Autoimmunity Flashcards
Tolerance - what is it?
Must be able to react to harmful foreign antigens (pathogens) and altered self (malignancy), but not self antigens
This lack of reactivity to self is known as immune tolerance
Lymphocytes that recognise self-antigens are killed or inactivated.
When this fails, then you get autoimmunity
Autoimmune Polyendocrinopathy (AIRE deficiency):
What caused a lack of central T cell tolerance?
What are some examples of this?
Inability to present antigens to the thymocytes in the thymus
Without this, central T cell tolerance is impaired and autoimmunity develops
Hypothyroidism, Addison’s, IDDM etc.
FOXP3 Deficiency (IPEX syndrome):
What does this cause?
Childhood - range of presentations
FOXP3 is essential transcription factor for development of T-reg cells
Deficiency leads to lack of T-reg cells
Examples of Autoimmune diseases?
Rheumatoid arthritis
Systemic lupus erythematosis (SLE)
Granulomatosis with polyangiitis (GPA), and other vasculitides
Psoriasis / Psoriatic arthritis
Graves’ disease
Goodpasture’s
Myasthenia gravis
Type 1 diabetes
Scleroderma
Sjogren’s syndrome
Pemphigus/pemphigoid
Antiphospholipid syndrome
Autoimmune haemolytic
anaemia, thrombocytopaenia
Classification used?
Gell Coombs classification
Type II Hypersensitivity:
What substance mediated this?
What Ig’s are involved? - 2
2 examples?
Antibodies mediated hypersensitivity
IgG/IgM
Goodpastures
MG
Graves
Type III Hypersensitivity:
Mechanism?
2 examples?
Immune complex mediated hypersensitivity
Immune complex formation
Deposition in tissue - local inflammation
RA
SLE
Lupus - Antibodies used? - 2
What complement would you measure?
What Ig can you measure?
ANA - anti-nuclear antibodies
dsDNA
Complement C3/C4
IgG
Lupus Features:
Cutaneous manifestations - 4
Systemic - 5
Treatment:
- Main treatment!
Arthritis
Oral ulceration
Photosensitive rashes
Raynauds
Pleurisy, pericarditis
Glomerulonephritis
Anaemia/thrombocytopenia
Neuro and Cardiac Disease
Anti-malarial drugs (hydroxychloroquine)