Module 9 - immunodeficiency and Autoimmune Disorders Flashcards
Autoimmune disorders occur when
the body systems cannot distinguish between self and non-self antigens, causing the body to attack its own tissues
Autoantibodies
antibodies that react with self-antigens
Organ-specific disease attack specific targets(organs/tissues) when
they become intolerant due to overexposure of antigens
-Autoantibodies in the targeted organ/tissue bind to self-antigens, leading to cellular destruction, overstimulation of cells, or cellular suppression.
What is rheumatic fever an example of?
heart (organ) - specific autoimmune disease
What is multiple sclerosis and Guillain-Barre’ syndrome an example of?
CNS(organ)-specific autoimmune disease
what is myasthenia gravis an example of?
Muscle (organ)-specific autoimmune disease
what is Addison’s and Grave’s disease and type 1 diabetes mellitus an example of?
endocrine (organ)-specific autoimmune disease
What is celiac and Crohn’s disease as well as other inflammatory bowel diseases an example of?
GI (organ)-specific autoimmune disease
What is glomerulonephritis and goodpasture syndrome an example of?
kidney (organ)-specific autoimmune disease
Systemic disease
autoimmune responses directed at self-antigens of many organs and tissues
What happens to tissues when a systemic disease is present
Systemic inflammation occurs with widespread tissue damage including the joints, kidneys, and heart.
What happens antibody reactions occur in the presence of systemic diseases
Autoantibodies interact with other cells such as RBCs and platelets resulting in apoptosis and thrombocytopenia as well as other bleeding disorders.
What is rheumatoid arthritis an example of?
Systemic disease
what is scleroderma an example of?
Systemic disease
what is systemic lupus erythematosus an example of
Systemic disease
Systemic Lupus Erythematosus
a chronic, multisystem inflammatory disease that damages multiple organs making diagnosis and treatment difficult
How is the musculoskeletal (joints and bone marrow) affected by Systemic Lupus
Polyarthritis, with swollen, painful joints, without damage; arthralgia
Anemia, leukopenia, thrombocytopenia
How is the integumentary (skin) affected by Systemic Lupus
Butterfly rash with erythema on cheeks and over nose
Rash on body
Hair loss
How is urinary (kidneys) affected by Systemic Lupus
Glomerulonephritis with antigen: antibody deposit in glomerulus, causing inflammation, marked proteinuria, and progressive renal damage
How is respiratory (lungs) affected by Systemic Lupus
Pleurisy: inflammation of the pleural membranes, causing chest pain
How is cardiovascular (heart and blood vessels) affected by Systemic Lupus
Carditis: inflammation of any layer of the heart
Most common: pericarditis
Raynaud’s phenomenon: periodic vasospasm in fingers and toes often accompanied by pain and tingling
How is gastrointestinal (oral cavity) affected by Systemic Lupus
Ulcerations in oral mucosa
How is the central nervous system (nerves, eyes) affected by Systemic Lupus
Psychosis, depression, mood changes, seizures
Photosensitivity: exacerbation with sun exposure
Immunodeficiency
The body’s inability to fight infection and other foreign invaders
immunodeficiency may be…
acute or chronic
immunodeficiency could be caused by
B-cell and T-cell production deficits
Primary immunodeficiency
Basic developmental failure somewhere in the system (i.e. bone marrow production of stem cells), thymus, or the synthesis of antibodies
Typically seen at birth (congenital) and typically hereditary (genetic)
Caused by mutations, even in one gene
Classified by the part of the body affected
what is ataxia - telangiectasia an example of?
Primary immunodeficiency
what is Inherited X-linked hypogammaglobulinemia (low antibody levels because of a B-cell defect) an example of?
Primary immunodeficiency
what is DiGeorge syndrome (hypoplasia of the thymus), developmental defect an example of?
primary immunodeficiency
What is Wiskott-Aldrich syndrome an example of?
primary immunodeficiency
Secondary/Acquired immunodeficiency
The loss of immune response due to a specific cause
Typically viral infections, splenectomy, malnutrition or liver disease, use of immunosuppressive drugs with organ transplants, and radiation and chemotherapy
Can occur at any point of someone’s lifespan
What happens in bone marrow that can cause secondary immunodeficiency?
Depressed bone marrow production of leukocytes - often due to malnutrition and blood loss associated with cancer and the effect of treatments
How does glucocorticoid drugs contribute to secondary immunodeficiency
Use of glucocorticoid drugs cause decreased leukocyte production as well as atrophy of lymph nodes and suppression of immune response
How does sever physical or emotional stress contribute to secondary immunodeficiency
Severe physical or emotional stress may cause a temporary immunodeficiency response and is attributed to high levels of glucocorticoid secretion in the body
What population is most affected by secondary immunodeficiency
older adults and people who are hospitalized
What is aplastic anemia an example of?
