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    Systemic Lupus Erythematosus

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    dzikroul
    Systemic Lupus Erythematosus (SLE) is an autoimmune disease caused by both genetic and environmental factors. It is more common in women and is characterized by abnormal autoantibody production. Common symptoms include fatigue, rashes, arthritis, and kidney and neurological involvement. Diagnosis requires meeting criteria from the American Rheumatism Association and testing positive for autoantibodies. Treatment involves medications like hydroxychloroquine, corticosteroids, immunosuppressants, and plasma exchange depending on disease severity. Prognosis has improved with 10-year survival rates exceeding 85% but infection remains a risk due to immunosuppressive therapies.

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    Systemic Lupus Erythematosus

    Uploaded by

    dzikroul
    29 views37 pages
    Systemic Lupus Erythematosus (SLE) is an autoimmune disease caused by both genetic and environmental factors. It is more common in women and is characterized by abnormal autoantibody production. Common symptoms include fatigue, rashes, arthritis, and kidney and neurological involvement. Diagnosis requires meeting criteria from the American Rheumatism Association and testing positive for autoantibodies. Treatment involves medications like hydroxychloroquine, corticosteroids, immunosuppressants, and plasma exchange depending on disease severity. Prognosis has improved with 10-year survival rates exceeding 85% but infection remains a risk due to immunosuppressive therapies.

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    Systemic Lupus Erythematosus (SLE) is an autoimmune disease caused by both genetic and environmental factors. It is more common in women and is characterized by abnormal autoantibody production. Common symptoms include fatigue, rashes, arthritis, and kidney and neurological involvement. Diagnosis requires meeting criteria from the American Rheumatism Association and testing positive for autoantibodies. Treatment involves medications like hydroxychloroquine, corticosteroids, immunosuppressants, and plasma exchange depending on disease severity. Prognosis has improved with 10-year survival rates exceeding 85% but infection remains a risk due to immunosuppressive therapies.

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    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    29 views37 pages

    Systemic Lupus Erythematosus

    Uploaded by

    dzikroul
    Systemic Lupus Erythematosus (SLE) is an autoimmune disease caused by both genetic and environmental factors. It is more common in women and is characterized by abnormal autoantibody production. Common symptoms include fatigue, rashes, arthritis, and kidney and neurological involvement. Diagnosis requires meeting criteria from the American Rheumatism Association and testing positive for autoantibodies. Treatment involves medications like hydroxychloroquine, corticosteroids, immunosuppressants, and plasma exchange depending on disease severity. Prognosis has improved with 10-year survival rates exceeding 85% but infection remains a risk due to immunosuppressive therapies.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    of 37

    Systemic Lupus

    Erythematosus
    Isbandiyah, dr, SpPD

    tiology and Pathogenesis of S

    1. Genetic factor

    Many studies have described familial


    aggregation of SLE. 5-13% of lupus have
    at least one first or second degree relative
    with lupus
    It was found a 24-58% concordance in
    monozygotic twins.
    2-5% concordance in dizygotic twins or
    siblings..
    The risk of a child developing lupus born
    from a mother (or father) with lupus is
    calculated to be 3-4% at worst.

    2. Environmental factors
    1. UV light, especially UVB
    2. Drug-induced lupus. Drugs ( hydralazine,
    procainamide, beta-blokers, isoniazid,
    penicillamine) can induce lupus.
    3. Allergy.
    4. Infection.

    3. Sex hormones
    Female : Male=9:1
    The sex difference is most
    prominent during the female
    reproductive years.
    In mice, castrating females and /or
    providing androgens or
    antiestrogens protects from
    disease,whereas castrating males
    and providing estrogens
    accelerates and worsens SLE.

