Systemic Lupus Erythematosus

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Systemic Lupus Erythematosus
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Case

• A 17-year-old girl was admitted with a chief
  complaint of recurrent fever for three years, a
  rash for 2 months, and abdominal pain for 5 days.
  She had suffered from recurrent high grade fever
  since 1998, accompanied by necrotic
  lymphadenitis. Her condition had improved with
  prednisone. Two months earlier, she began to
  notice fevers, dark-red maculopapules(???),
  Raynauds pheno-menon(?????), and severe
  alopecia. At the time, laboratory tests showed
  ANA 1320 (homogeneous pattern), negative
  anti-dsDNA, and anti-SSA 164. She was diagnosed
  with SLE .

Butterfly-erythema (????)
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What is SLE?

• SLE is the prototypic autoimmune disease
  belonging to a family of diseases often referred
  to as "connective tissue diseases" a term
  deriving from the observation that the connective
  tissues of multiple organs were abnormal in these
  diseases in the absence of obvious infection or
  neoplasm.

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• Cultural diversity
• SLE strikes women 10 times as often as men,
  increasing to 15 times as often during
  childbearing years(???). It occurs worldwide but
  is most prevalent among people of Asian,
  Hispanic, or African origin
• Population studies have shown an incidence
  ranging from 2 to 50 cases/100,000 with clear
  variation according to ethnic group.

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Causes

• The exact cause of SLE remains a mystery, but
  available evidence points to interrelated
  immunologic, environmental, hormonal, and genetic
  factors.

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Genetic factors

• There is an increased frequency of SLE and
  asymptomatic immunologic abnormalities in close
  relatives of SLE patients.
• Monozygotic twins(?????).
• Ethnic differences .
• Certain histocompatibility(?????) antigens (HLA)
  (e.g. HLA- DR2, DR3)

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Environmental factors

• Drug induced lupus
• procainamide(?????), chlorpromazine(???? )
• Diet
• Nutritional factors may modify disease but this
  has not been adequately studied.
• Infectious
• Viral and other infectious etiologies.

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• Certain related factors
• UV radiation(?????)
• Hairdye(???)

Hairdye
Sunshine
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UV light
infection
Drugs
Self Ag
Skin cell
APC
External Ag
T cell
T cell
APC
AB
IC
B cell
target
IC
Ab
Detective IC clearance and regulatory circuits
Overview of the pathogenesis of SLE
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Immune System
Genes
Loss self-tolerance (certain antigen)
Exogenous factor
SLE pathogenesis mechanism
Endogenous factor
Androgen
Estrogen
Humoral Immunity
Cellular Immunity
T suppressor ?
B cells
Cytokines(IL-10)
T help?
Increased Ab production to auto-antigen
Auto-antibodies
Immune complex formation
Genes C1?RBC CR1?MPS phagocytosis?
Poor immune complex clearance
Excess immune complex deposition
Inflammation
Pathology
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Pathophysiology

• Autoimmunity is believed to be the prime
  mechanism involved with SLE. The body produces
  antibodies against components of its own cells,
  such as the antinuclear antibody (ANA), and
  immune complex disease follows.
• Patients with SLE may produce antibodies against
  many different tissue components, such as red
  blood cells, neutrophils, platelets, lymphocytes,
  or almost any organ or tissue in the body.

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Characteristic pathology change???????

• Hematoxylin corpuscle(?????)_at_
• Onionskin(????) change, spleen central artery
  and other arteriescentripetal textile fiber
  proliferation(???????)_at_
• verrucous endocarditis(??????)_at_
• glomerulus fluorescent staining(????) appears the
  full house to be bright(???)_at_

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Main symptoms

• Weakness
• Fever
• more than 80 of the patients have a fever.
• Skin and mucosal damage
• 75 -80 of the patients skin or mucosal
  damage.
• Photosensitivity(???)
• Cheek erythema(????)
• Bullous lupus erythematosus(??????? )
• Alopecia (??)

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• Cheek erythema(????)
• First in cheek, suddenly appeared for the
  small light red, bright red or purple spots,
  gradually expanded to bridge of the nose, and
  linked into such as butterflies like grouper.
  Recurrence of this plaque can happen again.

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SLE CLINIC FEATURES(1)

• GENERAL
• Lethargy
• Fever
• Weight loss
• Lymphadenopathy
• DERMATOLOGICAL
• Vasculitis
• Alopecia
• Discoid
• Panniculitis
• Photosensitivity

• MUSCULOSKELETAL
• Arthralgia/Arthritis
• Myalgia/Myositis
• Tenosynovitis
• GASTROINTESTINAL
• Anorexia
• Nausea
• Abdominal pain
• Hepato/splenomegaly

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SLE CLINIC FEATURES (2)

• RENAL_at_
• Proteinuria
• Haematuria
• Casts
• Nephrotic syndrome
• CARDIOPULMONARY
• Shortness of breath
• Pleurisy
• Pleural effusion
• Cardiomegaly
• Pericarditis

• CEREBRAL
• Migraine
• Anxiety/depression
• Psychosis
• Seizures
• Cranial/Peripheral
• HEMATOLOGICAL
• Anemia-iron deficient
• Anemia-haemolytic
• Lymphopenia
• Thrombocytopenia

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Diagnosis

• Test results that may indicate SLE include
• complete blood count with differential possibly
  showing anemia and a decreased white blood cell
  count
• platelet count, which may be decreased
• erythrocyte sedimentation rate, which is often
  elevated
• serum electrophoresis, which may show
  hypergammaglobulinemia.

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Drugs for SLE

• Corticosteroids(?????)
• most commonly used. It can suppress
  inflammation and are commonly used in controlling
  SLE.
• Immunosuppressive Drugs(?????)
• CTX(????), AZA(????)
• IVIG(?????)

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Thank you!
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