Essentials of Human Diseases and Conditions 4 th edition Margaret Schell Frazier Jeanette Wist Drzymkowski

Chapter 3 Immunologic Diseases and Conditions

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 2 Learning Objectives  Name the functional components of the immune system.  Characterize the three major functions of the immune system.  List examples of inappropriate responses of the immune system.  Explain the difference between natural and artificial immunity.

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 3 Learning Objectives (cont’d.)  Trace the formation of T cells and B cells from stem cells.  Explain how T cells and B cells specifically protect the body against disease.  List the five immunoglobulins and explain complement fixation.  Explain the ways that HIV is transmitted.

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 4 Learning Objectives (cont’d.)  List the guidelines for universal precautions and infection control.  Describe the primary absent or inadequate response of the immune system in the following diseases:  Common variable immunodeficiency  Selective immunoglobulin A deficiency  Severe combined immunodeficiency disease

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 5 Learning Objectives (cont’d.)  Explain the destructive mechanisms in autoimmune diseases.  Describe the symptoms and signs of pernicious anemia. Name the primary treatment.  Describe the systemic features of SLE. Recall the diagnostic criteria.  Detail the pathology of rheumatoid arthritis.

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 6 Learning Objectives (cont’d.)  Specify the primary objectives of the treatment for RA.  Compare the pathology of multiple sclerosis to that of myasthenia gravis.  List the distinguishing diagnostic features of ankylosing spondylitis.  Describe the pathology of vasculitis in general terms.

Chapter 3 Lesson 3.1

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 8 Orderly Function of the Immune System  Functional components of the immune system:  lymphoid tissue Primary – thymus, bone marrow Primary – thymus, bone marrow secondary – tonsils, adenoids, spleen, Peyer patches, appendix secondary – tonsils, adenoids, spleen, Peyer patches, appendix  lymphocytes  antibodies  macrophages

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 9 Orderly Function of the Immune System (cont’d.)  Major functions of the immune system:  Protect the body against foreign organisms  Maintain homeostasis by eliminating damaged cells (phagocytosis)  Battles infection

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 10 Orderly Function of the Immune System (cont’d.)  Inappropriate responses of the immune system:  Hyperactive (allergic) – excessive response  Immunodeficient (e.g., AIDS) – inadequate response  Autoimmune (e.g., SLE) – misdirected response  Transplant rejection – attack to beneficial foreign tissues

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 11 Acquired Immunity

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 12 Orderly Function of the Immune System (cont’d.)  Cellular elements of natural immunity:  Natural killer cells (NK)  Polymorphonuclear neutrophils (PMN)  Macrophages  Cellular elements of acquired immunity:  T lymphocyte cells  B lymphocyte cells  Phagocytes

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 13 T cell and B cell Formation

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 14 Orderly Function of the Immune System (cont’d.)  T lymphocyte cells include:  Cytotoxic T cells: destroy virus-infected cells, tumor cells, or allograft cells  Helper T cells: stimulate B cells and activate cytotoxic T cells  Suppressor T cells: moderate the immune response by inhibiting B and T cells  Memory T cells: are reactivated only by re-exposure to previous antigen

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 15 Orderly Function of the Immune System (cont’d.)  B lymphocyte cells include:  Memory B cells: reactivated only by re-exposure to previous antigen  Plasma cells: secrete antibodies that attach to antigens

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 16 Orderly Function of the Immune System (cont’d.)  Immunoglobulins  IgG (75–85%)  IgA (5–15%)  IgM (5–10%)  IgD (0.2%)  IgE (0.5%)

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 17 Orderly Function of the Immune System (cont’d.)  Complement fixation.  Activation of normally inactive proteins found in plasma or body fluids. Pathogens are destroyed by lysis of the cell membrane or by phagocytosis.

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 18 Immunodeficiency Diseases  Increased susceptibility to bacterial infections resulting from a B cell deficiency.  Increased susceptibility to viral, fungal, and protozoan infections, usually from T cell deficiency.

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 19  Acquired Immunodeficiency Syndrome (AIDS)  Caused by human immunodeficiency virus (HIV)  Diagnosed by presence of HIV antibodies in the blood (common test: ELISA; confirmation: Western blot)  HIV attacks helper T lymphocytes  Reduces cell-mediated immunity  Attacks nervous system directly Immunodeficiency Diseases (cont’d.)

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 20  HIV transmission is through direct contact with blood or semen of infected person  sexual contact  blood transfusions and other blood products  contaminated shared needles (IV drug use, etc.)  accidental needle sticks  to fetus via placenta or during birth process from infected mother Immunodeficiency Diseases (cont’d.)