Secondary/Acquired immunodeficiency
What is sickle cell anemia an example of?
Secondary/Acquired immunodeficiency
What is leukemia an example of?
Secondary/Acquired immunodeficiency
What is epstein-barr virus an example of?
Secondary/Acquired immunodeficiency
What is cytomegalovirus an example of?
Secondary/Acquired immunodeficiency
What is downs syndrome an example of?
Secondary/Acquired immunodeficiency
What is hepatitis an example of?
Secondary/Acquired immunodeficiency
What is hodgkin’s lymphoma an example of?
Secondary/Acquired immunodeficiency
What are the examples of primary immunodeficiency and secondary immunodeficiency when humoral B cells are affected
(primary) Hypogammaglobulinemia (congenital)
(secondary) Epstein-Barre
What are the examples of primary immunodeficiency and secondary immunodeficiency when Cellular (cell-mediated) T cells are affected
(Primary) DiGeorge syndrome
(Secondary)AIDS, HIV infection, temporary with some viruses and disorders
What are the examples of primary immunodeficiency and secondary immunodeficiency when B&T cells are affected
(Primary)Either inherited combined immunodeficiency syndromes
(Secondary) Radiation, immunosuppressive drugs and cytotoxic drugs
What are the examples of primary immunodeficiency and secondary immunodeficiency when Phagocytes are affected
(Primary) Inherited chronic granulomatous diseases (CGDs)
(Secondary) Immunosuppression (glucocorticoid drugs, neutropenia); diabetes (decreased chemotaxis)
What are the examples of primary immunodeficiency and secondary immunodeficiency when Complement system (C1, C2, C4) are affected
(Primary) Inherited deficit of one or more systems
(Secondary) malnutrition (decreased synthesis), components liver disease—cirrhosis
Effects of Immunodeficiency
Patients become predisposed to the development of opportunistic infections caused by normal flora, normally harmless microorganisms
-May involved multiple organisms and be quite severe and can be difficult to treat successfully
Prophylactic drugs
Preventive antibiotics given to patients with immunodeficiency before an invasive procedure to prevent infection
Development of Acquired Immunodeficiency Syndrome (AIDS)
AIDS is a chronic disease caused by diseased and disorders that destroy helper T cells (CD4+ T cells) resulting in loss of immunity creating an environment that leaves the host susceptible to secondary infections
Aids is an acquired syndrome
Initial Stage of AIDS
Virus, bacteria, fungal infection, or autoimmune disorder attacks the body causing destruction of CD4+ T cells.
Initial stage may be asymptomatic (latent).
Some forms, such as HIV, may present flu like symptoms.
Active (symptomatic) infection of AIDS
Generalized lymphadenopathy due to infection occurs.
Other symptoms may appear related to cause.
Opportunistic infections and neoplasms begin to appear causing AIDS-related complex (ARC) which is regarded as a prodromal stage (precursor).
T lymphocytes continue to be destroyed.
AIDS diagnosis
CD4+ count drops below 200/mcl.
Life-threatening comorbid disorders begin to develop.
Unusual tumors may begin to appear.
Sexual transmission of HIV
Unprotected sexual contact with HIV-infected partner is the most common mode of HIV transmission
Includes contact with semen, vaginal secretions, and/or blood
Risk is greater for partner receiving semen
Sexual Activities causing trauma to local tissue increases the risk as do genital lesions from other sexually transmitted infections
Contact with blood and blood products transmission of HIV
When sharing drug-using paraphernalia (e.g., needles syringes, straws)
Puncture wounds - risk increases with the depth of the wound, a needle with a hollow bore and visible blood, or a device used for venous or arterial access
There is still a risk, although lower, when exposed to splashes of blood on skin with open lesions
Routine screening of blood donors and testing donated blood for the presence of HIV have improved the safety of the blood supply
Perinatal Transmission of HIV
An HIV infected mouth can transmit infection to her infant during pregnancy, delivery, or breastfeeding
Transmission can be reduced when women are routinely tested for HIV and treated with antiretroviral therapy (ART) (a combination of medication used to control and suppress HIV replication)
Asymptomatic infection in chronic HIV infection
Can be up to 10 years
Without symptoms, people may continue risky behaviors and spread HIV
Diagnosis and Diagnostic Criteria for Acquired Immunodeficiency Syndrome based on wasting syndrome
Defined as a loss of 10% or more of ideal body mass (the measure of fat based on height and weight)
Includes:
Weight loss
Muscle atrophy
Anorexia
Fatigue
Decrease in strength
