Patho Chapter 10

Primary

(congenital- tied to diff. chromosomes) congenital immune system dysfuntion [there are tons of disorders table 10-7] dont have to memorize

(EVOLVE QUESTIONS)

**************************

**********************************************

*********************************************

what is dysfunctional in autoimmunity?

- Thymus dysfunction (T cell theory) - B cell theory -Mast cell

What happens when you have too much immune function going on?

-Autoimmunity or -Hypersensitivity

[environmental triggers] ___________ virus and __________ bacteria have been found in cases to be contributors/ triggers of autoimmune disorders

-Epstein-Barr virus -H. pylori bacteria (keep in mind they are not sure why this happens)

Which immunodeficiency disorder is a primary immunodeficiency disorder? -Immunodeficiency associated with cancer chemotherapy -Immunodeficiency associated with DiGeorge syndrome - Immunodeficiency associated with corticosteroid use - Immunodeficiency associated with malnutrition

-Immunodeficiency associated with DiGeorge syndrome

Type I - most deadly (Atopic, Anaphylactic) 15-30 min ***antihistamine is what we want to administer!**** -might be administering epinephrine

-Mediated by: IgE (antibody E) -Histamine is key mediator -Immune response: Ag plus IgE = mast cell degranulation -Cause of reaction: T-cell deficiency, abnormal mediator feedback, environmental factors and antigens -Manifestations: Asthma, rhinitis, atopic eczema, bee sting

Type III 6 hr

-Mediated by: IgG -antigen-antibody complexes that dysfunction - Causes of reaction: Persistent infection (microbe antigen), extrinsic environmental antigens, autoimmunity-self antigens. -body does not effectively clear antibody/antigen complexes, so they accumulate -Manifestations: SLE,

[CH.10 study guide questions] STATE THE PATHOGENIC MECHANISM(S) WITH THE DISORDER..

....

Immunodeficiency disorders can be categorized as primary or secondary. Categorize each of the following disorders as primary (P) or secondary (S).

....................................

Framework of pathophysiology

1) Etiology 2) pathogenesis 3) clinical manifestations 4) treatment implications

Type II - deadly (but rare) (Cytotoxic, cytolytic) -tissue specific 15-30 min

Antibodies attack antigens on surface of cells or tissues -Mediated by: IgG and IgM -example: blood transfusion reactions = wrong blood compatibility-could also have a delayed reaction to the blood or cleansing agent relating to the blood. -Complement activation - immune response: Ag and Ab leading to killer cell cytotoxic action or complement-mediated lysis -system starts shutting down -Causes of reaction:Exposure to antigen or foreign tissue, cells, or graft -Manifestations: ABO transfusions,hemolytic disease of newborn, myasthenia gravis, thyroiditis, hyperacute graft rejection, and autoimmune hemolytic anemia.

What is the pathophysiologic abnormality underlying systemic lupus erythematosus (SLE)?

Autoimmunity

Aging affects the immune system in all of the following ways except A. reduced T-cell function. B. hyper-reactivity to new antigens. C. decrease in antibody production. D. diminished T-cell proliferation.

B. hyper-reactivity to new antigens

Type II hypersensitivity *****know this

Blood transfusion reaction- deadly! emergency! get help. STOP THE BLOOD TRANSFUSION. keep fluids running - normal saline don't want it to clot up

Mast cell theory [Mast cells reside in most mucous membranes waiting for foreign proteins or bacteria to invade.]

Causing hypersensitivity - allergies..allergic reaction to self. Histamine is a huge mediator of this component example: -rheumotoid arthritis -irritable bowel

5 antibodies G, A, M, E, D "IgG, IgA, IgM, IgE, IgD"

G is most predominant [look at page 186]

How are hypersensitivity disorders detected, prevented, and treated?

Hypersensitivity disorders are detected based on presenting symptoms and organ involvement, which can range from an acute systemic allergic reaction with anaphylaxis, to a more localized response, such as with immune complex glomerulonephritis, causing hematuria, proteinuria, oliguria, and red cell casts in the urine. Since hypersensitivity reactions are specific to a particular antigen, these disorders are prevented by avoiding the antigen if identified (if possible) or desensitization therapy. Treatment depends on the type of hypersensitivity reaction but may include immunosuppressive agents, such as corticosteroids or cytotoxins, or plasmapheresis

Hemolytic disease of the newborn (erythroblastosis fetalis) is an example of a type______ hypersensitivity reaction between the mother's Rh-positive__________ and subsequent fetal Rh-positive red blood cells.

