OVERVIEW OF THE IMMUNE SYSTEM

IMMUNE SYSTEM FUNCTIONS:

DEFENSE
HOMEOSTASIS

ORGANS / TISSUES

BONE MARROW AND CELLS OF THE IMMUNE

HEMATOPOETIC SYSTEM SYSTEM

THYMUS WBC

SPLEEN Granulocytes Agranulocytes

LYMPH NODES EOSINOPHILS

TONSILS / ADENOIDS/ NEUTROPHILS

MUCOID LYMPHATIC
TISSUES
BASOPHILS

LYMPHOCYTES MONOCYTES

MACROPHAGES
B CELLS T CELLS NATURAL
KILLER CELLS
DENDRITIC CELLS
IMMUNOGLOBULINS Helper T cells

Cytotoxic T cells
IgG Gamma delta T cells
IgA Suppressor T cells
IgM

IgE

IgD

IMMUNITY: the ability to resist damage from foreign substances, such as microorganisms, and harmful chemicals,
such as toxins released by microorganisms.
*** Immunity is categorized as:
I. innate immunity (non-specific inflammatory response) and
II. adaptive immunity (specific immunity).

I. Non-Specific Inflammatory Response: (innate immunity)

The body recognizes and destroys certain foreign substances, but the response to them is the same each
time the body is exposed to them.

Mechanical Mechanisms:
1. The skin and mucous membranes are barriers that prevent the entry of microorganisms into the body.
2. Tears, saliva and urine act to wash away microorganisms.

Chemical Mediators:
1. Chemical mediators kill microorganisms, promote phagocytosis, and increase inflammation.
2. Lysozyme in tears and complement in plasma are examples of chemicals involves in innate immunity.
3. Interferons prevent the replication of viruses.
 Cells
1. Chemotaxis is the ability of cells to move toward microorganisms or sites of tissue damage.
2. Neutrophils are the first phagocytic cells to response to microorganisms.
3. Macrophages are large phagocytic cells that are active in the latter part of microorganisms into tissues.
4. Basophils and mast cells promote inflammation, whereas eosinophils inhibit inflammation.
5. Natural killer cells lyse tumor cells and virus-infected cells.

Inflammatory Response - occurs whenever cells have been injured such as those that occur from trauma,
oxygen or nutrient deprivation, chemical agents, microorganism invasion, temperature extremes, or ionizing
radiation.
- Also occurs when dead cells are present
- Begins at the moment of injury and may continue for 4 to 6 days, depending on the extent of injury
- The inflammatory response is so necessary to healing that is commonly said “no inflammation, no
healing”
- Purpose of inflammation: to limit the effects of harmful bacteria or injury by destroying or neutralizing
the organism and by limiting its spread throughout the body

Walling-Off Effect
- Occurs in the damaged area to prevent the spread of injurious agents to other body tissues.
o Fibrinogen Clots block the lymphatic channels and spaces in the tissues so that fluid barely
flows in the area.

White Blood Cell Activity

- During the inflammation phase, the white blood cells become active to clean up the wound and
initiate further healing processes.
o Neutrophils
 vital defense organisms because they are both the first and the most numerous cell
types to arrive at any area of disease or injury.
 Phagocytose bacteria, dead cells, and cellular debris
 Short-lived but they are effective in clearing wound debris if bacteria are not
excessive
 Sometimes called polymorphonuclear neutrophils (PMNs), or polys, because of their
irregularly shaped nuclei
 Compose about 60% of the circulating WBCs
 Segs – mature neutrophils; the presence of increase in segmented WBCs indicates
a bacterial invasion
 Bands – immature cells; the presence of increased band neutrophils indicates more
severe infection because the bone marrow has released immature cells.
 Pavementing or Marginating– the process wherein the slowed flow of blood
allows the neutrophil to leave the center of the bloodstream and line the
walls of the capillaries. (the cells line up like bricks on a sidewalk)
 Diapedesis- the movement or passage of blood cells, through intact capillary
walls into surrounding body tissues. The neutrophils which are normally too
large to squeeze through the lining, can pass through the capillary wall and
enter the site of tissue injury to begin phagocytosis.

Mediators of the Inflammation Phase

Mast Cells
o when the mast cell is stimulated, it releases histamine and serotonin, which cause capillary
dilation.
o Mast cells can be stimulated by many factors such as physical injury (e.g. wounds, burns,
xray exposure), chemical injury (e.g. toxins, snake, bee venom), or immunologic means (e.g.
hypersensitivity reactions seen in allergies)
o Mast cells also synthesize leukotrienes and prostaglandins. These two chemicals cause the
same responses as histamine, but the response lasts longer. Prostaglandins also cause pain
and tend to appear in the later stages of inflammation.

