Autoimmune Diseases Flashcards
What might cause tissue damage in autoimmune diseases?
type ll (cytotoxic) and type lll (immune complex)
What can help diagnose autoimmune diseases?
identification of specific antibodies
What are the three basic requirements for pathologic autoimmunity?
- reaction
- primary pathogenic reaction
- no other disease is present
autoimmune diseases might develop from abnormalities in what?
T-lymphocyte system
What environmental factors can lead to loss of tolerance?
viruses, hormones, or drugs
What does Graves disease affect?
antibodies to the TSH receptor act as agonists and elevate thryroid hormones
Anitgens can do what two things?
- Alter function and 2. destroy self-tissues
What are 7 examples of autoimmune diseases?
- antibodies and immune complexes (Graves)
- systemic (systemic lupus erythematosus)
- autoimmunity or reactions to microbes (polyarteritis nodosa)
- T cells (organ specific like multiple sclerosis)
- systemic AD’s like rheumatoid arthritis
- autoimune hemolytic anemia
What is systemic lupus erythematosus?
chronic, autoimmune, multisystem, inflammatory disease.
- more common in females
systemic lupus erythematosus can lead to what 5 things?
- renal failure
- butterfly rash
- focal neurological deficits
- arthritis
- pericarditis or endocarditise
Some manifestations of SLE result from what?
tissue injury due to immune complex mediated vasculitis
SLE affects how many americans?
around 250, 000
Presence of what is a characteristic feature of SLE?
LE bodies
SLE leads to…
Mesangial, focal, or diffuse proliferative glomerulonephritis, or tubular and interstitial changes
What are the pathological characteristic lesions?
wire-loop lesion, hyaline thrombi, and fibrinioid degeneration
What is the clinical presentation of SLE?
- young female
- nonspecific fatigue
- fever
- arthralgia
- weight changes
- butterfly rash, arhtritis, and vasculitis
What are the MSK symptoms of SLE?
- 90 % have polyarthralgia (joint pain)
Whats the most common organ affected by SLE?
kidney
Libman-Sacks endocarditis
noninfectious but may look similar to infectious endocartitis
Discoid lupus
most common variet
- skin involvement only
- no antinuclear antibodies
sub-acute cutaneous lupus
papular and annular lesions (trunk)
Drug-induced lupus
Procainamide, hydralazine, isoniazid
- no sex predisposition and most are over 50 yoa
Rheumatoid arthritis
female: male 4:1
destruction of articular cartilage
Etiology of Rheumatoid arthritis
Genetically predisposed person is exposed to virus or self-Ag which activated T cells specific for a joint antigen which secretes cytokines like TNF alpha
What is the pre-dominant cytokine found in Rheumatoid arthritis?
TNF alpha
persistent tenosynovitis can lead to what?
synovial cysts and ruptured tendons
most common cardiovascular manifestation of RA?
artherosclerosis (leading cause of death in RA)
most common ocular manifestation?
keratoconjunctivitis of Sjogren’s syndrome
can food affect RA?
yes, but not according to the Arthritis foundation
- saturated fats may increase inflammation
Goodpastures syndrome (Anti-globmerluar basement membrane disease)
- attacks lungs and kidneys
- more renal and pulmonary disease
- some just kidneys
Felty’s syndrome
- recurrent bacterial infections and chronic refractory leg ulcers
- splenomegaly and leukopenia
- IgM anti-IgG autoantibodies
What is Sjogren syndrome?
inflammatory disease of the lacrimal and salivary gland
- keratoconjunctivitis (dry eyes)
- xerostomia (dry mouth)
systemic sclerosis
- difficulty swallowing
- dyspnea and chronic caugh
dermatomyositis
- eyelid rash
- proximal muscles
- dysphagia
autoimmune hemolytic anemia
antibody or compliment dependent RBC destruction
Good pasture syndrome
antibodies to basement membranes in lung and kidneys
Myasthenia gravis
anti-Ach receptor antibodies
hashimoto thyroiditis
most common hypothryoidism in nonendemic areas
Graves disease
overactive thyroid
Ulcerative colitis
inflammatory disease
- diarrhea
- bloody stools
ankylosing spondylitis
multisystem inflammatory disorder –> SI joints and axial skeleton
- whites: 92 %
- Blacks: 50 %
what causes ankylosing spondylitis?
the spondyloarthropathies are linked by common genetics… HLA-B27
symptoms of ankylosing spondylitis?
- intermittent back pain
- pain and stiffness (worsens with lack of activity)
- corticosteroids for treatment should be TEMPORARY
treatment of ankylosing spondylitis?
- TNF-alpha blocker
- anti-interleukin -6 inhibitors
- omega-3 fatty acids
immune deficiencies
primary is inhertied and secondary is infection, immunosupression, ect.
- Ig: bacterial
- T-cell: viral/fungal
Primary immunodeficies
- B cell (humoral)
- T cell (cellular)
- defects in phagocytes
- abnormalities in complement
these patients are subject to recurrent bacterial infections
some immunodeficiency disorders are caused by?
discrete molecular defect which causes maturations arrest in lymphocytes
Bruton x-linked Agammaglobulinemia
- pre B cells can’t differentiate–> B cells
- no immunoglobulin
- TREATED with intravenous pooled human Ig
selective IgA deficiency
- often asymptomatic but sometimes have respiratory/gastro intestinal infections
- MOST COMMON of all primary immune deficiencies
common variable immunodeficiency
sever hypo gama globulinemia
- more susceptible to infections
- malabsorption
hyper-IgM syndrome
- x-linked
- can’t make IgG, IgA, or IgE
DiGeorge syndrome
- developmental malformation of 3rd and 4th pharyngeal pouches
- bad thymus and low T cells
- TREATMENT –> thymus transplant
Severe combined immunodeficiency (SCID)
- group of syndromes with humoral and cell mediated immune defects
- varied infections
- TREATMENT - bone marrow transplantation
