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    Systemic Lupus Erythematosus: Alni Dwi Cahyani

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    Aji Prasetyo Utomo
    Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that can affect multiple organ systems. It is caused by abnormalities in the immune system including overactive B cells producing autoantibodies, suppressed regulatory T cell function, and activation of the complement system. Common symptoms include fatigue, joint and skin issues. Complications can include kidney disease, heart disease, and neurological issues. Diagnosis involves tests for autoantibodies, complement levels, and organ involvement. SLE can range from mild to life-threatening depending on the individual.

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    Systemic Lupus Erythematosus: Alni Dwi Cahyani

    Uploaded by

    Aji Prasetyo Utomo
    16 views21 pages
    Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that can affect multiple organ systems. It is caused by abnormalities in the immune system including overactive B cells producing autoantibodies, suppressed regulatory T cell function, and activation of the complement system. Common symptoms include fatigue, joint and skin issues. Complications can include kidney disease, heart disease, and neurological issues. Diagnosis involves tests for autoantibodies, complement levels, and organ involvement. SLE can range from mild to life-threatening depending on the individual.

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    Lupus Presentation

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    Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that can affect multiple organ systems. It is caused by abnormalities in the immune system including overactive B cells producing autoantibodies, suppressed regulatory T cell function, and activation of the complement system. Common symptoms include fatigue, joint and skin issues. Complications can include kidney disease, heart disease, and neurological issues. Diagnosis involves tests for autoantibodies, complement levels, and organ involvement. SLE can range from mild to life-threatening depending on the individual.

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    Download as ppt, pdf, or txt
    16 views21 pages

    Systemic Lupus Erythematosus: Alni Dwi Cahyani

    Uploaded by

    Aji Prasetyo Utomo
    Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that can affect multiple organ systems. It is caused by abnormalities in the immune system including overactive B cells producing autoantibodies, suppressed regulatory T cell function, and activation of the complement system. Common symptoms include fatigue, joint and skin issues. Complications can include kidney disease, heart disease, and neurological issues. Diagnosis involves tests for autoantibodies, complement levels, and organ involvement. SLE can range from mild to life-threatening depending on the individual.

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    of 21

    Systemic Lupus

    Erythematosus
    Alni Dwi Cahyani
    Definisi
    SLE adalah penyakit peradangan kronik
    multisistem yg dihubungkan dengan
    ketidaknormalan sistem imun. SLE
    berpengaruh pd kulit, persendian &
    membran serosa (pleura, perikardium),
    jantung, ginjal, sistem hematologi &
    neurologi (Lewis et al, 2004)
    History
    • 1948 – Malcolm Hargraves discovers the
    lupus erythematosus (LE) cell.
    • 1957 – The first anti-DNA antibody is
    identified.
    LE Cell
    • The LE cell is a
    neutrophil that has
    engulfed the antibody-
    coated nucleus of
    another neutrophil.
    • LE cells may appear in
    rosettes where there are
    several neutrophils vying
    for an individual
    complement covered
    protein.
    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
    Gambaran Klinis SLE
    Limphadenopati Kelelahan
    SSP 12-50% 90%
    20% Panas
    Hepotomepali/ 80-82%
    Splenomegali BB turun
    20% 60%

    Sal cerna
    SLE Artritis/Artralgia
    90%
    18%
    Kulit
    Paru 50-58%
    38% Ginjal
    Hematologi 50%
    Jantung Vaskulitis
    50%
    48%
    Symptoms
    • Non-specific:
    – Fatigue
    – Weight loss
    – Malaise = generally feeling ill
    – Fever
    – Anorexia (over time)
    – Arthritis
    • 90% of patients experience arthritic symptoms
    • Symmetrical
    • Appears in hands, wrists, and knees mainly
    Skin Manifestations

    • Malar or Butterfly Rash

    • Discoid Rash –
    Stimulated by UV light

    • Skin manifestations
    only appear in 30-40%
    of lupus patients.
    Renal (Kidney)
    Manifestations
    • 50-70% of all lupus
    patients experience renal
    developments.
    • Most Dangerous:
    – Glomerulonephritis
    where at least 50% of the
    glomeruli have cellular
    proliferation
    • Glomeruli – capillary
    beds in the kidney that
    filter the blood.
    Normal
    • Renal Failure because of
    Glomerulonephritis is the
    leading cause of death
    among lupus patients.

    Glomerulonephritis
    Other Manifestations
    • Cardiac
    • Central Nervous System
    • Hematological
    Main Pathology
    • The plasma cells are producing
    antibodies that are specific for self
    proteins, namely ds-DNA
    • Overactive B-cells
    • Suppressed regulatory function in T-cells
    • Lack of T-cells
    • Activation of the Complement system
    Overactive B-cells
    • Estrogen is a stimulator of B-cell activity
    – Lupus is much more prevalent in females of
    ages 15-45
    • Height of Estrogen production
    • IL-10, also a B-cell stimulator is in high
    concentration in lupus patient serum.
    – High concentration linked to cell damage
    caused by inflammation
    T-cell Malfunctions
    • Fc region switch
    – ζ  εγ
    – Leads to malfunction in signaling and
    decreased IL-2 production
    • Increased levels of Ca2+
    – Leads to spontaneous apoptosis
    T-cell Signal Transduction
    Activation of Complement
    System
    • Complement system is activated by
    the binding of antibodies to foreign
    debris.
    – In this case its over activation
    • RBCs lack CR1 receptor
    – Decreasing the affective removal of
    complexes
    IgG Pathogen
    • IgG is the most “pathogenic”
    because it forms intermediate sized
    complexes that can get to the small
    places and block them.
    DNA is the Main man
    • DNA is the main antigen for which
    antibodies are formed.
    • Extracellular DNA has an affinity for
    basement membrane where it is
    bound by autoantibodies.
    • Classical thickening of the basement
    membrane
    Testing
    • ESR
    • Urinalysis
    • Complement Test
    – Tests levels of C3, C4, CH50
    – Low levels indicates possible presence of
    disease
    • FANA – Fluorescent antinuclear antibody
    • Ouchterlony Test – shows interactions
    FANA
    • ELISA Test
    – Generally test for:
    • ds-DNA antibodies
    • Antihistone
    antibodies
    – Binds to DNA,
    major
    constituent of
    chromatin
    • Deoxyribonucleopr
    otein (DNP)
    Ouchterlony Test
    • Used to determine
    immunological
    specificity
    • Rules out a false
    positive
    • Shows the serum
    does or does not
    have antinuclear
    antibodies
    Summary
    • Lupus = Autoimmunity
    – Systemic and affects connective tissue
    • Caused by malfunctions of:
    – T-cells
    – B-cells
    – Complement System
    – Signal Transduction
    • Can be lethal or not
    • Unique to each individual

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