Autoimmune Autoimmune Disorders and Disorders and

DiseasesDiseases

AUTOIMMUNE DISORDERSAUTOIMMUNE DISORDERS• Autoimmune: development of

an immune response to one’s own tissues– An “immune attack” on the self– Failure to distinguish ‘self’ protein

from ‘foreign’ protein• For some unknown reason,

immune cells that are normally unresponsive (i.e. tolerant to self-antigens) are activated

AUTOIMMUNE DISORDERSAUTOIMMUNE DISORDERS

• Disorders include: pernicious anemia, Guillain-Barre’ syndrome, scleroderma, rheumatic fever, RA, myasthenia gravis, MS, autoimmune hemolytic anemia, Type 1 diabetes, glomerulonephritis, SLE

AUTOIMMUNE DISORDERSAUTOIMMUNE DISORDERS• Treatment• Plasmapheresis: the removal of plasma that

contains components causing or thought to cause disease

• When plasma is removed, it is replaced by substitution fluid (e.g. saline or albumin). “plasma exchange”.

• In autoimmune disorders, the rationale is to remove pathologic substances present in the plasma

AUTOIMMUNE DISORDERSAUTOIMMUNE DISORDERS• Nursing responsibilities with

plasmapheresis:– Most common complications are

hypotension or citrate toxicity• Hypotension is usually the result of

vasovagal reaction or transient volume changes

• Citrate is used as an anticoagulant and may cause hypocalcemia

Autoimmune DiseasesAutoimmune Diseases

• Includes: rheumatoid arthritis, systemic lupus erythematosus, myasthenia gravis, addison’s disease, crohn’s disease, multiple sclerosis, etc.

Rheumatoid Arthritis (RA)Rheumatoid Arthritis (RA)• RA is a chronic disease characterized by

joint stiffness

• Cause is unknown but seems to be a genetic predisposition

• • Immune complexes are formed within the

joint inflammation, swelling and deformity

• Pattern of joint involvement is symmetrical

Rheumatoid Arthritis (RA)Rheumatoid Arthritis (RA)• Thickening of synovial fluid leads to

calcification, joint pain, limited mobility, and deformity

• Damage to bone begins within the first two years of the onset of RA

• May have periods of Remissions and Exacerbations

Med/Surg Mgmt of RAMed/Surg Mgmt of RA• Medical

o Reduce inflammation, relieve pain, maintain normal joint function, and promote general good health

o Therapeutic regimen includes meds, rest, hot and cold applications, and stress management

o Dieto Nutritious, well balanced diet w/ foods

high in iron when RBCs are low

Med/Surg Mgmt of RAMed/Surg Mgmt of RA• Surgical

– Hip, knee and finger joints may be replaced

• Pharmacological– NSAIDS & Salicylates may relieve pain,

but do not control the disease– Deltasone, Gold Salts, Azulfidine,

Imuran, Plaquenil, Depen or Rheumatrex may be used

– May have serious side effects

Med/Surg Mgmt of RAMed/Surg Mgmt of RA• Activity

– PT and OT are part of the therapeutic team

– ROM, resting splints, and assistive devices are often used

RA Nursing ProcessRA Nursing Process• Nursing Management

– Encourage relaxation techniques, warm showers, and planned rest periods

– Emphasize importance of doing ROM several times daily

RA AssessmentRA Assessment• Subjective

– Client hx reveals a gradual development of symptoms beginning initially w/ early a.m. stiffness and pain in finger joints

– Eventually other joints involved– Fatigue, muscle weakness, malaise and

loss of appetite develop– Obtain information about remissions and

exacerbations

RA Nursing ProcessRA Nursing Process• Objective

– Skin may show presence of ulcers and rheumatoid nodules

– Eye tissue may be inflamed– Reduction in tear and saliva production

can occur (Sjogren’s Syndome)– May have weight loss and elevated

temperature– X-rays demonstrate the amount and

degree of deformity

RA Nursing ProcessRA Nursing Process• Hands may reveal the classic

deformities associated w/ RA:•Boutonniere deformity

•Ulnar drift

•Swan-neck deformity

RA LabsRA Labs• No specific lab test confirms RA

though alterations in the following may occur:– RBC’s decrease as disease

progresses– Elevation of:

•WBCs•ESR•ANA•CRP•Platelet count

SLE (Systemic Lupus Erythematosus)

• A chronic, progressive, incurable disease affecting multiple body organs

• Characterized by remissions and exacerbations

• Occurs most commonly in women during childbearing years

• 2-3 times more common in African - Americans

SLE

• Abnormal B-lymphocyte cells produce “auto-antibodies” that destroy body cells

• Immune complexes are formed and circulate in serum causing inflammation in the skin, brain, kidney, lung, heart or joints

• If 4 or more established criteria are present, diagnosis of SLE is confirmed

SLE Medical-Surgical Managementt•Medical

– Treatment aimed at decreasing tissue inflammation and destruction

– Client can assist in controlling the disease by:•Stress management•Rest•Exercise•Taking medications as prescribed

SLE (Systemic Lupus Erythematosus)SLE (Systemic Lupus Erythematosus)Medical-Surgical ManagementMedical-Surgical Management

• During acute exacerbations, plasmapheresis may be used

• SLE clients are carefully monitored for renal, cardiac, pulmonary, hematological and neurological damage

• Large percentage of clients eventually have renal failure, requiring dialysis to maintain life

SLE Medical-Surgical Management• Pharmacological

– NSAIDs are used for joint and muscle pain

– Lowest possible doses of corticosteroid is used to suppress immune system activity

– Higher doses may be required during periods of exacerbations

SLE Med-Surg Management• Pharmacological cont.