Kinins
o causes increased vascular permeability, fluid in the wound, and the number of leukocytes
available to assist phagocytosis.
o The primary kinin is bradykinin
o Early stage – increase vascular permeability and allow the leukocytes to enter the tissues
o Later stage – act with prostaglandin to cause pain and smooth muscle contraction and to
increase leukocyte chemotaxis

Cytokines
o regulate the mobility, differentiation, and growth of leukocytes
o among the best understood cytokines are interleukins and interferons
o Interleukins – promote the growth and function of several cells; can account for many of the
clinical manifestations of both acute and chronic inflammation such as fever , anorexia,
cachexia, and movement of PMNs to the site of injury.
o Interferons – augment immunity through several processes especially the promotion of B-cell
maturation and moderation of suppressor T-cell function.
 The Complement System
- composed of a group of plasma proteins that normally lie dormant in the blood, interstitial fluid, and
mucosal surfaces. Microorganisms (or antigen-antibody complexes) activate the complement
system.
- Complement activation promotes inflammation and induces movement of leukocytes into the area of
injury.
- The final aspect of complement activation is the coating of microbes to make them vulnerable to
phagocytosis

II. Specific Immune Response: (adaptive immunity)

- the body recognizes and destroys foreign substances, but the response to them improves
each time the foreign substance is encountered.

1. Antigens are molecules that stimulate adaptive immunity.

2. B cells are responsible for humoral, or antibody-mediated, immunity. T cells are involved with cell-
mediated immunity.

Antibody-Mediated Immunity

1. Antibodies are proteins. The variable region combines with antigens and is responsible for antibody
specificity. The constant region activates complement or attaches the antibody to cells. The five classes
of antibodies are IgG, IgM, IgA, and IgD.
2. Antibodies directly inactive antigens or cause them to clump together. Antibodies indirectly destroy
antigens by promoting phagocytosis and inflammation.
3. The primary response results from the first exposure to an antigen. B cells from plasma cells, which
produce antibodies, and memory B cells.
4. The secondary (memory) response results from exposure to an antigen after a primary response.
Memory B cells quickly form plasma cells and new memory B cells.

Cell-Mediated Immunity

1. Exposure to an antigen activates cytoxic T cells and produces memory T cells.

2. Cytotoxic T cells lyse virus-infected cells, tumor cells, and tissue transplants. Cytotoxic T cells produce
cytokines, which promote inflammation and phagocytosis.

NATURAL OR ACQUIRED IMMUNITY

Natural
1. Passive natural immunity is the transfer of antibodies from a mother to her fetus during gestations or baby
during breastfeeding.
2. Active natural immunity results from everyday exposure to an antigen against which the person’s own
immune system mounts a response.
Artificial
1. Passive artificial immunity is the transfer of antibodies from an animal or another person to a person requiring
immunity.
2. Active artificial immunity results from deliberate exposure to an antigen (vaccine) to which the person’s own
immune system responds.
Summary: IMMUNE RESPONSE
1. Begins when a macrophage encounters a virus and consumes it. Meanwhile, other viruses look for nearby
cells to infect.
2. Next, the macrophage digests the virus and displays pieces of the virus called antigens on its surface.
Nearby cells have now become infected by the attacking viruses.
3. Unique among the many different helper T cells in the body, one particular helper T cell now recognizes the
antigen displayed and binds to the macrophage.
4. This union stimulates the production of chemical substances -- such as interleukin-1 (IL-1) and tumor
necrosis factor (TNF) by the macrophage, and interleukin-2 (IL-2) and gamma interferon (IFN-y) by the T cell
-- that allow intercellular communication.
5. As part of the continuing process, IL-2 instructs other helper T's and a different class of T cells, the killer T's,
to multiply. The proliferating helper T's in turn release substances that cause B cells to multiply and produce
antibodies.
6. The killer T cells now begin shooting holes in host cells that have been infected by viruses.
7. The antibodies released by the B cells bind to antigens on the surfaces of free-floating viruses. These
binding signals blood components called complement to puncture holes in the viruses.
8. Finally, as the infection is brought under control, the activated T and B cells are turned off by suppressor T
cells. However, a few "memory cells" remain behind to respond quickly if the same virus attacks again.

RISK FACTORS
1. Environmental factors ( air pollution, exposure to Hypersensitivity Reaction
second hand smoke, tabacco smoke) (Anaphylactic)
 Exposure to allergens (influence the
likelihood of development of allergy are
a person’s age at the time of exposure
—exposure in early life).
  Type of allergen (house dust mite,
cockroach, various medications, and
pollen)
 Allergen load (lower levels are capable
of inducing specific IgE production)
 The month of person’s birth (a greater
affinity for allergies is seen in those
born in the spring and fall).
Route of Entrance into the body (inhalation,
injection, ingestion, and direct contact).
2. Age: Infants and very young children (food allergies) Hypersensitivity Reaction (Anaphylactic)
Age: common in young adults (ages 19-29); leading Immunodeficiency
cause of death among adults (ages 25 to 44). Women (HIV/AIDS)
older than 50 acquire HIV through heterosexual
contact.
Age: Childbearing years (African-American Women)

4. Gender: Women, men, heterosexuals Immunodeficiency

(HIV/AIDS)
Gender: Female to male prevalence (10:1 for SLE
women in the childbearing years)
5. Genetic Factors: Familial aggregation occurs in SLE
10% of people having a first degree relative with SLE,
including its occurrence in identical twins.

