OBJ - Introduction to Autoimmune Disease Flashcards
Describe which autoantibodies are associated with which autoimmune diseases, and the importance their use in clinical diagnosis.
Table 4-10/Screenshot
Be able to ID/match
First Aid Immunology FC’s
Autoimmunity:
- Breakdown in self-tolerance resulting in immune-mediated injury of host tissue
- Inheritance of susceptibility genes (HLA and non-HLA genes)
- HLA: pattern and strength of peptide binding for presentation of T Cells
- non-HLA: involve various components of immune system
Genetic factors? Unclear, but increased incidence in monozygotic twins
No known cause for loss of tolerance
Systemic: (SLE)
Organ specific: (Graves Disease, Myasthemia gravis)
Progressive with relapses & remission
Type II, III, IV Hypersensitivity mechanisms of injury (isolated or combined)
Central vs Peripheral Tolerance
Both happen to ensure we don’t attack ourself
(This is supposed to happen & doesn’t in Autoimmune)
Central tolerance
- Deletion of self-reactive lymphocytes in thymus & bone marrow following encounter with self antigen ( in primary lymph organs)
- AIRE = Autoimmune regulatory: factor produced to prevent IS from attaching itself)
- Self reactive B cells - signaled to either apoptosis or receptor editing (mulligan)
not 100% - some get through
Peripheral tolerance
- Deletion of self-reactive lymphocytes in periphery once they’ve matured
- Anergy - functional inactivation; get exposure to TCR or BCR but no costimulation by APC => no activation
- Treg-suppression - regulatroy t cells
- Activation induced cell death (AICD) - fas mediated apoptosis
Systemic Lupus Erythematosis
Lupus = chronic inflammatory disease from body’s immune system attaching own tissues & organs
SLE: type II & type III hypersensitivity
Antibodies = ANA test: Anti-Smith & Anti-dsDNA
Anti-smith = Ab to spliceosomal snRNPs
Anti-cardiolipin (lupus anticoagulant)
1) Nuclear antigen targeted
2) Twins study Achy muscles & joints, ulcers in mouth, pericarditis, pleuritis
Poor circulation in fingers & toes
Constrictive pericarditis
Raynaud’s syndrome
Systemic sclerosis (scleroderma)
as unique pathologic entities and identify features of the epidemiology, genetics, and pathogenesis.
- Immune activation and vascular abnormalities with excessive fibrosis
- Increased synthesis of ECM proteins -> fibrosis of skin & parenchymal organs)
Antibodies:
Anticentromere
Anti-Scl-70 (anti-DNA topoisomerease)
Sjoegren syndrome
as unique pathologic entities and identify features of the epidemiology, genetics, and pathogenesis.
S/s: enlarged salivary gland, dry eyes, dry mouth.
Isolated = primary/disease by itself )
Associated = secondary/ with other AID like RA/SLE
Ex: Maury Gibson
Anti-SSA, Anti-SSB, Anti-Ro, Anti-La
Rheumatoid arthritis
as unique pathologic entities and identify features of the epidemiology, genetics, and pathogenesis.
Autoantibody = RF = Rheumatoid Factor
Inflammatory myopathies
Polymyositis/Dermatomyositis
Amyloidosis
- Deposition of extracellular MISFOLDED proteins (Amyloid Fibrils) that aggregate to form insoluble fibrils
- Can be localized or systemic
- Causes tissue injury & impairs normal organ function
- Stain with CONGO RED -> APPLE GREEN
Nonbranching fibrils of crosslinked beta pleated sheet wound together - morphologically look the same
Normally degraded by proteosome/macrophages; these fail -> deposition, accumulation, aggregation
A-Beta amyloid = Alzheimers
Beta 2 Microglobulin: long term hemodialysis
-> in MHC I - supporting structure that holds up cleft
AL Protein = light chain from plasma cells
AA Protein = depositiion of SAA
- SAA = serum amyloid A protein whose
concentration increases during inflammation
(acute phase reactant)
ATTR Protein = Transthyretin (TTR) = a serum and cerebrospinal fluid carrier of the thyroid hormone thyroxine (T4) and retinol-binding protein bound to retinol.
- Deposited in the heart in senile SA
