Lupus

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• Lupus
• Systemic Lupus Erythematosus
• SLE

Dr. M Jokar
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Definition

• Lupus wolf
• Chronic
• Unknown etiology
• Connectie tissue dis.
• Autoimmune
• Multisystem

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Lupus Types

• SLE
• DLE(Discoid lupus erythematosus)
• Drug induced lupus
• Neonatal lupus

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Epidemiology

• Prevalence 40-150 in 100000
• Urban area gt rural areas

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Epidemiology

• Race
• The prevalence of SLE is higher among Asians,
  Afro-Americans, Afro-Caribbeans, and Hispanic
  American

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Epidemiology

• Sex
• 95 in women
• Children 31
• In adults 7-151
• Older individuals 81

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Epidemiology

• Age all ages but more common in child- bearing
  ages
• 20 lt16 years
• 65 16-55 years
• 15 gt55 years

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Pathogenesis

• Genetic background Environmental factors
• Abnormal immune response
• B and T cell hyperactivity
• Autoantibodies Immue complexes
• Tissue damage

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Genetic background

• Evidence for genetic predisposition
• concordance in monozygotic 24-58
• first degree relatives 5-12
• genes
• C4AQO HlA-DR2 HLA-DR3
  
  

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Environmental factors

• UV-B(sometimes UV-A)
• Chemicals hydrazines-hair dye?
• Viruses

• Drugs
• Sex hormones
• Stress
• Foods

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Autoantibodies

• ANA
• Anti-DNA
• Anti-Sm
• Anti-RNP ,Anti-RoSS-A , Anti-LaSS-B
• Anti-Histone , Antiphospholipid , Antiplatelet
• Antierythrocyte , Antilymphocyte
• Antineuronal

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Clinical manifestations

• Most patients experience exacerbations
  interspersed with periods of relative quiescence.
  
• True remissions with no symptoms and requiring no
  therapy occur in up to 20 but are usually not
  permanent.
• Systemic symptoms are usually prominent and
  include fatigue, malaise, fever, anorexia, and
  weight loss.

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Clinical manifestations

• Can affect any organ in the body including the
  joints, skin, lungs, heart, blood, kidney, or
  nervous system.
• Can range from mild to life threatening.
• No two people will have identical symptoms.

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Musculoskeletal

• Almost all patients experience arthralgias and
  myalgias
• most develop intermittent arthritis
• Joint deformities are unusual
• Erosions are rare
• Myopathy and myositis
• Osteoporosis and AVN

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Cutaneous Manifestations

• Malar ("butterfly") rash

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Cutaneous Manifestations

• Discoid lupus erythematosus (DLE)

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DISCOID LUPUS

• Affects the skin, hair or mucous membranes.
• Identified by a rash or lesions.
• Negative or low titer ANA.
• Diagnosed by biopsy of rash.
• 10 will evolve into SLE.

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Cutaneous Manifestations

• Maculopapular rash
• Subacute cutaneous lupus erythematosus (SCLE)
• Alopecia
• Photosensitivity
• Other rashesmaculopapular,urticaria, bullae,
  erythema multiforme

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Alpecia
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Renal Manifestations

• One-half have clinical nephritis
• Most have immunoglobulins deposited in glomeruli
• Early in the disease most are asymptomatic
• Urinalysis shows hematuria, cylindruria, and
  proteinuria

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WHO classification of lupus nephritis

• Class I normal
• Class II mesangial lupus nephritis
• Class IIIfocal proliferative nephritis
• CLASS IVdiffuse proliferative nephritis
• Class V membranous lupus nephritis
• Class VI ESRD

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Nervous System

• CNS
• PNS

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CNS

• Any region of the brain can be involved in SLE
• Mild cognitive dysfunction is the most
• Seizures ,Headaches, demyelinating disorders,
  cerebrovascular disease, movement disorders,
  aseptic meningitis, myelopathy, subarachnoid
  hemorrhage
• Psychosis,mood disorders, depression and anxiety

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PNS

• Mononeuropathy
• Polyneuropathy
• Mononeuritis multiplex
• Acute demyelinating polyneuropathy
  (Guillain-Barre)