Genetic disposition: 32% in identical twins rather than Rheumatoid Arthritis

9% rate in fraternal twins
Genetic differences between mother and fetus (RA
clients that joint pain and swelling disappear during
pregnancy)

5. Sexual Practices (exposure to blood and body fluids Immunodeficiency

through perinatal transmissions, men who have sex (HIV/AIDS)
with men, unsafe sexual practices, oral sexual
practices); Engaging into sexual activity while under the
influence of drugs or alcohol; Having multiple sexual
partners
6. IV drug users (paraphernalia were shared) Immunodeficiency
(HIV/AIDS)
7. Medical Factors: Transfusion of infected blood Immunodeficiency
products; Transplantation of infected donated tissues or (HIV/AIDS)
organs, needle stick exposure; Dental Care
(inadequate disinfection and sterilization of
instruments)
8. Occupational exposure to blood and blood products Immunodeficiency
(police officers etc) (HIV/AIDS)
9. Perinatal HIV exposure (vaginal delivery, post Immunodeficiency (HIV/AIDS)
partum breasfeeding)

ASSESSMENT / SCREENING / DIAGNOSTIC PROCEDURES

1. HEALTH HISTORY
 Elicit a description of the present illness and chief complaints (onset, course, duration, location and
precipitating and alleviating factors.)
 Elicit a description of the client’s overall health status, including immunizations status, usual childhood
diseases, known allergies, and a history of past and present medications.
 Explore the client’s health history for risk factors.

2. PHYSICAL EXAMINATION
 INSPECTION:
o Inspect skin and mucous membranes for rashes, lesions, dermatitis, purpura (subcutaneous
bleeding), and any type of inflammation or drainage.
o Assess the joints for tenderness, edema, and range of motion.
o Inspect ears for drainage, inflammation, and scarring from ear infections.
 PALPATION:
o Palpate the anterior and posterior cervical, axillary and inguinal lymph nodes for enlargement.
o Note the location, size and consistency of lymph nodes.
 AUSCULTATION:
o Auscultate lungs for abnormal lung sounds such as wheezing, crackles, and rhonchi.
o Auscultate heart sounds for abnormalities such as palpitations and dysrhythmias.

3. SCREENING PROCEDURES AND DIAGNOSTIC EXAMINATIONS

 Immunologic Status Test

 1. WBC and differential
2. Immunoglobulin levels
3. Total serum complement

 Skin Testing- diagnostic of choice in allergic disease. Introducing a small quantity of allergen into the skin by
quickly pricking, scratching, or puncturing it.
- Patch test can be used to evaluate contact allergies; the allergen is applied directly to the
skin and then covered with a gauze dressing.
- Scratch tests (tine tests or prick tests)
- Delayed-type hypersensitivity skin testing

 Radioallergosorbent (RAST) – uses the principle of immunoabsorption and reveals elevated levels of
allergen-specific IgE antibodies in the client’s blood. The allergen of interest is first bound to some solid
surface, usually a paper disk. The client’s blood is then incubated with the disk. If the client has antibodies
specific to the allergen being tested, they bind to that allergen. The unbound antibodies are washed away,
and the level of antigen specific IgE can be measured. This test is somewhat less sensitive than skin testing
and is more time consuming and costly.

 ImmunoCAP FEIA (Fluoroenzyme Immunoassay Test) – This method uses as the solid phase a flexible,
hydrophobic cellulosic disk to which an allergen has been linked. The advantage of this testing compared to
RAST testing is that it has a very high antigen binding capacity and has a minimal nonspecific binding with
high total IgE output.

 Blood Assays – Immunometric blood assays measure the total amount of IgE normally present in the
circulation. IgE concentration increase in the presence of hypersensitivities. Elevated serum eosinophil levels
also may suggest hypersensitivities.

 ELISA - The enzyme-linked immunosorbent assay (ELISA), or enzyme immunoassay (EIA), was the first
screening test commonly employed for HIV. It has a high sensitivity. This is the first step of an HIV test. This
test detects the presence of HIV antibodies in the blood. If the test is negative then the person is determined
not to be HIV infected and testing stops there. If the test is positive the second step of the test is run to
confirm the positive results of the first step.

 Western Blot. This test is used to confirm the positive Elisa test results. The Western Blot test detects
specific protein bands that are present in an HIV infected individual. In combination with a positive Elisa, a
positive Western Blot is 99.9 percent accurate in detecting that HIV infection has occurred.
RESULT INTERPRETATION:
o A positive result means that the person is HIV infected, but it does not predict the future course of
disease.
o A negative result means that HIV antibodies were not detected.
o Indeterminate results means that the enzyme immunoassay test was positive, but the Western Blot
test did not confirm the findings.

