-
Autoimmunity:
A disease produced when the bodies own tolerance of the antigens (proteins) on its own cells become disrupted
Autoantibodies produced by B-Lymphocytes or T-Lymphocytes attack normal cells whose surface contains self antigen or auto antigen destroying the tissues
-
Important hints!
- Vi Ra L: viral, right shift, increased lymphocytes
-LBN: bacterial, left shift, increased neutrophils
- Cyte- cell
- Leuko- white
- Erythro- red
- Exo- outside
- Emia- blood
- Dys- difficult or painful
-
Blood Cell Production:
- A Pluripotent Stem Cell can turn into anything
- When a pluripotent stem cell turns into a Myeloid Stem Cell it then turns into various precursor or blast cells then turning into neutrophils, eosinophils, basophils (mast cell), monocyte (macrophage), platelets, and erythrocytes (red blood cells).
- When a Pluripotent Stem Cell turns into a Lymphoid Stem Cell then into Blast cells, finally turning into Lymphocytes.
-
Hypersensitivity Reactions
- - Altered immune response leading to disease or damage
- -Allergy = exogenous antigens (outside the body)
- -Alloimmune = immune response against tissues of another (transplant needs to be on an antirejection drug whole life)
- -Autoimmune = disturbance in the immnologic tolerance of self-antigens
- --Tissue injury from auto-antibodies or auto-reactive T cells
- ***Muscle pain, diarrhea, n/v = NOT ALLERGIC REACTION
-
Factors in Autoimmunity
- - Genetic predisposition
- - Insult or trigger- which alters immunologic hemeostasis
- - The actual immunologic process that causes the disease symptoms
- --IgE, tissue specific, immune complex related, or cell mediated
-
Immune Tissues
- - Appendix
- - Spleen
- - Thymus
- - Skin
- - Bone Marrow
- - Thymus (kids)
- - Lymph nodes
-
Rheumatoid Arthritis
- -One of the most common connective tissue diseases AND the most destructive to the joints
- -More common in middle-aged women
- *Chronic, progressive, systemic inflammatory autoimmune disease that affects primarily synovial joints
- Early Manifestations:
- Joint:
- -Inflammation
- Systemic:
- -Low grade fever
- -Fatigue
- -Weakness
- -Anorexia
- -Paresthesias
- Late Manifestations:
- Joint:
- -Deformities
- -Moderate to severe pain and morning stiffness
- Systemic:
- -Osteoporosis
- -Severe fatigue
- -Anemia
- -Weight loss
- -Subcutaneous nodules
- -Peripheral neruopathy
- -Vasculitis
- -Pericarditis
- -Fibrotic lung disease
- -SJOGRENS SYNDROME: most common associated syndrome; look for dry eyes(often feel "gritty" as if sand is in there eyes), dry mouth, or dry vagina.
- -Renal disease
- Labs:
- -Decreased albumin (normal: 3.5-5)
- -Positive rheumatoid factor
- -Positive ANA
- -Increased ESR (normal: up to 15 male, up to 20 female)
- -Presence of HLA
-
Lupus Erythematosus
- -Chronic, multi-system inflammatory
- -Generalized throughout the body
**MOST COMMON:BLACK WOMEN LATE ADOLESCENCE TO YOUNG ADULTHOOD
-Most common systems to be affected: joint, kidney, skin, heart, brain, hematology
-AVOID SUNLIGHT!
