Chapter 15: Autoimmunity Flashcards
Autoimmune Diseases
○ Humoral and cell-mediated immune responses directed toward self antigens
○ Cause tissue and organ damage
○ Systemic or organ-specific
self-tolerance
Ability of the immune system to accept self-antigens and not initiate an immune response against them
Systemic Lupus Erythematosus (SLE)
○ A chronic systemic inflammatory disease that affects multiple organ systems
○ Patients develop numerous autoantibodies
○ Immune complexes form, triggering C’ activation, chemotaxis of neutrophils, and inflammation
Clinical Symptoms of SLE
○ Joint involvement
○ Skin rashes (butterfly rash)
○ Renal involvement
○ Neurologic symptoms
○ Anemia, leukopenia, thrombocytopenia
Laboratory Tests to Diagnose SLE
○ CBC
○ Urinalysis
○ CRP/ESR
○ Complement quantitation
○ ANAs
Antinuclear Antibodies (ANAs)
○ Directed against antigens in cell nuclei
○ Present in more than 95% of lupus patients
ENA Ouchterlony Test
Immunodiffusion test detects antibodies to specific ENA
Phospholipid Antibodies
○ Found in approximately 60% of lupus patients
○ Can cause false-positive results in nontreponemal tests for syphilis
○ the lupus anticoagulant (produces prolonged APTT and PT)
Rheumatoid Arthritis (RA)
Chronic arthritis of the peripheral joints that can progress to joint deformity and disability
Some patients with RA can also develop
- Subcutaneous nodules
- Pericarditis
- Interstitial lung disease
- Vasculitis
Pathology of RA
○ Inflammation destroys the bone and cartilage
○ TNF-a plays a key role in the process
Overly active osteoclasts absorb the bone
Treatment of RA
○ NSAIDs
○ Disease-modifying anti-rheumatic drugs (DMARDs)
○ Biologic agents that target TNF-a
Laboratory Testing for RA
○ Rheumatoid factor- Autoantibody (usually IgM) that reacts with Fc portion of IgG
○ Anti-CCP- Autoantibody directed against cyclic citrullinated peptide
○ ANAs
○ ESR, CRP, C’
Of the tests for RA which is specific for RA and which isn’t?
Anti-CCP is specific, Rheumatoid factor is not
Granulomatosis with Polyangiitis
○ Aka: Wegner’s granulomatosis
○ Rare autoimmune disease involving inflammation of small- to medium-sized blood vessels and respiratory tract
Anti-Neutrophil Cytoplasmic Antibodies (ANCA)
○ Produced against proteins in neutrophil granules
○ Strongly associated with syndromes involving vascular inflammation
Hashimoto’s Thyroiditis
○ Immune destruction of the thyroid gland produces hypothyroidism
○ Symptoms include fatigue, dry skin, weight gain, brittle hair, formation of a goiter
Characterized by Normal levels of TSH and low Levels of FT4
Graves Disease
○ An AITDS characterized by hyperthyroidism
○ Symptoms: nervousness, weight loss, rapid heart beat, goiter, exophthalmos
○ Low TSH and high FT4
Type I Diabetes Mellitus (T1D)
○ Endocrine disorder characterized by hyperglycemia
Type 1 destruction of b cells in pancreas results in insulin deficiency
Celiac Disease
○ Affects small intestine and other organs
○ Triggered by gluten
Autoimmune Liver Diseases
Immune-mediated liver diseases that can lead to end stage liver failure if untreated
Autoimmune hepatitis (AIH)
○ Hepatocytes targeted
○ AIH-1- Positive for SMA, ANAs
○ AIH-2- Positive for LKM-1 or LC-1 antibodies
Primary biliary cirrhosis (PBC)
○ Destruction of intrahepatic bile ducts; cholestasis
○ Majority of patients produce mitochondrial Abs (AMAs)
Multiple Sclerosis (MS)
involves inflammation and destruction of the central nervous system
Myasthenia Gravis
Affects neuromuscular junction, resulting in weak skeletal muscles
Goodpasture’s Syndrome
Patients produce autoantibodies to basement membranes lining the renal glomeruli and lung alveoli