    4. Abnormal immune
    system
    Sustained presence of autoantigens: increased
    apoptosis , impaired clearance of apoptosis
    Hyperactivity in B and T lymphocyte.
    Increased expression of surface molecules
    participating in cell activation in both B- and Tcell.
    Overproduction of IL-6 and IL-10
    Defective regulatory mechanism.
    Autoantibodies to DNA, RNA, and a host of
    other cell nucleus antigens.
    Circulating immune complexes

    Clinical manifestations
    of SLE

    General symptoms

    The most common symptoms listed


    as initial complaints are fatigue,
    fever, and weight loss.
    Fever: fever secondary to active disease
    was recorded from 50% to 86%.
    Fatigue is common in patients with SLE,
    especially during periods of disease
    activity

    Dermatological
    involvement

    Up to 85% of SLE
    Butterfly rash
    Maculopapular eruption
    Discoid lupus
    Relapsing nodular non-suppurative
    panniculitis
    Vasculitic skin lesin
    Livedo reticularis
    Purpuric lesions
    Alopecia
    Oral ulcer

    Malar rash: This is


    a "butterflyshaped" red rash
    over the cheeks
    below the eyes
    and across the
    bridge of the nose.
    It may be a flat or
    a raised rash.
    The rashes are
    made worse by
    sun exposure.

    Maculopapular
    eruption

    Discoid lupus
    These are red,
    raised patches
    with scaling of the
    overlying skin.

    Vasculitic skin
    lesin

    Alopecia

    Oral ulcer:
    Painless sores in
    the nose or mouth
    need to be
    observed and
    documented by a
    doctor.

    Musculoskeletal system
    The arthritis of lupus is usually found
    on both sides of the body and does not
    cause deformity of the joints. Swelling
    and tenderness must be present.
    The most frequently involved joints are
    those of the hand, knees, and wrists.
    People with lupus can suffer from a
    certain type of low blood flow injury to
    a joint causing death of the bone in the
    joint.

    Kidney system
    Haematuria
    Proteinure (>0.5g protein/d or 3+ )
    Cast

    Nervous system
    The brain , nerve problems and psychiatric
    syndromes are common in lupus affecting
    up to two-thirds of people.
    Potential disorders include seizures, nerve
    paralysis, severe depression, and even
    psychosis.
    Spinal cord involvement in lupus is rare

    Hematological
    abnormalities
    Red blood cells
    a normochromic, normocytic anemia is frequently
    found in SLE.
    haemolytic anemia as detected by the Coombs test
    is the feature of SLE.
    Platelets.
    thrombocytopenia (<100*109/L) appears to be mediated by
    anti-platelet antibodies or/and anti-phospholipid antibodies
    White blood cell
    leucopenia (<4.0*109/L), its cause is probably a
    combination of destruction of white cells by
    autoantibodies, decreased marrow production
    Lymphopenia - less than 1,500/mm3

    Pulmonary
    manifestations
    Pleurisy
    it is the most common manifestation
    of pulmonary involvement of SLE. The
    volume of pleural effusions usually is
    small to moderate and maybe
    unilateral or bilateral. Large pleural
    effusion are uncommon. It usually
    exudative in character.
    Pleural effusions may also occur in SLE
    patients with nephrotic syndrome, infection,
    cardiac failure.

    Lung
    1) acute lupus pneumonitis:
    fever, dyspnea, cough with scanty
    sputum, hemoptysis, tachypnea
    and pleuritic chest pain.
    2) pulmonary hemorrhage
    3) chronic diffuse interstitial
    lung disease.
    the diagnosis should not be made until
    infectious processes such as viral
    pneumonia, tuberculosis, and other
    bacterial, fungal and pneumocystis

    Cardiovascular
    manifestations
    Pericarditis is the most common
    cardiac manifestation of SLE.
    Myocarditis (the clinical features of
    lupus myocarditis resembles that of
    viral myocarditis)
    Endocarditis and valvular disease
    Hypertension, cardiac failure

    Gastrointestinal and hepatic


    manifestation
    Esophagitis, dysphagia, nausea,
    vomiting: (drug related in most
    cases)
    Chronic intestinal pseudoobstruction, mesenteric vasculitis,
    protein-losing enteropathy
    Pancreatitis
    Lupus hepatitis

    Laboratory
    investigation

    Autoantibodies in SLE
    Antibodies to cell nucleus component
    ANA, anti-dsDNA, antibodies to extracellular
    nuclear antigen (ENA, anti-Sm, anti-RNP, anti-Jo1)
    Antibodies to cytoplasmic antigens
    anti-SSA, anti-SSB
    Cell-specific autoantibodies
    lymphocytotoxic antibodies, anti-neurone
    antibodies, anti-erythrocyte antibodies, antiplatelet antibodies
    Antibodies to serum components
    antiphospholipid antibody
    anticoagulants antiglobulin (rheumatoid factor)

