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Immu 101-1 - Autoimmunity
68K - Immu 101-1 - Autoimmunity
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Immunology
Not Applicable
02/10/2012

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Term
Autoimmunity
Definition
exists when the immune system fails to recognize the body's own cells, and begins to attack them as if they were foreign.
Term
Autoimmune Disorders
Definition
are diseases caused by the body producing an inappropriate immune response against it's own tissues?
Term
Autoantibodies
Definition
Antibodies that attack it's own cells, tissues, and/or organs.  This causes inflammation and damage and it leads to autoimmune disorders.
Term
What are the two general types of Autoimmune Disorders?
Definition

Systemic - Those that damage many organs

Localized - Those where only a single organ or tissue is directly damaged by the autoimmune process.

Term
Examples of Systemic Autoimmune Diseases
Definition
  • RA and Juvenile RA
  • lupus
  • Scleroderma
  • Sjögren's Syndrome
  • Goodpasture's Syndrome
  • Wegener's Granulomatosis
  • Polymyalgia Rheumatica
  • Guillain-Barre Syndrome
  • Multiple Sclerosis
Term
Examples of Localized Autoimmune Disease
Definition
  • Type 1 diabetes mellitus (Pancreas islets)
  • Hashimoto's Thyroiditis & Graves Disease (Thyroid)
  • Celiac Disease, Crohn's Disease, & Ulcerative Colitis (GI Tract)
  • Addison's Disease (adrenal)
  • Primary Biliary Cirrhosis, Sclerosing Cholangitis, Autoimmune Hepatitis (Liver)
  • Temporal Arteritis, Giant Cell Arteritis (Arteries of the head and neck)
Term
Autoimmune disease is usually prevented by the normal function of what mechanisms?
Definition
immunologic regulatory mechanisms
Term
What can happen when the immunologic regulatory mechanisms malfunction?
Definition
Antibodies to self antigens may be produced, and bind to antigens in the circulation to form circulating immune complexes, or bind to antigens deposited in specific tissue sites.
Term
What are some causes of autoimmune responses?
Definition
  • Antigens that do not normally circulate in the blood
  • Altered antigens
  • A foreign antigen that is shared or cross-reactive with self-antigens or tissue components.
  • Mutation of immunocompetent cells (WBCs) to acquire a response to self-antigens
  • Loss of the immunoregulatory function by T-cell subsets
Term
Rheumatoid Arthritis
Definition
is a progressive inflammatory disease of the joints, the disease can be very mild or severe.
Term
Destructive Polyarthritis
Definition
Severe cases of Rheumatoid Arthritis can manifest as a destructive polyarthritis where the hands and feet are severely deformed.
Term
What are the serological findings of RA?
Definition

Low serum iron and low or normal iron-binding capacity, mild to moderate decrease in serum albumin, durring the accute and chronic phases the following are usually elevated;

  • Erythrocyte sedimentation rate
  • Gamma globulins (commonly IgM & IgG)
  • Platelet count roughly in proportion to the severity of overall joint inflammation.
Term
Rheumatoid Factor
Definition
An IgM antibody that reacts against a patient's own IgG antibodies, demonstrable in 70-80% of patients with RA, not a cause of RA but produced during the course of the disease, high titers associated with severe rheumatoid disease.
Term
Antinuclear Antibodies (RA)
Definition
Demostrable in 20% of RA patients, titers lower than in SLE (systemic lupus erythematosus)
Term
Complement levels in RA patients
Definition

Usually normal except in those with vasculitis, depressed hymolytic complement levels:

  • Found in less than 1/3 patients 
  • Usually found in patients with very high levels of rheumatoid factor (RF) and immune complexes
Term
Felty's Syndrome
Definition
  • Association of RA with splenomegaly and leukopenia
  • Almost always develops in patients with a high titer rheumatoid factor assay, a positive antinuclear antibodies (ANA) assay, and rheumatoid nodules
  • Joint fluid examination is valuable, reflecting abnormalities that are correlated with varying degrees of inflammation.
Term
What are the two most common autoimmune diseases that affect the thyroid?
Definition

Hashimoto's thyroiditis

Grave's disease

Term
Hashimoto's Thyroiditis
Definition
  • Thyroiditis - Inflammation of the thyroid gland
  • Hypothyroidism - Underactive thyroid
  • Large and rubbery thyroid gland (goiter)
  • Symptoms of hypothyroidism:
    • Bear
Term
Grave's Disease
Definition
  • Manifests by overproduction of thyroid hormones
  • Goiter that is soft instead of rubbery
  • Leads to hyperthyroidism (overactive thyroid)
  • Symptoms of Graves Disease:
    • Raver
Term
Important proteins, enzyme, and hormones of the thyroid include:
Definition
  • Thyroglobulin
  • Thyroid peroxidase (TPO)
  • Thyroid stimulating hormone (TSH)
  • Triiodothyronine (T3) Thyroxine hormones(T4)
Term

(Hashimoto's Thryroiditis)

Hormone test

Definition
  • Slight increase in the plasma TSH concentration (early phase)
  • Fall of thyroid hormones T4 and serum T3 (later phases)
Term
Hashimoto's Thyroiditis - Antibody test
Definition

The following autoantibodies found in moderate to high titers in 50% of patients.

  • Antithyroglobulin
  • Antimicrosomal antibodies (considered more diagnostic)
  • More than 90% have anti-TPO (autoantibody to TPO)
Term
Grave's Disease - Hormone test
Definition

Decreased levels of TSH, Elevated levels of hormones T3 and T4.

Increased uptake of radioactive iodine (confirmatory test)

Anti-TPO antibodies found in 50% of patients.

Term
SLE - Systemic Lupus Erythematosus
Definition

Classic model of autoimmune disease

Chronic systematic inflammatory disease

1 in 2000 (trippled over last 40 yrs)

Mostly affects women

Onset usually between 20-40 years

Usually follows a chronic and irregular course with periods of exacerbations and remissions.

Term
What is the most common cause of death in patients with SLE?
Definition
Renal failure and infectious complications.
Term
What are the early signs of SLE?
Definition
Fatigue, weight loss, malaise fever, and loss of appetite.
Term
In SLE patients, what happens to the joints?
Definition
90% of patients have joint pain or arthritis.
Term
In SLE patients, some patients develop a butterfly rash, what percentage of patients get this rash and where is it located?
Definition
Butterfly rash is a rash across the nose and cheeks, and is seen in more than 40% of patients.
Term
Patients with SLE have an increased risk of developing __________ and _________ _________.
Definition
common and opportunistic infections.
Term
SLE can involve the following systems;
Definition

Renal

Cardiovascular

Gastrointestinal

Nervous

Musculoskeletel

Term
What is the hallmark autoantibody of systemic lupus erythematosus (SLE)?
Definition
Antinuclear Antibodies (ANA)
Term
Antinuclear Antibodies
Definition
(True autoantibodies) Heterogeneous group of immunoglobulins (IgM, IgG, and IgA) that react with whole nucleus or nuclear components such as DNA and nuclear proteins.  Found in other diseases such as RA.
Term
What are the potential blood test findings associated with SLE?
Definition

Moderate anemia

Lymphocytopenia

Thrombocytopenia

Term
What are the serological manifestations associated with SLE?
Definition

High levels of ANAs

Immune Complexes

Complement level depression

Tissue deposition of Igs and complement

Other autoantibodies.

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