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 21  AIDS treatment  Highly active antiviral therapy (HART)  Many drug combinations can be used  Drug toxicity and side effects exist  Prophylactic antibiotics  Currently no cure Immunodeficiency Diseases (cont’d.)

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 22 Universal Precautions  Thorough and frequent hand washing.  Personal protective equipment worn as required by specific task (e.g. gloves, gown, goggles, mask).  Proper disposal of all sharps in designated biohazard containers.  No needle recap prior to disposal.  Caution in handling of laboratory specimens.  Proper containment and disinfection of blood and body fluid spills.  Use clean mouthpieces and resuscitation bags. Immunodeficiency Diseases (cont’d.)

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 23  Common Variable Immunodeficiency  acquired B cell deficiency  Selective Immunoglobulin A Deficiency  inadequate production of IgA  Severe Combined Immunodeficiency  ineffective development and function of both T cells and B cells Immunodeficiency Diseases (cont’d.)

Chapter 3 Lesson 3.2

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 25 Autoimmune Diseases  Lymphocytes and antibodies are sensitized to develop against the body’s own organs or tissues.  May be triggered by disease, injury, metabolic changes, or mutation in immunologically competent cells.  May be caused by certain drugs or chemicals, trauma, or viral infection.

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 26 Autoimmune Diseases (cont’d.) Pernicious anemia  Impaired absorption of B 12 and B 12 deficiency due to decreased gastric production of hydrochloric acid and shortage of intrinsic factor.  Treatment consists of monthly intramuscular injections of vitamin B 12 during the person’s entire lifetime.

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 27 Autoimmune Diseases (cont’d.) Pernicious Anemia  Symptoms: weakness and tingling and numbness in extremities, altered vision, lightheadedness, tinnitus, palpitations, weight loss, digestive disturbances, sore tongue.  Signs: decreased red blood cell, white blood cell, and platelet counts; increased red blood cell mean volume; decreased hemoglobin levels; jaundice; optic nerve atrophy; decreased amount of gastric acid.

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 28 Autoimmune Diseases (cont’d.) Connective Tissue Diseases  Systemic lupus erythematosus  Scleroderma  Sjogren syndrome  Rheumatoid arthritis  Juvenile rheumatoid arthritis  Ankylosing spondylitis  Polymyositis

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 29 Autoimmune Diseases (cont’d.) Systemic Lupus Erythematosus (SLE)  Inflammation of skin, joints, nervous system, kidneys, lungs, and other organs.  Butterfly rash across face may be present.  Other rashes may appear on other exposed skin areas.

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 30 Autoimmune Diseases (cont’d.)  Butterfly face rash  Alopecia  Photosensitivity  Raynaud’s phenomenon  Thrombocytopenia  Discoid skin lesion  Nasopharyngeal ulceration  Polyarthritis  Pleuritis or pericarditis  Protein or casts in the urine  Hemolytic anemia  False-positive serology  Abnormal blood antibodies  LE cells (leukocytes) present in lab testing Systemic Lupus Erythematosus (SLE)

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 31 Autoimmune Diseases (cont’d.)  Rheumatoid arthritis (RA)  Chronic, inflammation and edema of the synovial membranes surrounding joints.  Destruction of cartilage and adjacent bone.  May produce weight loss, fever, and malaise.  Joint pain and stiffness, especially in morning.  Bilateral joint tenderness, edema, erythema, warmth.

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 32 Autoimmune Diseases (cont’d.) Rheumatoid arthritis treatment aimed at:  Reducing inflammation and pain  Preserving joint function  Preventing joint deformities

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 33 Autoimmune Diseases (cont’d.) Ankylosing spondylitis  Inflammation and ossification of the joints leading to decreased range of motion and spinal bone fusion.  Sacroiliac spine area is usually affected first and results in recurring low back pain and morning stiffness.  History may include inflammatory bowel disease, heel pain, and family incidence of arthritic conditions

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 34 Autoimmune Diseases (cont’d.)  Multiple sclerosis (MS)  Inflammatory disease of the central nervous system.  Demyelination of nerves occurs in the brain and spinal cord.  Signs and symptoms are primarily neurologic and related to areas of myelin destruction.

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 35 Autoimmune Diseases (cont’d.) Myasthenia gravis (MG)  Chronic progressive neuromuscular disorder.  Autoantibodies produced to acetylcholine receptor in muscle cells.  Ineffective nerve-muscle junction results in severe weakness, difficulty talking and swallowing, drooping eyelids and diplopia.

Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc. 36 Autoimmune Diseases (cont’d.)  Vasculitis  Inflammation in the walls of blood vessels that may lead to necrosis, thrombus formation, and local infarct  Can be classified as small vessel or systemic; affecting medium and large arteries