- MANIFESTATIONS:
- -Inflamed
- -Butterfly rash (sometimes scarring from inflammation can develop)
- -Nephritis
- -Pericarditis
- -Raynauds phenomenon
- -Pleural effusions
- -CNS lupus
- -Abdominal pain
- -Joint inflammation
- -Myositis
- -Fever
- -Fatigue
- -Anorexis
- -Vasculitis
- Labs:
- -False positive VDRL for syphilis is common
- -+ANA titre
- -ESR (sed rate) will be increased
- Diagnosis:
- ATLEAST three of the following:
- -facial or discoid rash, oral ulcers, photosensitivity, non-erosive arthritis of atleast 2 peripheral joints, proteinuria, neurologic(seizures, psychoses), hematologic disorders and immunologic (+LE cell prep, anti double stranded DNA, ASA, false + syphilis), +ANA titre
- -Fever, fatigue
- -Numerous inflammatory and immunologic tests
- -Skin lesions of present
- -CBC: commonly shows pancytopenia (decrease of all cell types)
- -Electrolytes, renal, cardiac, elect, liver, and clotting
- Collaborative Management:
- -Symptomatic
- -Exacerbations
- -Hydroxychloroquine (Plaquenil): Main drug, FREQUENT EYE EXAMS
- -Steroids
- -Immunosuppressive agent: Methotrexate
- -AVOID SUN, STRESS, AND FREQUENT EYE EXAMS WITH PLAQUENIL
- *Monitor body temperature because it is the major sign of exacerbation
- *Avoid sun exposure, and other forms or ultraviolet lighting
- *Use mild soap to wash the skin and avoid harsh, perfumed substances
- *Alopecia is common during exacerbations
-
Progressive Systemic Sclerosis (Scleroderma)
- -When systemic: similar to SLE
- -Connective tissue: inflammation, fibrosis (tough like scar tissue, and sclerosis (hardening)
- -Skin, calcium deposits, Raynauds, esophogeal dysmotility, spider like hemangiomas, internal organs
- -Not as responsive to steroids and immunosuppressants
- -Affects almost every oran in the body
- Symptoms:
- -Inflamed
- -Fibrotic
- -Sclerotic
- -Edematous
- -Renal failure: what will kill you with this disease
- -Myocardia fibrosis
- -Raynauds phenomenon
- -Interstitial fibrosis
- -Pulmonary hypertension
- -Esophagitis
- -Ulcers
- -Joint inflammation
- -Myositis
- -Fever
- -Fatigue
- -Anorexia
- -Vasculitis
- -Shiny, taut digits, sausage like
- -Initially swelling, then hard, tight, thickened: decreased ROM, ulcers
- -Dysphagia, GERD
- -Claw like appearance to fingers
- CREST:
- Calcinosis
- Raynauds
- Esophagus dysmotility
- Sclerodactyly
- Telangiecstasia (spider veins)
Organ damago most commonly goes to GI tract, cardiovascular system, pulmonary system, and renal system
*Large doses of immunosuppressants and streroids are used but are not as responsive
-
Raynauds
- -Vasospasm of the arterioles and arteries in the periphery
- -Femals are more common than males
- -Blanching followed by hyperemia (too much blood), pain, swelling
- -Avoid the cold, DON'T smoke (because makes vasospasm worst)
- Parashthesia: disturbed feeling
- -Can develop necrosis
-
Sjogrens Syndrome
- -Inflammatory cells and immune complexes block of secretory ducts and glands
- -Effects glandular of secretory ducts
- -Dry eyes, dry mouth, dry vagina
- -Associated with other autoimmune diseases (most often rheumatoid arthritis or fibromyalgia)
- -Effects women ages 35-45 most frequently
- -Local management
- -Keep up with dental and eye appointments
- -More prone to dental problems with dry mouth
- *Treat symptomatically
- -Methotrexate or cyclophosphamide (both of these have serious long term s/e especially on liver and bone marrow function)
- -Corticosteroids, cyclosporine, and plaquenil
-
Sarcoidosis
- -Widespread granulomatous lesions
- -90% in lungs
- -Symptomatic
- -End up with restrictive lung disease
- -Diagnose by bronchoscopy with biopsy
- -Steriods
- -Manage like restrictive lung diseases
- -Manage with steroids
-
Vitiligo
Loss of pigmentation
- -Melanocytes of the skin are destroyed
- -White patches on the skin of which is exposed to the sun
- -Topical steroid therapy can be used to treat
-
Ankylosing Spondylitis
- -Fusion of inflamed vertebrae
- -More common in males than females
- -Late teens, early 20s
- -Back pain and stiffness
- -Systemic- lung, bowel, cardiac(aortic valve), other joint
- -Treatment- physical therapy, NSAIDS
-
Hypersensitivity
Hypersensitivity or Allergy: increased or excessive response to the presence of an antigen(foreign protein or allergen) to which the patient has been previously exposed
- These problems can range from:
- -Uncomfortable: itchy, watery eyes, or sneezing
- TO
- -Life Threatening: allergic asthma, anaphylaxis, bronchorestriction, or circulatory collapse
-
Type 1: Rapid Hypersensitivity Reactions
- -Most common type of allergy
- Results from the increased production of the immunoglobulin E antibody class
Examples: anaphylaxis and allergic asthma
When all blood vessels and bronchiolar smooth muscle causing widespread blood vessel dilation, decreased cardiac output, and bronchoconstriction= ANAPHYLAXIS
-
Type 2: Cytotoxic Reactions
- -The body makes special autoantibodies directed against self cells that have some form or foreign protein attatched to them
- -the autoantibody then binds to the self cell and forms an immune complex
- -The self cell is then destroyed along with the attatched protein by phagocytosis or lysis
Examples: hemolytic anemia, thrombocytopenia prpura, hemolytic transfusion reactions (pt receiving wrong blood type during a transfusion), good-pastures syndrome, and drug-induced hemolytic anemia
- Treatment:
- -Discontinue the offending drug or blood product
- -Plasmapheresis (filtration of the plasma to remove specific substances) to remove autoantibodies may be beneficial
- -Otherwise treatment is symptomatic
- -Complications such as hemolytic crisis and renal failure can be life threatening
|
|