Systemic Lupus Erythematosus

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Systemic Lupus

Erythematosus

_{Erythematosus}

A multiA multi--system autoimmune diseasesystem autoimmune disease

Etiology unknownEtiology unknown

Most common in women, young to Most common in women, young to

middle

middle––agedaged

Highly variable clinical presentation Highly variable clinical presentation

in individual patients in individual patients

Harvard-MIT Division of Health Sciences and Technology HST.021: Musculoskeletal Pathophysiology, IAP 2006 Course Director: Dr. Dwight R. Robinson

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SLE, Systems Involved

-

_-

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₍₁₎

Skin: Skin: photosensitive photosensitive erythematouserythematous

eruption eruption

Joints: Joints: inflammatory arthritis, usually inflammatory arthritis, usually

symmetrical without joint destruction symmetrical without joint destruction

Kidneys: Kidneys: GlomerulonephritisGlomerulonephritis

Cardiopulmonary: Cardiopulmonary: Pleurisy, Pleurisy, pericarditispericarditis, ,

pneumonia and pulmonary hemorrhage, pneumonia and pulmonary hemorrhage,

myocarditis

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SLE, systems involved

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_-

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₍₂₎

Central nervous system: Central nervous system: Seizures, Seizures,

psychiatric symptoms, psychiatric symptoms,

cerebrovascular

cerebrovascular accidents_accidents

Blood: Blood: Anemia, often hemolytic. Anemia, often hemolytic.

Leukopenia

Leukopenia. Thrombocytopenia_{. Thrombocytopenia}

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SLE: Anti

-

_-

Nuclear Antibodies

_{Nuclear Antibodies}

Serum Antibody Nucleus

Cytoplasm Slide

Fluorescent Labeled Anti-Immunoglobulin

PRINCIPLE OF INDIRECT IMMUNOFLUORESCENCE

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SLE: Prognosis and Treatment

Prognosis varies from mild to severe Prognosis varies from mild to severe

or fatal. or fatal.

Treatment is nonTreatment is non--specific, and is not specific, and is not

curative. curative. • • NSAIDsNSAIDs • • HydroxychloroquineHydroxychloroquine • • GlucocorticoidsGlucocorticoids •

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Diffuse Systemic Sclerosis

(Scleroderma)

A chronic, progressive inflammatory A chronic, progressive inflammatory

auto

auto-_-immune disease leading to _{immune disease leading to}

fibrosis in several organ systems and fibrosis in several organ systems and

in the vasculature in the vasculature

No specific treatment; No specific treatment; only palliative only palliative

measures with limited efficacy. measures with limited efficacy.

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CLASSIFICATION OF SCLERODERMA

Diffuse Cutaneous Scleroderma Limited Cutaneous Scleroderma CREST Syndrome Systemic Sclerosis (SSc) Localized Scleroderma Morphea Linear Scleroderma Overlap Syndromes Scleroderma-Like Syndromes

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Idiopathic Inflammatory

Myopathy

Inflammatory Inflammatory myopathymyopathy of unknown of unknown

etiology etiology

Probably has an autoimmune Probably has an autoimmune

pathogenesis pathogenesis

Usually a chronic progressive diseaseUsually a chronic progressive disease

Causes proximal skeletal muscle weaknessCauses proximal skeletal muscle weakness

This entity and other diseases of muscle This entity and other diseases of muscle

will be discussed by Drs. Brown and Johns will be discussed by Drs. Brown and Johns

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POLYMYOSITIS: CLASSIFICATION Adult Polymyositis

Adult Dermatomyositis

Inflammatory Myositis Associated with Cancer Childhood Dermatomyositis or Polymyositis

Myositis Associated with Connective Tissue Disease

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Idiopathic Inflammatory

Myositis

_Myositis

Diagnosis

Elevation of serum levels of enzymes Elevation of serum levels of enzymes

intrinsic to skeletal muscle;

intrinsic to skeletal muscle; creatinecreatine phosphokinase

phosphokinase is the most sensitive _{is the most sensitive} and specific.

and specific. TransaminasesTransaminases also are also are elevated.

elevated.

MyopathicMyopathic changes on EMGchanges on EMG

Abnormal muscle biopsyAbnormal muscle biopsy

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Idiopathic Inflammatory

Myositis

_Myositis

Treatment

GlucocorticoidsGlucocorticoids: : Relatively high Relatively high

doses required. Toxicity is frequent. doses required. Toxicity is frequent.

Other immunosuppressive agents: Other immunosuppressive agents:

methotrexate

methotrexate, , azathioprineazathioprine

Physical therapyPhysical therapy

Search for underlying malignancy Search for underlying malignancy

where appropriate where appropriate

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Sjogren

’

_’

s

_s

Syndrome

_Syndrome

(

Keratoconjunctivitis

_{Keratoconjunctivitis}

sicca

_sicca

)

₎

An inflammatory autoAn inflammatory auto--immune disease immune disease

involving the salivary and

involving the salivary and lacrymallacrymal glands, glands, sometimes other exocrine glands.

sometimes other exocrine glands.

Causes dryness of the eyes and mouth.Causes dryness of the eyes and mouth.

May be associated with other rheumatic May be associated with other rheumatic

diseases, such as rheumatoid arthritis and diseases, such as rheumatoid arthritis and

SLE. SLE.

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Vasculitis

A bewildering array of clinical A bewildering array of clinical

syndromes with the common feature syndromes with the common feature

of necrotizing inflammation of blood of necrotizing inflammation of blood

vessels. vessels.

The etiology is often unknown, but The etiology is often unknown, but

some infections, e.g., hepatitis C, some infections, e.g., hepatitis C,

can cause

can cause vasculitisvasculitis through immune through immune complex deposition

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Vasculitis

Vasculitis may be classified on the basis of may be classified on the basis of the size of the arteries involved

the size of the arteries involved

VASCULITIS SYNDROMES Polyarteritis Nodosa Churg-Strauss (Allergic Granulomatosis and Angiitis) Hypersensitivity Vasculitis

Small, Medium Arteries Small, Medium Arteries

Arterioles, Venules, Capillaries, Rarely Small Arteries

Vasculitis Syndrome Vessel Involved

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VASCULITIS SYNDROMES (Cont.)

Henoch-Schonlein Purpura Takayasu's Arteritis

Temporal Arteritis

Wegener's Granulomatosis

.. _{Venules, Arterioles, Capillaries}

Medium, Large Arteries Medium, Large Arteries

Small Arteries, Veins, Medium Arteries

Vasculitis Syndrome Vessel Involved

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Vasculitis

: Diagnosis

_{: Diagnosis}

Recognition of clinical syndromes Recognition of clinical syndromes

caused by

caused by vasculitisvasculitis

Elevated levels of acute phase Elevated levels of acute phase

reactants reactants

Positive test for antiPositive test for anti--neutrophilneutrophil

cytoplasmic

cytoplasmic antibodies (ANCA), are _{antibodies (ANCA), are} present in some syndromes

present in some syndromes

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Vasculitis

: Treatment

_{: Treatment}

Immunosuppressive therapy with Immunosuppressive therapy with

glucocorticoids

glucocorticoids and and cytotoxiccytotoxic or or antimetabolic

antimetabolic drugs_drugs

Prognosis is guarded, but most Prognosis is guarded, but most

syndromes are treated effectively, syndromes are treated effectively,

although cures are not always although cures are not always

achieved achieved