II ; ANTIBODIES

Most autoimmune disorders are mediated through type _________or____________ hypersensitivity mechanisms.

II, OR III

Environmental triggers for autoimmune diseases include___________ , and occupational or environmental______________

INFECTION; STRESS

Which type of hypersensitivity response is mediated by T cells?

IV

The health history of a 6-month-old breastfed child reveals serious, recurrent bacterial and fungal infections. The most likely cause of these infections is

Immune deficiency

Which immunodeficiency disorder is a primary immunodeficiency disorder?

Immunodeficiency associated with DiGeorge syndrome In this disorder, the aplastic or hypoplastic thymus is unable to assist in the maturation of T cells. Therefore T cells are deficient. B cells are normal.

A 39-year-old patient who has been taking a specific antibiotic for years without problems develops tachycardia, lowered blood pressure, wheezing, and urticaria when given this antibiotic in the clinic. The most likely explanation for this occurrence is that the patient

Is experiencing an anaphylactic hypersensitivity reaction to the antibiotic

[genetic factors] MHC- or HLA

Major histocompatibility complex - or Human leukocyte antigen: located on chromosome 6p21 often assoc. with certain autoimmune disorders.

Type IV 24-48 hr

Mediated by T cells delayed reaction - contact dermatitis It is an immune or inflammatory response to a wide variety of plant oils, chemicals, ointments, clothing, cosmetics, dyes, and adhesives. Contact hypersensitivity is an epidermal phenomenon.

What are the common features of autoimmune disorders and certain types of hypersensitivity disorders?

Most autoimmune reactions toward self tissues are mediated through type II (cytotoxic) and type III (immune-complex) hypersensitivity mechanisms

Secondary

Neuro-endocrine-immune -recall the effects of stress can happen by: illness, surgery, pregnancy, medications, nutritional status, rest, decreased functional reserve (aging).

HYPERSENSITIVITY [Too much!] (normal pathways but overstimulated)

Normal immune response that is inappropriately triggered or excessive or produces undesirable effects on the body.

___________is a therapy for some autoimmune diseases where________________ are removed and replaced with colloid solutions.

PLASMAPHERESIS; ANTIBODIES

DiGEORGE SYNDROME

PRIMARY

HIV/AIDS

PRIMARY

SELECTIVE IgA DEFICIENCY

PRIMARY

SEVERE COMBINED IMMUNODEFICIENCY

PRIMARY

How do the etiologic processes of primary and secondary immune deficiency disorders differ?

Primary deficiencies are congenital, genetic, or acquired defects that directly affect immune cell function. (these may be congenital or acquired.) Secondary deficiencies are conditions that impair immune function as a result of other nonimmune system disorders.are a consequence of other processes or treatments in the body. Examples of secondary disorders include those associated with hyperlipidemia or malnutrition, medical treatments such as cancer chemotherapy, or biopsychosocial stress such as postsurgical immune system problems.

CHEMOTHERAPY

SECONDARY

MALNUTRITION

SECONDARY

Type IV is .... (more delayed reaction in body)

T cell - cell mediated

ALLERGIC RHINITIS

TYPE I

ASTHMA

TYPE I HYPERSENSITIVITY

HEMOLYTIC DISEASE OF NEWBORN

TYPE II

GRAVES DISEASE

TYPE II AND AUTOIMMUNE

MYSATHENIA GRAVIS

TYPE II AND AUTOIMMUNE

TYPE 1 DIABETES MELLITUS

TYPE II AND AUTOIMMUNE

Poststreptococcal glomerulonephritis

TYPE III

RHEUMATOID ARTHRITIS

TYPE III AND AUTOIMMUNE

SLS

TYPE III AND AUTOIMMUNE

CONTACT DERMITITIS

TYPE IV

IMMUNE DEFICIENCY [Too little!]

There's primary and secondary *she wants us to focus on AIDS*

Type I hypersensitivity ****know this

Treatment of type I: epinephrine, corticosteroids -the goal is to keep the airway open through reducing swelling and constriction

The antibody type IgE is involved in which type of hypersensitivity reaction?

Type II (cytotoxic) reaction

Type III hypersensitivity

Type III hypersensitivity reactions occur when antigen-antibody complexes are deposited in tissues and result in the activation of complement and subsequent tissue inflammation and destruction. Antigen-antibody complexes activate the complement cascade and subsequently attract phagocytic cells to the tissue.

