8.3. Mulit-System Autoimmune Disease - Immunological Investigation Flashcards Preview

3rd Year - MSK Diseases > 8.3. Mulit-System Autoimmune Disease - Immunological Investigation > Flashcards

Flashcards in 8.3. Mulit-System Autoimmune Disease - Immunological Investigation Deck (26):
1

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

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

2

What can Mulit-System Autoimmune Disease mimic?

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

3

What are the 3 immune Pathology's?

1. Autoimmunity
2. Hypersensitivity
3. Immune Deficiency

4

What is the pathology of Autoimmunity?

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)

5

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

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

6

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

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

7

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

1. Planning
2. Selection
3. Triage
4. Monitoring
5. Evaluation

8

What are the main Immunology Tests?

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

9

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

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

10

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

1. Homogeneous
2. Speckled
3. Nucleolar
4. Peripheral

11

When are Homogeneous Antinuclear Antibodies (ANA) present?

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)

12

When are Speckled Antinuclear Antibodies (ANA) present?

When there are Autoantibodies directed against Non-Chromosomal Nuclear Proteins

13

When are Nucleolar Antinuclear Antibodies (ANA) present?

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

14

When are Peripheral Antinuclear Antibodies (ANA) present?

Where Staining is confined to the Nuclear Membrane:
dsDNA - SLE / some autoimmune Liver Disease

15

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

Screening only:
1. Being ANA positive has little useful diagnostic specificity
2. Should lead onto more specific tests

16

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

1. Diagnosis
2. Monitoring of Disease Activity
3. Subclassification of Disease

17

What is Antinuclear Cytoplasmic Antibodies (ANCA) associated with?

Vasculitis:
1. Granulomatosis Polyangiitis (Wegner's Granulomatosis)
2. Microscopic Polyangiitis
3. Eosinophilic Granulomatosis Polyangiitis

18

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

1. Cytoplasmic ANCA (C-ANCA)
2. Perinuclear ANCA (P-ANCA)

19

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

It is not as it is not disease-specific:
1. Infection
2. Inflammation
3. Drugs
4. Connective Tissue Disease
5. Inflammatory Bowel Disease

20

What are the 2 types of Antiphospholipid Antibody Syndrome?

1. Primary
2. Secondary

21

What can cause Secondary Antiphospholipid Antibody Syndrome?

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

22

What are the Major Features of an Antiphospholipid Antibody Syndrome?

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

23

What are the common Antiphospholipid Antibodies?

1. Antocardiolipin Antibodies
2. Anti-Beta2 Glycoprotein 1 Antibodies
3. "Lupus Anticoagulant"

24

What is the main function of the Complement System?

Maintaining the Solubility of Immune Complexes

25

What are the Clinical Uses of Complement Asseys?

1. Diagnosis / Monitoring of a Disease Activity
2. Detection of Hereditary Deficiencies

26

What are Cryoglobulins?

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

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