 Anti-nuclear antibody (ANA) assay - The most sensitive test for SLE. Almost all clients with SLE have
ANAs, but they also occur in many other situations, such as infections, advanced age, and RA and with
certain drug therapy regimens.

 Radiologic examinations. Confirm disease activity (soft tissue swelling), massive tissue swelling of the
entire joint; Subchrondal bone erosions or destruction

 Synovial fluid analysis. (Joint fluid analysis). Synovial fluid analysis may be ordered to help diagnose the
cause of joint inflammation, pain, swelling, and fluid accumulation.

Physical characteristics – the normal appearance of a sample of synovial fluid is usually:

 Straw colored
 Clear
 Moderately viscous – drops of it from a syringe needle will form a “string” a few inches long.
Changes in the physical characteristics may provide clues to the disease present such as:
 Less viscous fluid may be seen with inflammation.
 Cloudy synovial fluid may indicate the presence of microorganisms, white blood cells, or crystals.
 Reddish synovial fluid may indicate the presence of blood, but an increased number of red blood
cells may also be present in cloudy synovial fluid.
Chemical tests – tests that may be performed on synovial fluid samples may include:
 Glucose—typically a bit lower than blood glucose levels. May be significantly lower with joint
inflammation and infection.
 Protein—increased with bacterial infection.
 Lactate dehydrogenase—increased LD (LDH) level may be seen in rheumatoid arthritis, infectious
arthritis, or gout.
 Uric acid—increased with gout

PATHOPHYSIOLOGIC MECHANISMS

I. INFLAMMATORY AND INFECTION PROCESS

 Microorganisms
Trauma
Oxygen and nutrient deprivation
Chemical agents

Activates the cells of the body

(macrophages, mast cells, etc)

Release of inflammatory Protein rich fluid exudates from Movement of phagocytic cells
mediators (bradykinin, capillaries into the interstitial to the site of injury
histamine, serotonin, space
leukotriense, prostaglandins)

Activates endothelial cells

Leukocytes, platelets, and RBCs

become sticky and adhere to
the endothelial cells

INFLAMMATION

Fever
Leukocytosis
Increased plasma
proteins
Malaise
Increased ESR
Increased Fibrinogen

AUTOIMMUNE DISORDERS are conditions in which the body no longer differentiates self from nonself. The cause
of autoimmune diseases is still unknown but theories do exist.
 Abberations in HLAs acitivity genetic coding – the body may lose its ability to recognize and
differentiate self from nonself
 HLAs – human leukocyte antigen genes constitute major histocompatibility complex (MHC)
which is a large cluster of genes. Under normal conditions, if a peptide of foreign,
pathogenic, source is detected, it alerts the immune system that the cell may be infected
with a virus, and, thus, target the cell for destruction.
 Abnormal B cells and T cells reactivity - T cells or B cells produce autoantibodies that can cause
tissue injury.
 Genetic disposition

1. RHEUMATOID ARTHITIS

Definition: A chronic, symmetrical, progressive disease involving erosive inflammation of synovial tissue of the joints.

Incidence: Greater in women than in men; between age 30 to 60 years old.

Etiology: The cause is unknown although it is characterized by presence of RF (Rheumatoid factor) – autoantibody
against IgG; antibodies against collagen, Epstein barr virus; genetics factors
Pathophysiology:
INITIATION PHASE UNKNOWN

T LYMPHOCYTES MIGRATE TO THE INFLAMED AREA

IMMUNE RESPONSE
PHASE
PRODUCTION OF AUTOANTIBODIES

RHEUMATOID FACTORS against IgG

Deposition of Immune complexes lead to SYNOVIAL

INFLAMMATION

MORE LEUKOCYTES MIGRATE TO THE SYNOVIAL FLUIDS

INFLAMMATORY
PHASE SWELLING – DAMAGES TINY BLOOD VESSELS IN THE
SYNOVIAL MEMBRANE

THE BODY RELEASES ARACHIDONIC ACID AND LYSOSOMES

ENZYMES

FISSURES

FURTHER INFLAMMATION

DESTRUCTION PANNUS IS FORMED

PHASE

BONY EROSIONS

JOINT FUSION WITH PERMANENT

DEFORMITY

RHEUMATOID ARTHRITIS
EXPLANATION:
Joint deformity in RA occurs in four phases:
A. Initiation Phase. Some changes in the synovial lining are present without known etiology.
B. Immune response phase
Due to synovial changes, T cells migrate to the inflamed area and production of autoantibodies starts
in the form of RF (Rheumatoid Factor) against IgG leading to the formation of antigen-antibody
immune complexes. These complexes will be deposited to the synovium leading to inflammation.
C. Inflammatory phase
Swelling damages tiny blood vesselsin the synovial membrane leading to that contains synovial fluid
which leads for the body to release substances (arachidonic acid / lysosyomes) that create fissures
in the surface of the synovium. These intensify inflammation.
D. Destruction phase
Further inflammation leads to formation of PANNUS (thickened fibrous scar tissue). This will adhere
to the articular surface of the cartilage that will invade the joints that will lead to bony erosions.
Calcification of joint tissue can lead to joint fusion with permanent deformity.