Autoimmune disorders tend to be found more in

WOMEN

AUTOIMMUNITY [Too much!]

When the immune system attacks its own tissues "friendly fire" "ITS OFTEN CALLED IDIOPATHIC"

Antibodies are the mediators of all of the hypersensitivity reactions except A. the type I (anaphylactic) reaction. B. the type II (cytotoxic) reaction. C. the type III (immune complex) reaction. D. the type IV (delayed) reaction.

[D] the type IV (delayed) reactio

Type I hypersensitivity

an immediate allergic or anaphylactic type of reaction mediated primarily by sensitized mast cells. Mast cell degranulation releases chemicals that mediate the signs and symptoms of anaphylaxis, including vascular permeability, vasodilation, hypotension, urticaria, and bronchoconstriction.

Types I, II, III are ... (more immediate reaction in body) [table 10-2]

antibody B cell - humoral humoral= (aspect of immunity that is mediated by macromolecules (as opposed to cells) found in extracellular fluids such as secreted antibodies, and complement proteins.

Secondary deficiencies

are conditions that impair immune function as a result of other non-immune system disorders, such as poor nutrition, pregnancy, stress, or drugs, that secondarily suppress immune function.

Severe combined immunodeficiency disorders

are usually due to autosomal recessive anomalies.

[environmental triggers] stress

can wear down your immune system

Pharmacotherapies (immunosuppressive therapy is common for autoimmune disease)

have to do with how we treat these things. back to illistration figure 9-42- is the disease's mechanism about cytokines, histamines, capillary permiability, ect. other processes from that picture [due to mechanism of disease therapies are chosen] -the immunosuppressive treatment for each type of autoimmune disease is individualized depending on disease expression *drugs with corticosteroids and certain chemotherapeutic agents to inhibit excessive or abnormal immune responses. *Antiinflammatories, steroids, immunosuppresents, cytotoxins

Primary deficiencies

in immune function may be from congenital, genetic, or acquired defects that directly affect immune cell function.

Alterations in immune function may be related to nutritional deficits or excesses. An example of this is

insufficient calorie and protein intake results in decreased numbers and function of T cells.

Severe combined immunodeficiency is a disorder of

lymphocyte stem cell failure.

Certain genotypes have been shown to be associated with a higher risk of developing autoimmune diseases. These genes are of the class

major histocompatibility complex (MHC) genes

Type II hypersensitivity

occurs when antibodies are formed against antigens on cell surfaces, usually resulting in lysis of target cells. Cell lysis may be mediated by activated complement fragments or by phagocytic cells that are attracted to target cells by the attached antibodies.

Chronic stress may cause secondary immunosuppression due to

overproduction of cortisol.

Type IV hypersensitivity

reactions are T-cell-mediated and do not require antibody production. Sensitized T cells react with altered or foreign cells and initiate inflammation

Mast cells release the cytokine interleukin-1 (IL-1), attracting and enlisting inflammation-inducing cells to joints and leaking fluid into joints in

rheumotoid arthritis

The heart pumps the blood. what pumps the lymph?

skeletal muscle - moving your body! immune disorders happen with stagnant lymph - so move yo ass!

The antigenic mimicry theory proposes that

small alterations in self tissue may lead to immunogenic attack; self antigens that do not come in direct contact with lymphocytes during fetal development

Chronic VS not being chronic

something thats been going on for at least 6 months = chronic

Thymus Dysfunction (T cell theory)

thymus is supposed to help recognize friendly cells. when thymus has dysfunction you start to recognize yourself as enemy: Examples of diseases assoc. with T cell theory of dysfunction: -Systemic Lupus Erythematosus (SLE): dysfunction of the antigen-antibody complex - antigen isn't thoroughly eliminated- not phagocytosed or eaten and hangs around in body. Plasmaphoresis is used for patients with SLE. (like dialysis - cleansing of the blood) -Diabetes mellitus type I: body targets its own pancreatic cells as enemys = absolute loss of insulin

Tissue injury associated with autoimmune disease is mediated through

type II and III hypersensitivity responses

Graft-versus-host disease is an example of a

type III (immune complex) reaction.

B cell Theory [B cells are involved with: -humoral -antibodies -antigens - where antibodies attack]

when B cells create self-antibodies - not recognizing its own histocombalibility components