Musculoskeletal Flashcards
What is the difference between autoimmunity and self-tolerance?
- Autoimmunity= reactions against self by self antigens.
2. Self-tolerance= Immune system does not react against self antigens.
What is central tolerance
Antigen- induced deletion of self-reactive T and B lymphocytes during maturation in thymus and bone marrow, respectively.
What is peripheral tolerance?
Several mechanisms silence potential auto-reaction:
- Anergy= lack of co-stimulation.
- Suppression by regulatory T cells
- Deletion by apoptosis.
- Some self antigens hidden because they are isolated from blood/lymph
What factors lead to failure of self-tolerance?
Combined effects of susceptibility genes, which influence lymphocyte tolerance, and environmental factors that alter display/reactions against self-antigens.
Define systemic lupus erythematous (SLE)
autoimmune disease involving multiple organs where vast array of auto-antibodies bind to immune complexes that deposit into cells and tissues causing injury.
What are the genetic factors of systemic lupus erythematous (SLE)?
- Familial association
- HLA association
- Other defects in classical pathway of complement, and Fc receptor on B cells
What are the environmental factors of systemic lupus erythematous (SLE)?
- UV radiation
- X chromosome (female>male)
- Drugs (hydralazine, procainamide, D-penicillamine)
Describe the kidney problems in systemic lupus erythematous (SLE).
deposition of immune complex within the glomeruli (Type III hypersensitivity)
Describe the joint problems in systemic lupus erythematous (SLE).
non-erosive synovitis with little deformity.
Describe the skin problems in systemic lupus erythematous (SLE).
butterfly rash, urticaria, bullae, maculopapular lesions, ulcerations
Define sjogren syndrome
Chronic disease characterized by dry eyes, dry mouth, resulting from immunologically mediated destruction of the lacrimal and salivary glands
What is the common autoantibody profile of Sjogren syndrome?
SS-A (more sensitive)
SS-B ( more specific)
What diseases are most commonly associated with Sjogren syndrome?
- Rheumatoid arthritis
- SLE
- Polymyositis
- Scleroderma
- Vasculitis
- Mixed connective tissue disease
- Autoimmune thyroid disease
What are the common clinical features of Sjogren disease?
- More common in women between 50-60
- Keratoconjunctivitis
- Xerostomia
- Parotid gland enlargement
- Dryness of respiratory tract
- Tubulointerstitial nephritis
What are the common characteristics of scleroderma?
- Excessive fibrosis in multiple tissues.
- Obliterative vascular disease.
- Evidence of autoimmunity (mainly autoantibodies)
What is diffuse systemic sclerosis?
initial widespread skin involvement, rapid progression and early visceral involvement.
What is limited systemic sclerosis?
mild skin involvement (often only fingers/face), visceral involvement occurs late, disease generally follows benign course. AKA CREST syndrome: 1. Calcinosis 2. Raynaud's 3. Esophageal dysmotility 4. Sclerodactyly 5. Telangiectasia
Describe bone matrix
Organic compound mixture of osteoid (35%) and a mineral component (65%); bone hardness due to hydroxyapetite
What is the relationship between RANKL and osteoprotegerin (OPG) and bone remodeling?
- Decreased RANKL and increased OPG= no osteoclast activity (bone formation)
- Increased RANKL and decreased OPG= stimulated osteoclast activity (bone resorption)
What is the difference between osteopenia and osteoporosis?
- Osteopenia: decreased bone mass ( 1 to 2.5 standard deviations below the mean peak bone mass)
- Osteoporosis: osteopenia that is severe enough to significantly increase risk of fracture (2.5 or greater standard deviations below mean peak bone mass)
Define Paget disease of bone (osteitis deformans)
Condition of increased, disorderly and structurally unsound bone.
Mainly affects bones of pelvis, skull, spine, and legs.
Susceptible to fractures, increased risk of osteoarthritis and osteosarcoma.