26- year old female

presenting with
progressive chest pain

josephdcausapin
 SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

SYSTEMIC LUPUS
ERYTHEMATOSUS (SLE)
 DEFINITION

Systemic lupus
erythematosus (SLE) is an
autoimmune disease in
which organs and cells
undergo damage initially
mediated by tissue-
binding antibodies and
immune complexes.
 PATHOPHYSIOLOGY
PREDISPOSING FACTORS PRECIPITATING FACTORS

age gender hereditary hormones race environment drugs infection

UNKNOWN
ETIOLOGY
 PATHOPHYSIOLOGY
UNKNOWN ETIOLOGY

Female producing 1st generation familial

estrogen Infectious
possession of influencing
SLE DNA agent in the body

Unknown cause of
estrogen influencing HLA class 1 and 2 in HLA class 1 and 2 in
immune response of the chromosome 6 possess chromosome 6 possess
HLA system in multiple genes influenced in multiple genes influenced in
chromosome 6 inheriting SLE inheriting SLE

HLA Class 1 and 2 in chromosome 6 possess multiple genes

influenced in inheriting SLE
 PATHOPHYSIOLOGY
HLA Class 1 and 2 in chromosome 6 possess multiple genes
influenced in inheriting SLE

Fewer of defective Tingible Body Defective B- cell activation by

Macrophages in the body autantigens

Defective clearance of early Defect in mechanism of Hyper- reactivity of defective B-

apoptotic cells immune complex clearance cells

Secondary necrosis of the cells Release of danger signals Production of self and non- self
antibodies and B- memory cells
Release of nuclear fragments as Endocytosis of Ag material
potential autoantigens by the dendritic cells

Induced mutation of dendritic Production of defective

cells helper T- cells
 PATHOPHYSIOLOGY
Production of self and non- self antibodies and B- memory cells

Various Autoantibodies Autoreactive cytotoxic T- Negative abnormal B- cells

production cell activation contribution to already deficient
immune system

Inflammation of the affected

system

Induced mutation of dendritic Defect in mechanism of

cells immune complex clearance

SYSTEMIC LUPUS ERYTHEMATOSUS

PATHOPHYSIOLOGY OF CLINICAL
MANIFESTATIONS OF SLE
Various Autoantibody production

Production of ANA in renal cells

Antibodies bind with antigen

Formation of immune complexes

Leukocyte infiltration

Compliment protein cascade Proteinuria

Recruitment of inflammatory cells

Alteration in the permeability and

structure of the glomerular basement

Induced glomerular injury

 PATHOPHYSIOLOGY OF CLINICAL
MANIFESTATIONS OF SLE
Production of ANA, anti- phospholipids and
other specific autoantibodies

Anti- erythrocyte Lymphocytotoxic Antiphospholipid

antibody activation antibody activation antibody activation

Formation of defective
immune complex

Hemolysis Hemolytic Cellular membrane

anemia component damage
Reduced RBC count
Direct WBC attack
Platelet destruction
Anemia and reduction
Reduced WBC count
Platelet aggregation
and clot formation
Leukopenia Reduced WBC count
Thrombocytopenia
 PATHOPHYSIOLOGY OF CLINICAL
MANIFESTATIONS OF SLE
Production of ANA, anti- phospholipids and
other specific autoantibodies

Formation of the
immune complex

Immunoglobulin and
Vascular inflammation
compliment deposition

Loss of blood supply to Tissue damage in the

the bone acute, sub- acute and
chronic
Avascular bone necrosis
Malar rash
Myalgias Photosensitivity
Collapsed of the
Arthritis Discoid rash
involved joint
 PATHOPHYSIOLOGY OF CLINICAL
MANIFESTATIONS OF SLE
Production of ANA, anti- phospholipids and
other specific autoantibodies

Formation of defective
immune complex Immune deposition/ Activation of Altered cerebral
activation cerebral vasculature functioning

Non- infective inflammation Non- infective inflammation

of pericardium, myocardium of the membrane around
Macro and microvascular Psychosis
and endocardium the lungs
thrombosis Lupus headache
seizures

Cerebral edema and ischemia

SEROSITIS
PATHOPHYSIOLOGY OF CLINICAL
MANIFESTATIONS OF SLE
Production of ANA, anti- phospholipids and
other specific autoantibodies

Formation of the Ineffective biliary cycle

immune complex

Increased of bilirubin in
Upper and lower GI the body
inflammation

Jaundice
Gastric irritability Peritoneal spams

Reflux of the gastric acid

Abdominal pain and
ineffective defecation
Nausea and vomiting
PATHOPHYSIOLOGY OF CLINICAL
MANIFESTATIONS OF SLE
 DIAGNOSIS
Malar rash

Crea. of 1.4 mg/dl and >50/hpf RBC

Obtunded state

Hemolytic anemia

Polyarthralgia

Immunologic manifestations

ANA
Anti- dsDNA
Low serum complement
Direct coomb’s test
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YOU