Clinical manifestations:
 Morning stiffness in and around the joints
 Soft tissue swelling of three or more joint area
 Swelling of at least one wrist
 Simultaneous symmetrical swelling of joints
 Limited range of motion in affected joints
***Three types of hand deformities:
 Swan neck deformity – hyperextension of the PIP (proximal interphalangeal) joint with flexion
of the MCP (metacarpophalangeal) and DIP (distal interphalangeal) joints
 Boutonniere deformity – flexion of PIP joints and hyperextension of the DIP joints
 Ulnar drift - a hand deformity in which the swelling of the metacarpophalangeal joints (the big
knuckles at the base of the fingers) causes the fingers to become displaced, tending towards
the little finger
 Shoulder arthritis
 Cock-up toes – plantar subluxation of the metatarsal heads
 Baker’s cysts (Popliteal cysts)
 Subcutaneous rheumatoid nodules around affected joints
 Sjogren syndrome - a systemic autoimmune disease in which immune cells attack and destroy
the exocrine glands that produce tears and saliva
 Felty’s syndrome - characterized by the combination of rheumatoid arthritis, splenomegaly and
leucopenia and leg ulcers
 In later stage, weight loss, fever, anemia, muscle atrophy
 Systemic manifestations: Fatigue, weakness, anorexia
 Impaired immobility and ability to perform ADL

Diagnostic Procedures:
 Radiologic studies reveal abnormalities, progressive joint damage
 Presence of Rheumatic factor
 Erythrocyte sedimentation rate – increase
 C-reactive protein – increase
 Anti CCP (cyclic citrullinated protein sntibodies) – predictive of future development of rheumatoid
arthritis
 Synovial fluid analysis – milky, cloudy, or dark yellow fluid
 Arthroscopic exam – presence of pannus
 Bone and joint scan – confirm the diagnosis

Management:
 Pharmocological Management:
 Aspirin and NSAID – control local inflammatory process
 COX 2 inhibitor – Celecoxib (Celebrex)
 Ibuprofen (Advil)
 Piroxicam (Feldene)
 DMARD/ BRM (disease modifying antirheumatic drug / biologic response modifier) – modify
the destructive capacity of the disease
 Methotrexate - immunosupressant
 Oral glucocorticorticoids
 Antiiflammatory and immunosuppressive
 Prednisone
 Maintain function
 Therapeutic exercise: ROM; strengthening exercises (isotonic and isometric)
 Splinting
 Supportive pillows and foam mattress
 Heat and cold therapy

** The Prosorba column is a device which filters harmful immune complexes or antibodies from a patient's
blood. The actual Prosorba column itself is a small cylinder made of plastic about the size and shape of a small soda
can. Apheresis involves the collection of whole blood from patients with subsequent separation of its blood
components. It is used to remove a specific component from the blood. The Prosorba column, which is also called
"Protein-A Immunoadsorption Therapy", contains a sand like substance coated with Protein A, which has the ability to
bind antibodies. It consists of an inert silica matrix to which a component of Staphylococcus bacterium is covalently
bound. This protein (protein A) has the propensity to bind immunoglobin G (IgG) and IgG bound to antigen."
Each treatment takes approximately two hours and is done weekly for a course of 12 weeks. During the
treatment, the patient's blood channels through tubing attached to a catheter in the patient's arm and passes into the
Prosorba column. Another apparatus attached to the column serves to separate plasma from red blood cells. The
plasma is then filtered through the column, which is a plastic cylinder containing highly purified Protein A on a silica
matrix. Protein A binds to antibodies in the plasma. The plasma is then combined again with the red blood cells and
reinfused into the patient through another catheter in the opposite arm.

Surgical management:
 Tendon transfer and osteotomies – nodules may be surgically removed;
 Synovectomy – surgical removal of synovial
 Arthrodesis - the artificial induction of joint ossification between two bones via surgery
 Joint replacement
2. SYSTEMIC LUPUS ERYTHEMATOSUS

Definiton: A chronic systemic inflammatory disease affecting multiple body sytems.

ncidence: More on women than men; between 15 to 40 years of age

Etiology: UNKNOWN; certain factors that can trigger the disease: genes, infection, environmental irritants, physical
and emotional stress, exposure to ultraviolet radiation, viruses

Pathophysiologic Mechanisms:

UNKNOWN Defect in T suppressor cells

Production of autoantibodies Suppressed NK cells

ANAs Cells die

Nucleus are released

IMMUNE COMPLEX

DEPOSITION IN TISSUE AND

KIDNEY, Heart, Brain FURTHER INFLAMMATION

Decrease of oxygen in organs

SLE
and tissues

Clinical Manifestations:
These may be insidious or acute; client may remain undiagnosed for many years; these involve multiple body
systems:
 Musculoskeletal: arthralgias and arthritis; joint edema and tenderness; pain on movement and
morning stiffness
 Integumentary: Butterfly rash across the bridge of the nose and cheeks; oral ulcers
 Cardiovascular: pericarditis; popular; erythematosus, purpuric lesions on fingers, elbows, toes,
forearms, hands
 Respiratory: pleural effusion; pleuritis
 Neurologic: subtle changes in personality and cognitive ability; depression and psychosis
 Other systems: lymphadenopathy; sings of glomerulonephritis

Laboratory and Diagnostic study findings:

 ANA test is positive – most sensitive test for SLE
 RBC and WBC- decreased (thrombocytopenia, severe anemia, leukocytosis, leukopenia)
 C reactive protein – elevated in active infection
  Elevated ESR (erythrocyte sedimentation rate)
 Anti-DNA (antibody against DNA) test – high titer – most specific test for SLE
 Urine testing – proteinuria, cellular cast (due to kidney involvement)

Management:
 Pharmacologic management
 NSAIDs
 Glucosorticoids - Prednisone
 Immunosuppressant – Methotrexate
 IV immunoglobulins
 Antimicrobial agent – Dapsone (anti leprosy drug) – used to manage cutaneous
manifestations of lupus
 Dialysis / kidney transplantation

3. SYSTEMIC SCLEROSIS

Definiton: Scleros = hard

Derma = skin
Systemic sclerosis is also known as scleroderma.
Systemic sclerosis (SSc) is a systemic autoimmune connective tissue disease.
**Types: Localized scleroderma – affects the skin
Diffuse scleroderma or systemic sclerosis – internal organ involvement

Etiology:
 Environmental factors - Working with plastics, coal, silica dusts
 High alcohol intake
 Genetic factors
Phenylketonuria - is a rare condition in which a baby is born without the ability to properly break down an amino
acid called phenylalanine.
 Metabolic disorders
Hashimoto’s thyroiditis – or chronic lymphocytic thyroiditis is an autoimmune disease in which the thyroid gland is
gradually destroyed by a variety of cell- and antibody-mediated immune processes
 Malignancies
 Postinfectious disorders
 Neurologic conditions Environmental factors
Genetic factors
Pathophysiologic Mechanisms: Metabolic disorders
Malignancies
UNKNOWN Postinfectious disorders
Neurologic conditions

Lymphocytes accumulate in the lower dermis

Lymphokines stimulate fibroblasts to

produce excessive amount of procollagen

Procollagen matures

Insoluble collagen

Fibrotic changes: Skin

Loss of elasticity and movement Vascular changes: vascular endothelium is

injured

Localized scleroderma Damaged blood vessel

Overproduction of collagen from

vasoactive substances
 Fibrous thickening and narrowing of lumina

Tissue Ischemia

Systemic Sclerosis

Clinical Manifestations:
 Limited cutaneous scleroderma
Skin changes: fingers and distal portion of the extremities
skin may appear tight, reddish or scaly. Blood vessels may also be more visible.

CREST
o Calcinosis – calcium deposit in the tissues
o Raynaud’s phenomenon – intermittent vasospasm of the fingertips
o Esophageal hardening
o Sclerodactyly – scleroderma of the digits
o Telangiectasis – capillary dilations that form vascular lesions on the face

 Diffuse scleroderma
o Thickening of the skin on the extremities, face and trunk
o Swelling of the fingers, face and feet
o Tense, wrinkle-free appearance of the skin
o Hypo/hyperpigmentation – due to loss of normal skin folds
o Distal thickening
o Mask-like face
o Rigid mouth
o Blunt expression
o May develop problems in the following organs:
 Musculoskeletal: non specific joint pains, which can lead to arthritis, or cause discomfort
in tendons or muscles. Joint mobility, especially of the small joints of the hand, may be restricted
by calcinosis or skin thickening. Patients may develop muscle weakness, or myopathy
 Lungs: Some impairment in lung function is almost universally seen in patients with diffuse scleroderma
on pulmonary function testing; however, it does not necessarily cause symptoms, such as shortness of breath.
Some patients can develop pulmonary hypertension, or elevation in the pressures of the pulmonary arteries.
This can be progressive, and lead to right sided heart failure.
 Gastrointestinal tract: The most common manifestation in the esophagus is reflux esophagitis, which may
be complicated by peptic stricturing, or benign narrowing of the esophagus. Scleroderma can
decrease motility anywhere in the gastrointestinal tract. The most common source of decreased motility
involvement is the esophagus and the lower esophageal sphincter, leading to dysphagia and chest pain. As
Scleroderma progresses, esophageal involvement from abnormalities in decreased motility may worsen due
to progressive fibrosis (scarring). If this is left untreated, acid from the stomach can back up into the
esophagus causing esophagitis, and GERD. The small intestine can also become involved, leading
to bacterial overgrowth and malabsorption of bile salts, fats, carbohydrates, proteins, and vitamins.
The colon can be involved, and can cause pseudo-obstruction or ischemic colitis.

