Flashcards in 8.3. Mulit-System Autoimmune Disease - Immunological Investigation Deck (26):
What is the Diagnosis of Mulit-System Autoimmune Disease dependent on?
1. Cardinal Clinical Features (History / Exam)
4. Tissue Biospy
5. Exclusion of Differential Diagnosis
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
What are the 3 immune Pathology's?
3. Immune Deficiency
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)
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
What is the purpose of testing, with relation to the Disease?
3. Prognosis / Risk Stratification
4. Monitoring / Progression
What is the purpose of testing, with relation to the Treatment?
What are the main Immunology Tests?
1. Antinuclear Antibodies (ANA)
2. Antinuclear Cytoplasmic Antibodies (ANCA)
3. Antiphospholipid Antibodies
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
What are the 4 patterns Antinuclear Antibodies (ANA) can present in?
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)
When are Speckled Antinuclear Antibodies (ANA) present?
When there are Autoantibodies directed against Non-Chromosomal Nuclear Proteins
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
When are Peripheral Antinuclear Antibodies (ANA) present?
Where Staining is confined to the Nuclear Membrane:
dsDNA - SLE / some autoimmune Liver Disease
What type of test is an Antinuclear Antibodies (ANA) test?
1. Being ANA positive has little useful diagnostic specificity
2. Should lead onto more specific tests
What is the function of an Antinuclear Antibodies (ANA) test?
2. Monitoring of Disease Activity
3. Subclassification of Disease
What is Antinuclear Cytoplasmic Antibodies (ANCA) associated with?
1. Granulomatosis Polyangiitis (Wegner's Granulomatosis)
2. Microscopic Polyangiitis
3. Eosinophilic Granulomatosis Polyangiitis
What are the 2 sub-classes of Antinuclear Cytoplasmic Antibodies (ANCA)?
1. Cytoplasmic ANCA (C-ANCA)
2. Perinuclear ANCA (P-ANCA)
How useful is the Antinuclear Cytoplasmic Antibodies (ANCA) test alone?
It is not as it is not disease-specific:
4. Connective Tissue Disease
5. Inflammatory Bowel Disease
What are the 2 types of Antiphospholipid Antibody Syndrome?
What can cause Secondary Antiphospholipid Antibody Syndrome?
1. Connective Tissue Disorders
2. Chronic Infection
4. Lymphoproliferative Disease
What are the Major Features of an Antiphospholipid Antibody Syndrome?
1. Vascular Thrombosis
2. Recurrent Foetal Loss
3. Liverdo Reticularis
What are the common Antiphospholipid Antibodies?
1. Antocardiolipin Antibodies
2. Anti-Beta2 Glycoprotein 1 Antibodies
3. "Lupus Anticoagulant"
What is the main function of the Complement System?
Maintaining the Solubility of Immune Complexes
What are the Clinical Uses of Complement Asseys?
1. Diagnosis / Monitoring of a Disease Activity
2. Detection of Hereditary Deficiencies