– Plaquenil sulfate is used to treat side effects; can lead to development of retinal toxicity

– Cytoxan or Imuran may be used for severe SLE

SLE Medical-Surgical Management

• Diet– Low in sodium and glucose & high in

potassium due to effects from corticosteroids

– Discourage excessive fluid intake

• Activity– Sleep at least 8 hours at night, schedule

rest during the day– Regular exercise helps prevent muscle

weakness and fatigue

SLE Nursing Process

• Nursing Management– Teach client importance of avoiding

direct sunlight and the use of protective clothing and sunscreen

– Encourage client to balance rest & activity and to eat a balanced diet with low sodium

– Emphasize signs of exacerbation and early signs of infection

SLE Nursing AssessmentSLE Nursing Assessment

– Subjective• Ask when disease began, what symptoms

have developed, and how they have been treated

• Note medications and side effects, and degree of fatigue

• Determine client’s understanding of disease and how they are coping

• Client may describe malaise, photosensitivity, pain in joints, irregular menses, irritability, confusion, hallucinations

SLE - Medical-Surgical ManagementSLE - Medical-Surgical Management

• Nursing Process Assessment– Objective Data

• Head to toe assessment

SLEMost Common Objective Findings

– Joint swelling and pain

– Fever– Swollen glands– N/V– Anorexia– Hypertension– Respiratory

and cardiac infections

– Renal infections

– Enlarged liver and spleen

– Skin lesions, especially “butterfly rash”

– Raynauds phenomenon may develop with exposure to cold

SLE (Systemic Lupus Erythematosus)SLE (Systemic Lupus Erythematosus)Medical-Surgical ManagementMedical-Surgical Management

• Labs– Frequently reveal serum ANA and anti-

DNA antibodies

– LE cells are present in most cases

– Anemia, leukopenia, and thrombocytopenia are evident

Myasthenia Gravis (MG)

• Autoimmune disease characterized by extreme muscle weakness due to the body’s inability to transmit nerve impulses to voluntary muscles

Myasthenia Gravis

• It is thought that MG clients develop antibodies that act to decrease the number and effectiveness of acetylcholine receptor sites at neuromuscular junctions

• Voluntary muscles are most commonly involved

Myasthenia Gravis (MG) Myasthenia Gravis (MG)

• In mild conditions known as Group I ocular myasthenia, only the eye muscles are involved

Myasthenia Gravis (MG)Myasthenia Gravis (MG)

• As severity increases, symptoms of Group II generalized myasthenia develop

• Facial, neck, skeletal, and respiratory muscles become affected

• Periods of remission and exacerbation occur

Myasthenia Gravis (MG)Myasthenia Gravis (MG)

• Three possible complications:– Respiratory distress, such as dyspnea,

tachypnea, tachycardia, and diaphoresis– Myasthenia crisis is an acute

emergency; muscle weakness, difficulty swallowing, chewing, or talking, and respiratory distress

– Cholinergic crisis is a result of an overdose of anticholinesterase medications

MG Med/Surg Management

• Medical– Use of anticholinesterase medications

and plasmapheresis– Used primarily for acute crisis or no

response to drug therapy or prior to a thymectomy

MG Med/Surg Management

• Surgical– Removal of the thymus gland has shown

the best results in young people early in the course of the disease

MG Med/Surg ManagementMG Med/Surg Management

• Pharmacological– Anticholinesterase medications such as

Mestinon, Prostigmin, and Mytelase are prescribed

– Individual dosages must be determined– Steroids also prescribed to slow down

immunological response

MG Med/Surg Management

• Diet– Encourage clients to eat a snack before

taking anticholinesterase medications

– Diet may need adjustment with chewing and swallowing difficulties

• independent

MG Med/Surg Management• Activity

– Client should avoid excessive muscular activity and rest periodically throughout the day

– ROM exercises, braces, splints, and walkers assist in keeping the client

MG Med/Surg ManagementMG Med/Surg Management

• Nursing Management– Teach client airway protective

techniques

– Encourage client to change daily activity pattern, and ROM exercises

– Emphasize need to see physician at first sign of upper respiratory infections

MG Nursing ProcessMG Nursing Process

• Objective Data• Must access level of muscle groups

affecting the eyes, face, neck, and chest– Look for Diplopia – double vision– Look for ptosis – drooping upper eyelids– Look for facial symmetry

MG Nursing Process

• Objective Data– Note chewing or swallowing problems

– Vocal tones and breath sounds should be assessed

– Level of weakness in arm and leg muscles as well as breathing muscles should all be noted

MG Nursing Process

• Objective Data cont.– Ach receptoros antibody and LE cell

tests are often positive

– X-rays and CT scans detect enlargement of thymus

– EMG determines extent of muscle damage

Matching (?)Matching (?)1. Crohn’s Disease2. Graves’ Disease3. Systemic Lupus

Erythematosus4. Multiple Sclerosis5. Rheumatoid

Arthritis

A. Skeletal SystemB. Nervous SystemC. Digestive SystemD. Integumentary

SystemsE. Endocrine Gland