    Diagnosis

    Criteria of the ARA for the classification of SLE


    1. Malar rash: Fixed erythema over malar areas, sparing nasolabial folds
    2. Discoid rash: Erythematous raised patches with keratotic scaling and follicular plugging
    3. Photosensitivity: Skin rash after exposure to sunlight, history or physical exam
    4. Oral ulcers: Oral or nasopharyngeal, painless, by physical exam
    5. Arthritis:Tenderness, swelling, effusion in 2 or more peripheral joints
    6. Serositis: A) pleuritis or B) pericarditis
    7. Renal disorder A) proteinuria>0.5g/24hour or 3+ or B) cellular
    casts
    8. Neurological disorder: A) seizures or B) psychiatric disorder
    (having excluded other
    causes, e.g. drigs)
    9. Haematological disorder: A) haemolytic anaemia or B)
    leucopenia or C) thrombocytopenia
    10. Immunologic disorder: A) positive LE cells or B) raised antinative DNA antibdy binding or C) anti-Sm antibody or D) false
    positive serological test for syphilis.
    11. Positive antinuclear antibody:

    Management and
    treatment

    Grading clinical activity


    SLE disease activity index (SLEDAI)
    Clinical feature
    score
    seizure , psychosis , organ brain syndrome
    8
    visual disturbance, cranial nerve disorder
    8
    lupus headache, cerebrovascular accidents,
    8
    vasculitis
    8
    arthritis
    4
    myositis
    4
    urinary casts, hematuria, proteinure, pyuria
    4

    SLE treatment I.
    Mild cases (mild skin or joint
    involvement): NSAID, local
    treatment, hydroxy-chloroquin
    Cases of intermediate severity
    (serositis, cytopenia, marked skin or
    joint involvement): corticosteroid
    (12-64 mg methylprednisolon),
    azathioprin, methotrexat

    SLE treatment II.


    Severe, life-threatening organ involvements (carditis,
    nephritis, systemic vasculitis, cerebral manifestations):
    high-dose intravenous corticosteroid + iv.
    cyclophosphamide + in some cases: plasmapheresis or
    iv. immunoglobulin, or, instead of cyclophosphamide:
    mycophenolate mofetil (not registered in the EU)
    Some cases of nephritis (especially membranous),
    myositis, thrombocytopenia: cyclosporine

    Use of corticosteriod to treat various lupus


    manifestation

    Clinical featureinitial

    dose of prednisolone

    Arthritis (poorly responding to NSAIDs)


    pleuritis
    Pericarditis

    20-30mg/d, reducing
    by about 5mg/wk if
    symptoms abate

    Haemolytic anemia
    Thrombocytopenia

    1mg/kg/d for about 1M


    reduce by 10mg/d if
    blood tests improve

    Nephritis

    1mg/kg/d for about 1M

    Neuropsychiatric

    controversal!
    1-2mg/kg/d,
    0.5-1g/d

    methylprednisolone

    Other therapy

    Plasma exchange
    Intravenous Immunoglobulin
    Stem cell transplantation
    Immune therapy ( anti-IL10, antiCD20, and immune tolerance
    therapy)

    LUPUS RELATED SYNDROMES


    Drug Induced Lupus
    Classically associated with hydralazine,
    isoniazid, procainamide
    Male:Female ratio is equal
    Nephritis and CNS abnormalities rare
    Normal complement and no anti-DNA
    antibodies
    Symptoms usually resolve with stopping drug

    LUPUS RELATED SYNDROMES


    Antiphospholipid Syndrome (APS)
    Hypercoagulability with recurrent thrombosis of either
    venous or arterial circulation
    Thrombocytopenia-common
    Pregnancy complication-miscarriage in first trimester
    Lifelong anticoagulation warfarin is currently
    recommended for patients with serious complications
    due to common recurrence of thrombosis
    Antiphospholipid Antibodies
    Primary when present without other SLE feature.
    Secondary when usual SLE features present

    PROGNOSIS
    Unpredictable course
    10 year survival rates exceed 85%
    Most SLE patients die from
    infection, probably related to
    therapy which suppresses immune
    system

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