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Autoimmune Disorders and Diseases AUTOIMMUNE 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 selfantigens) are activated AUTOIMMUNE DISORDERS • Disorders include: pernicious anemia, GuillainBarre’ syndrome, scleroderma, rheumatic fever, RA, myasthenia gravis, MS, autoimmune hemolytic anemia, Type 1 diabetes, glomerulonephritis, SLE AUTOIMMUNE 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 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 Diseases • Includes: rheumatoid arthritis, systemic lupus erythematosus, myasthenia gravis, addison’s disease, crohn’s disease, multiple sclerosis, etc. 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) • 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 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 Diet o Nutritious, well balanced diet w/ foods high in iron when RBCs are low Med/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 RA • Activity – PT and OT are part of the therapeutic team – ROM, resting splints, and assistive devices are often used RA Nursing Process • Nursing Management – Encourage relaxation techniques, warm showers, and planned rest periods – Emphasize importance of doing ROM several times daily RA 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 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 Process • Hands may reveal the classic deformities associated w/ RA: •Boutonniere deformity •Ulnar drift •Swan-neck deformity RA 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 Management • 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) Medical-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 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 Management • Nursing Process Assessment – Objective Data • Head to toe assessment SLE Most 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) Medical-Surgical Management • Labs – Frequently reveal serum ANA and antiDNA 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) • In mild conditions known as Group I ocular myasthenia, only the eye muscles are involved 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) • 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 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 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 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 (?) 1. Crohn’s Disease 2. Graves’ Disease 3. Systemic Lupus Erythematosus 4. Multiple Sclerosis 5. Rheumatoid Arthritis A. B. C. D. Skeletal System Nervous System Digestive System Integumentary Systems E. Endocrine Gland