Autoimmune Disorders and Diseases

1. Autoimmune Disorders and Diseases

2. 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 self-antigens) are activated

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

4. 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

5. 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

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

7. 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

8. 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

12. Med/Surg Mgmt of RA • Medical • Reduce inflammation, relieve pain, maintain normal joint function, and promote general good health • Therapeutic regimen includes meds, rest, hot and cold applications, and stress management • Diet • Nutritious, well balanced diet w/ foods high in iron when RBCs are low

13. 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

14. Med/Surg Mgmt of RA • Activity • PT and OT are part of the therapeutic team • ROM, resting splints, and assistive devices are often used

15. RA Nursing Process • Nursing Management • Encourage relaxation techniques, warm showers, and planned rest periods • Emphasize importance of doing ROM several times daily

16. 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

17. 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

18. RA Nursing Process • Hands may reveal the classic deformities associated w/ RA: • Boutonniere deformity • Ulnar drift • Swan-neck deformity

21. 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

22. 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

23. 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

26. 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

27. 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

28. 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

29. 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

30. 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

31. 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

32. 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

33. SLE - Medical-Surgical Management • Nursing Process Assessment • Objective Data • Head to toe assessment

34. 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

35. SLE (Systemic Lupus Erythematosus)Medical-Surgical Management • Labs • Frequently reveal serum ANA and anti-DNA antibodies • LE cells are present in most cases • Anemia, leukopenia, and thrombocytopenia are evident

36. Myasthenia Gravis (MG) • Autoimmune disease characterized by extreme muscle weakness due to the body’s inability to transmit nerve impulses to voluntary muscles

37. 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

38. Myasthenia Gravis (MG) • In mild conditions known as Group I ocular myasthenia, only the eye muscles are involved

39. 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

40. 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

41. 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

42. 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

43. 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

44. 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

45. 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

46. 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

47. 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

49. 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

50. 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