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-MSK Diseases > 8.3. Mulit-System Autoimmune Disease - Immunological Investigation > Flashcards

8.3. Mulit-System Autoimmune Disease - Immunological Investigation Flashcards

1
Q

What is the Diagnosis of Mulit-System Autoimmune Disease dependent on?

A
  1. Cardinal Clinical Features (History / Exam)
  2. Immunology
  3. Imaging
  4. Tissue Biospy
  5. Exclusion of Differential Diagnosis
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2
Q

What can Mulit-System Autoimmune Disease mimic?

A
  1. Drugs - Cocaine / Minocycline / PTU
  2. Infection - HIV / Endocarditis / Hepatitis / TB
  3. Malignancy - Lymphoma
  4. Cardiac Myxoma
  5. Cholesterol Emboli
  6. Scurvy
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3
Q

What are the 3 immune Pathology’s?

A
  1. Autoimmunity
  2. Hypersensitivity
  3. Immune Deficiency
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4
Q

What is the pathology of Autoimmunity?

A
  1. Genetic / Regulatory / Hormonal / Environmental / Other Factors
  2. Breakdown of Immunlogical Tolerance
  3. Pathological Autorecognition (T-Cell / B-Cell / Innate Immune Mechanisms)
  4. Inflammation and Tissue Damage (Autoimmunity - Hypersensitivity)
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5
Q

What does the Spectrum of Autoimmune Diseases, align the different Autoimmune Diseases to?

A
  1. Organ Specific
  2. Non-Organ Specific
    Note - There are many which are somewhere in between
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6
Q

What is the purpose of testing, with relation to the Disease?

A
  1. Diagnosis
  2. Sub-Clasification
  3. Prognosis / Risk Stratification
  4. Monitoring / Progression
  5. Screening
  6. Research
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7
Q

What is the purpose of testing, with relation to the Treatment?

A
  1. Planning
  2. Selection
  3. Triage
  4. Monitoring
  5. Evaluation
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8
Q

What are the main Immunology Tests?

A
  1. Antinuclear Antibodies (ANA)
  2. Antinuclear Cytoplasmic Antibodies (ANCA)
  3. Antiphospholipid Antibodies
  4. Complement
  5. Cryoglobulins
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9
Q

What is the Antinuclear Antibodies (ANA) used to diagnose?

A
  1. Systemic Lupus Erythematous (SLE) (>95%)
  2. Other Connective Tissue Mulit-System Autoimmune Diseases (Sjogren’s, Scleroderma, Dermomysitis)
    Note - This has a high Sensitivity for SLE, but a low Specificity - if ANA is negative SLE is very unlikely
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10
Q

What are the 4 patterns Antinuclear Antibodies (ANA) can present in?

A
  1. Homogeneous
  2. Speckled
  3. Nucleolar
  4. Peripheral
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11
Q

When are Homogeneous Antinuclear Antibodies (ANA) present?

A

Where there are Autoantibodies directed against Chromosomal Autoantigens:

  1. dsDNA (SLE / some autoimmune liver disease)
  2. ssDNA (non-specific / many inflammatory disorders)
  3. Histone Proteins (Drug Induced Lupus / other Connective Tissue Disorder)
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12
Q

When are Speckled Antinuclear Antibodies (ANA) present?

A

When there are Autoantibodies directed against Non-Chromosomal Nuclear Proteins

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13
Q

When are Nucleolar Antinuclear Antibodies (ANA) present?

A

Where Autoantibodies are directed solely against the Nucleolar RNA:

  1. Scleroderma, Systemic Sclerosis, Overlap Syndormes
  2. Clinical Variants
  3. A range of Variant-Specific Antibodies
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14
Q

When are Peripheral Antinuclear Antibodies (ANA) present?

A

Where Staining is confined to the Nuclear Membrane:

dsDNA - SLE / some autoimmune Liver Disease

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15
Q

What type of test is an Antinuclear Antibodies (ANA) test?

A

Screening only:

  1. Being ANA positive has little useful diagnostic specificity
  2. Should lead onto more specific tests
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16
Q

What is the function of an Antinuclear Antibodies (ANA) test?

A
  1. Diagnosis
  2. Monitoring of Disease Activity
  3. Subclassification of Disease
17
Q

What is Antinuclear Cytoplasmic Antibodies (ANCA) associated with?

A

Vasculitis:

  1. Granulomatosis Polyangiitis (Wegner’s Granulomatosis)
  2. Microscopic Polyangiitis
  3. Eosinophilic Granulomatosis Polyangiitis
18
Q

What are the 2 sub-classes of Antinuclear Cytoplasmic Antibodies (ANCA)?

A
  1. Cytoplasmic ANCA (C-ANCA)

2. Perinuclear ANCA (P-ANCA)

19
Q

How useful is the Antinuclear Cytoplasmic Antibodies (ANCA) test alone?

A

It is not as it is not disease-specific:

  1. Infection
  2. Inflammation
  3. Drugs
  4. Connective Tissue Disease
  5. Inflammatory Bowel Disease
20
Q

What are the 2 types of Antiphospholipid Antibody Syndrome?

A
  1. Primary

2. Secondary

21
Q

What can cause Secondary Antiphospholipid Antibody Syndrome?

A
  1. Connective Tissue Disorders
  2. Chronic Infection
  3. Drugs
  4. Lymphoproliferative Disease
22
Q

What are the Major Features of an Antiphospholipid Antibody Syndrome?

A
  1. Vascular Thrombosis
  2. Recurrent Foetal Loss
  3. Liverdo Reticularis
  4. Thrombocytopenia
23
Q

What are the common Antiphospholipid Antibodies?

A
  1. Antocardiolipin Antibodies
  2. Anti-Beta2 Glycoprotein 1 Antibodies
  3. “Lupus Anticoagulant”
24
Q

What is the main function of the Complement System?

A

Maintaining the Solubility of Immune Complexes

25
Q

What are the Clinical Uses of Complement Asseys?

A
  1. Diagnosis / Monitoring of a Disease Activity

2. Detection of Hereditary Deficiencies

26
Q

What are Cryoglobulins?

A

Immunoglobulin Molecules which reversibly Precipitate out of Solution at Temperatures >37 degrees

-MSK Diseases (43 decks)

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