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Vascular System

• Thrombosis, emboli
• vasculitis
• Atherosclerosis
• Raynauds phenomenon

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Hematologic Manifestation

• Anemia Anemia of chronic disease,hemolytic
  anemia
• Leukopenia (usually lymphopenia)
• Thrombocytopenia
• Antiphospholipid antibodies and lupus
  anticoagulant
• Bleeding syndromes
• Splenomegaly,lymphadenopathy

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Heart

• Pericarditis
• Myocarditis
• Valvular insufficiency (usually aortic or mitral)
• Libman-Sacks endocarditis
• Myocardial infarcts

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Respiratory tract

• Pleurisy and pleural effusions
• pneumonitis
• Interstitial pneumonitis leading to fibrosis
• Pulmonary hypertension
• Respiratory distress syndrome
• Intraalveolar hemorrhage
• Infections

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Gastrointestinal System

• Nausea, diarrhea, and vague discomfort
• Peritonitis
• Vasculitis of the intestine
• Acute pancreatitis
• Hepatitis
• Hepatomegaly

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Ocular Manifestation

• Retinal vasculitis
• conjunctivitis
• episcleritis
• optic neuritis
• sicca syndrome

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LABORATORY MANIFESTATIONS

• CBCanemia, leukopenia, lymphopenia, and
  thrombocytopenia
• ESR,CRP
• Urinalysis proteinuria, hematuria, and cellular
  or granular casts

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LABORATORY MANIFESTATIONS

• Immunologic tests
• ANA
• AntiDNA
• AntiSm
• Anticardiolipin
• C3,C4,CH50

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PREGNANCY

• Fertility rates are normal
• Spontaneous abortion and stillbirths are frequent
  (10 to 30), especially in women with LA and/or
  aCL
• Disease flares in a small proportion, especially
  during the 6 weeks postpartum

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Neonatal lupus

• Caused by transmission of maternal anti-Ro across
  the placenta
• Transient skin rash
• Permanent heart block
• Transient thrombocytopenia

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DRUG-INDUCED LUPUS

• Drugsprocainamide, hydralazine, isoniazid,
  chlorpromazine, D-penicillamine, methyldopa,
  quinidine, interferon a, and possibly hydantoin,
  ethosuximide, and oral contraceptives
• Clinical manifestations systemic complaints
  arthralgias polyarthritis and pleuropericarditis
• Renal and CNS involvement are rare
• LabAll patients have ANA and most have
  antibodies to histones,

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DRUG-INDUCED LUPUS

• Anti dsDNA and hypocomplementemia are rare
• Anemia, leukopenia, LA, aCL, thrombocytopenia,,,
  false-positive VDRL, and positive direct Coombs'
  tests can occur
• Symptoms rarely persist more than 6 months ANA
  may persist for years

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PROGNOSIS

• Survival 90 to 95 at 2 years
• 82 to 90 at 5 years
• 71 to 80 at 10 years
• 63 to 75 at 20 years
• Poor prognosis factors
• high serum
  creatinine,Black Race ,hypertension,nephrotic
  syndrome thrombocytopenia, serious CNS
  involvement antibodies to phospholipids

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Causes Of Death

• Cardiac involvement
• Pulmonary involvement
• CNS involvement
• Renal involement
• Infections

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DIAGNOSIS

• Medical history (including family history)
• Complete physical examination
• Laboratory tests
• Skin or kidney biopsy

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ACR DIAGNOSTIC CRITERIA

• Skin criteria
• 1. Butterfly rash
• 2. Discoid rash
• 3. Photosensitivity
• 4. Oral ulcers

• Systemic criteria
• 5. Arthritis
• 6. Serositis
• 7. Kidney disorder
• 8. Neurologic disorder

Laboratory criteria 9. Hematologic
abnormalities 10. Immunologic disorder 11.
Antinuclear antibody
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COMMON LUPUS MEDICATIONS

• NSAIDs
• Antimalarials
• Corticosteroids
• Immunosuppressants
• Investigational (research)