Laboratory and Diagnostic study findings:

 Decreased RBC – anemia due to damage to RBC from diseased blood vessel
 Increased ESR
 Protenuria (if with kidney involvement)
 Positive titers for ANA
 Anticentromere and anticentriole antibodies
A centromere is a region of DNA typically found near the middle of a chromosome
A centriole is a barrel-shaped cell structure

Management:
 Drug therapy
o Vasoactive agents
o Ca channel blockers for Raynaud disease
o Anti-inflammatory
o Glucocorticoids
o Low dose steroid (Prednisone is preferred)
o Immunosuppressive drugs
o Penicillamine (immunomodulating agent) – interferes with the cross-linking of collagen
o ACE inhibitors - Controlling hypertension in patient with renal complications
 ** Nursing Management
o BP monitoring
o Maintain a full ROM to facilitate muscle and joint movement
o Facial exercises
o No to alcohol and caffeine (raynaud’s disease)
o Appropriate easy to swallow, high caloric foods
o WOF diarrhea (malabsorption syndrome) or constipation (decreased motility of GI tract)

1. ACQUIRED IMMUNODEFICIENCY SYNDROME (AIDS)

Definition:
A severe immunodeficiency caused by HIV (human immunodeficiency virus). This virus causes cell death
and a decline in immune function resulting in opportunistic infections, malignancies, and neurologic problems.
** genetic promiscuity – capability of HIV to mutate rapidly; hallmark of HIV

**incubation period – 6 months to 5 years (average 2 years)

Etiology and Risk factors:

**HIV is sexually transmitted and thru direct contact with blood and blood products.
**Persons at risk of contracting HIV
 Anyone who engages in unprotected sex activity with an infected partner
 Recipient of transfused blood or blood components
 IV drug abusers
 Children of mother with HIV
 Health care workers – needle stick injuries

Pathophysiologic mechanisms:

HIV Retrovirus

Enters the host and attaches to the target cells CD4 T cells

HIV uncoated and RNA enters the cells

Reverse transcriptase

Viral RNA transcribed into DNA

Integrase
Viral DNA will integrate to the cellular DNA

Cell will function abnormally

Host cell die and viral budding occurs Loss of CD4 T cells

Significant decrease in coordinated immune activity

AIDS

Clinical Manifestations:
 Fatigue
 Fever and night sweats
 Weight loss
 Generalized lymphadenopathy - swollen/enlarged lymph nodes
 Nonproductive cough, shortness of breath
 Skin lesions, dry skin, pallor
 GI upset and chronic diarrhea
 Edema
 Visual impairment
  Painful oral lesions
 Brusing / bleeding tendencies
 Joint pain
 Opportunistics infections:
 Pneumocystis carinii pneumonia - a form of pneumonia, caused by the yeast-
like fungus Pneumocystis jirovecii. This pathogen is specific to humans. Pneumocystis is
commonly found in the lungs of healthy people, but being a source of opportunistic
infection it can cause a lung infection in people with a weak immune
system. Pneumocystis pneumonia is especially seen in people with cancer, HIV/AIDS and
the use of medications that affect the immune system.
 Kaposi sarcoma - a cancer that causes patches of abnormal tissue to grow under the skin, in
the lining of the mouth, nose, and throat or in other organs. The patches are usually red or
purple and are made of cancer cells and blood cells. The red and purples patches often
cause no symptoms, though they may be painful.

 Neurologic deficits – AIDS dementia complex or HIV-associated dementia (HAD), occurs primarily in
persons with more advanced HIV infection. Symptoms include encephalitis (inflammation of the
brain), behavioral changes, and a gradual decline in cognitive function, including trouble with
concentration, memory, and attention. Persons with ADC also show progressive slowing of motor
function and loss of dexterity and coordination.

Laboratory and Diagnostic study findings

 An overall decline in the total numbers of white blood cells
 Decreases in both the total number and the percentage of lymphocytes
 Significant changes in the CD4+/ CD8+ ratio
 Decreased CD4+ T-cell rest findings
 Absent or decreased skin test reactivity
 Increased immunoglobulin levels
 ELISA test – indicates exposure to or infection with HIV but does not diagnose AIDS
 Western blot test – identifies HIV antibodies

Management:
 Pharmacological management:
 Antiretroviral Therapy: Zidovudine (AZT); Delavirdine (Rescriptoe), Ritonavir (Norvir)
 Antibiotics
 Antidiarrheals
 Antiemetics

