharmacology
Steroid
Imunosupresan
PHARMACOLOGY
Methylprednisolon Pulse
INDICATION:
Therapy
1.Severe lupus neuropsychiatry (seizures, decreased consciousness, transverse
myelitis, cerebrovascular accident, psychosis, organic brain syndrome, multiplex
mononeuritis)
2.WHO Class III, IV or V lupus nephritis with progres- sivity of disease activity
3.Hematological disorders (severe refractory thrombocytopenia with hemorrhage,
hemolytic anemia)
4. Severe pulmonary hemorrhage
5.General vasculitis
CONTRAINDICATION:
1.Known hypersensitivity / allergy to drug therapy pulse
2.Being an acute infection (a relative contraindication), if very needed, pulse
administration is done immediately after administration of antibiotics
3.Hypertension (if blood pressure has been lowered with antihypertensive drugs,
pulse administration can be done)
SLEDAI (Systemic lupus Erythematous Disease
Activity Index) is one of the major scoring
systems to evaluate the activity of lupus in
clinical studies. The evaluation of the disease
could be used as the reference for the treatment.
Disease Severity
Classification
Mild SLE:
a)Clinically calm
b)No signs or symptoms are life-threatening.
c)The functions of organs are stable , which is: kidney, lung, heart,
gastrointestinal, central nervous system, joints, hematology and skin.
The example is SLE with arthritis and skin manifestations.
Moderate SLE:
a)Mild to moderate nephritis (Lupus nephritis class I and II).
b)Thrombocytopenia (thrombocyte 20-50x103 / mm3)
c)Serositis major
Severe SLE:
a)Heart: Libman-Sacks endocarditis, coronary artery
vasculitis, myocarditis, cardiac tamponade, malignant
hypertension
b)Lung: pulmonary hypertension, pulmonary bleeding,
pneumonitis, pulmonary embolism, Pulmonary infarction,
interstitial fibrosis, shrinking lung
c)Gastrointestinal: pancreatitis, mesenteric vasculitis.
d)Kidney: proliferative and or membranous nephritis.
e)Skin: severe vasculitis, diffuse rash accompanied by ulcers
or blisters.
f)Neurology: seizures, acute confusional state, coma, stroke,
transverse myelopathy, mononeuritis, polineuritis, optic
neuritis, psychosis, demyelinating syndrome.
g)Hematology: hemolytic anemia, neutropenia (leukocytes
<1,000/mm3),Thrombocytopenia <20.000 / mm3, thrombotic
thrombocytopenia purpura, thrombosis of veins or arteries.
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