NURSING DIAGNOSIS
 Ineffective airway clearance r/t increased bronchial secretions (PCP, hypersensitivity)
 Risk for infection r/t weak or suppressed immune system (AIDS, RA, SLE)
 Pain r/t inflammation and increased disease activity, tissue damage, fatigue or lowered tolerance
level (RA)
 Fatigue related to increased disease activity, inadequate sleep and rest, emotional stress and
depression (RA, AIDS)
 Impaired physical mobility r/t decreased ROM, muscle weakness, pain on movement, lack of or
improper use of ambulatory devices (RA)
 Self care deficit r/t contractures, fatigue, loss of motion (RA, SSc)
 Body image disturbance r/t physical and psychological changes and dependency imposed by chronic
illness (RA)
 Impaired skin integrity r/t cutaneous manifestations (AIDS, SLE, SSc, KD)
 Fluid volume deficit r/t to loss of body fluids secondary to diarrhea (AIDS)
 Knowledge deficit r/t disease process (autoimmune disorders)
 Risk for injury r/t decreased ROM, muscle weakness, pain on movement, lack of or improper use of
ambulatory devices (RA, SSc)
 Ineffective individual or family coping related to actual or perceived lifestyle or role changes (RA,
AIDS)
 Anticipatory grieving r/t changes in lifestyle and roles and to unfavorable prognosis (AIDS)
 Social Isolation r/t stigma of the disease, withdrawal of support systems, isolation procedures and
fear of infecting others

PRINCIPLES OF MANAGEMENT FOR INFLAMMATORY AND IMMUNOLOGIC DISORDERS:

MEDICAL MANAGEMENT:
1. PHARMACOLOGICAL MANAGEMENT FOR IMMUNE DISORDERS:
a. Antimicrobial drugs – prevent and treat infections
b. Antipruritic agents (topical steroids) – relieve or prevent itching
c. Antihistamine – treat allergic responses
d. Anti-inflammatory – suppresses inflammation
e. Antiretrovirals – for patients with AIDS
f. Bronchodilators – relaxes bronchial smooth muscle
 g. Corticosteroids – suppress inflammation
h. Mast cell inhibitors – inhibits mast cells; release of chemical mediator that promotes bronchodilation
i. Vasopressors – restore blood pressure in anaphylaxis

2. IMMUNOTHERAPY – Hypersensitivity and allergic disorders

3. PROSORBA COLUMN - RA
4. DIALYSIS - SLE
5. KIDNEY TRANSPLANTATION - SLE

SURGICAL MANAGEMENT specific to RHEUMATOID ARTHRITIS:

 Tendon transfer and osteotomies – nodules may be surgically removed
 Synovectomy – surgical removal of synovial
 Arthrodesis - the artificial induction of joint ossification between two bones via surgery
 Joint replacement

NURSING MANAGEMENT:
 Assess respiratory status
 Assessment of lungs, rate and depth of respiration, effort of breathing, use of accessory
muscles, cyanosis, restlessness, anxiety, or any change in level of consciousness
 Minimize the risk of infection
 Instruct the client on ways to avoid infection (personal hygiene, and avoidance of people with
infections and large crowds)
 Instruct patients to wash area with warm water before applying topical creams; instruct client
to before and after administering topical creams
 Mouth care
 Avoid exposure to individuals who are sick
 Well balanced diet
 Provide pain relief
 Assess client’s pain, rule out any complications
 Implement nonpharmacologic intervention (ice, coldm massage) to relieve pain
 Administer pain medication
 Evaluate effectiveness of interventions
 Promote skin integrity
 Assess skin and mucous membrane for any rashes, color changes, lesions, pallor, purpura,
hydration and inflammation
 Keep skin clean and dry. Do not use harsh soaps.
 Instruct client to avoid scratching, adhesive tapes
 Maintain fluid balance.
 Monitor intake and output (UO)
 Assess for dehydration: dry mouth, the eyes stop making tears, sweating may stop, muscle
cramps, nausea and vomiting, heart palpitations, and lightheadedness (especially when
standing).
 Prevent diarrhea: avoid bowel irritants such as raw fruits, vegetable, spicy foods and hot/cold
foods.
 Provide client and family teaching.
 Possible triggers of the disease (allergens)
 Danger signs and symptoms (signs of respiratory distress and infection)
 Teach methods for airway clearance: turning, coughing, and deep breathing, increase fluid
intake, maintain semifowler’s position
 Energy conservation techniques: sitting while doing morning care
 Promote self-care
 Assist the client in ADL as needed but promote independence.
 Prevent Injury
 Instruct client to wear tags or bracelets concerning disease or allergies.
 Proper body alignment to prevent contractures.
 Promote adequate rest and sleep
 Comfort measures thru Foam mattress and supportive pillows for RA patients
 Relaxation techniques: diversional activities
 Maintain nutritional status
 Control nausea and vomiting: food that are easy to swallow, high protein, high caloric foods
 Administer appetite stimulant as ordered.
 Promote client and family coping.
 Encourage verbalization of feelings and ways to prevent exacerbations.
 Provide referrals to counselors and support groups; and collaborate with physical and
occupational therapists (for patient with RA in the use of braces, splints, assistive mobility
devices).