1. Musculoskeletal: Autoimmune; Inflammatory; Metabolic; Infectious Disorders Rheumatoid Arthritis; SLE; Paget’s Disease; Gout; Osteomyelitis Carolyn Morse Jacobs, RN, MSN, ONC 10/ 24/ 04

2. Autoimmune and Inflammatory Disorders: Rheumatoid Arthritis Chronic systemic, inflammatory disease characterized by recurrent inflammation of connective tissue, primarily of joints ( diarthroidal) and related structures.

3. Pathophysiology Rheumatoid Arthritis Normal antibodies (immunoglobulins) become autoantibodies and attack host tissues (RF) Neutrophils, T cells synovial fluid cells acitavted; Cystokines, interleukin-1 and TNR (tumor necrosing factor) alpha; chrondroytes attack cartilage; Synovium digests cartilage; inflammatory molecules released containing interleukin-1 and TNF alpha

4. Pathophysiology: Rheumatoid Arthritis Pathophysiology IgG/RF (HLA)= antigen-antibody complex Precipitates in synovial fluid Inflammatory response Cartilage connective tissue primarily affected!

5. Rheumatoid arthritisRheumatoid arthritis: assessment: manifestations and complications Fatigue, weakness, pain Joint deformity Rheumatic nodules Multisystem involvement

6. How does Rheumatoid Arthritis Compare to Osteoarthritis ? Definition: *wear and tear, progressive, non-systemic, Degenerative Joint Disease (DJD) Pathophysiology

7. Identify which joints are primarily affected with osteoarthritis. What factors contribute to the development of osteoarthritis? Osteoarthritis (top slide only) Ankylosing spondylitis (what type of arthritis is this?)

8. Structural changes with Osteoarthritis Early Cartilage softens, pits, frays Progressive Cartilage thinner, bone ends hypertrophy, bone spurs develop and fissures form Advanced Secondary inflammation of synovial membrane; tissue and cartilage destruction; late ankylosis

9. Normal Knee structure Moderately advanced osteoarthritis Advanced osteoarthritis What signs and symptoms does the person with osteoarthritis experience?

10. What symptoms/assessment for the patient with osteoarthritis? Onset of pain is insidious, individual is healthy! Pain is aching in nature; relieved by rest!. Local signs and symptoms: swelling, crepitation of joint and joint instability, asymmetrical joint involvement

11. Deformities with Osteoarthritis Genuvarus Herberden’s nodes Carpometacarpocarpal joint of thumb with subluxation of the first MCP

12. Osteoarthritis (review only) Diagnostic Tests None specific Late joint changes, boney sclerosis, spur formation Synovial fluid inc., minimal inflammation Gait analysis Nursing diagnosis Interventions determined by complications Supportive devices Medications (no systemic treatment with steroids) Dietary to dec. wt. Surgical Intervention (joint replacement) Teaching

13. Comparison of RA and OARAOA RA Cause unknown; auto-immune factor Onset sudden Remissions *Body parts affected, systemic, small joints, symmetrical Causes redness, warmth, swelling of joints Females, age 20-30; 3-1 ratio OACause “wear and tear”, develops slowly Non-systemic, weight bearing joints Middle-aged and elderly, males 2-1 affected Does not cause malaise Begins after 40

14. Manifestations of RA Systemically ill Hematologic Pulmonary/CV Neurologic Ocular symptoms (Sjorgen’s) Skin Musculoskeletal deformity, pain Pain! Pain

15. Joints changes with RA Early Pannus Granulation, inflammation at synovial membrane, invades joint, softens and destroys cartilage

16. Mod advanced Pannus joint cartilage disappears, underlying bone destroyed, joint surfaces collapse Fibrous Ankylosis Fibrous connective tissue replaces pannus; loss of joint otion Bony Ankylosis Eventual tissue and joint calcification RA

17. Joint Changes RA Bilateral, symmetrical, PIP’s, MCP’s Thumb instability Swan neck, boutonniere deformity Tensynovitis Multans deformity Subcutaneous nodules Genu valgum Pes plano valgus Prominent metatarsal heads Hammer toes

18. Assessment RA Deformities that may occur with RA Synotenovitis Ulnar drift Swan neck deformity Boutonniere deformity

19. Mutlans deformity (rapidly progressing RA) Hitch-hiker thumb Genu valgus

20. Subcutaneous nodules ( disappear and appear without warning)

21. Hammer toes

22. Diagnostic Tests RA ESR elevated + RA, ^ RA titer Sed rate increased CBC C-reactive protein Dec. serum complement Synovial fluid inflammation Joint and bone Swelling,inflammation

23. Interventions RA Nursing Diagnosis Comfort Physical mobility Self image Goals Team Approach Pain management Exercise Surgery Teaching

24. Medications RA ASA & NSAIDS Corticosteroids; low dose DMARDs (diverse group) of remitting agents: including antimalarial (hydroxychloroquine:plaquenil) *eye effects; Penicillamine (empty stomach); Gold (Auranofin) *dermatitis, blood dyscrasia ; and Immunosuppressive agents as methotrexate and cyclosporine Biologic response modifiers Adalimumab (Humira) Infliximab (Remicade)

25. Joint Protection: Do’s and Don’t’s

26. Case Presentation; Mrs. Michaels with Rheumatoid Arthritis (PDS: Adult Health: Musculoskeletal Health: Mrs. Michaels)Mrs. Michaels with Rheumatoid Arthritis Comparison to ‘usual’ course Diagnostic tests Nursing diagnosis Therapies Medications used Exercise Joint Protection ResourcesResources on the Web

27. Systemic Lupus Erythematous (SLE) Lupus Chronic multisystem disease involving vascular and connective tissue Lupus Foundation

28. Etiology and Pathophysiology SLE Exact etiology unknown: genes (HLA), hormones, environment involved Formation auto-antibodies; immune complexes deposited Inflammatory response triggered by deposition of immune complexes (kidney, brain) Drug induced syndrome similar to SLE (Procan-SR, hydralazine, isonaiazid) Pneumonia, infections, *renal, CNS involvement!; Course of disease varies Mild Episodic Rapidly fatal

29. Manifestations/Complications of SLE Types: Discoid, SLEDiscoid, SLE Incidence: 1:2000 Women 1-9, child- bearing age, african americans Periods remission and exacerbation Stress Environmental factors Assessment Low grade fever Integumentary MS involvement CV Respiratory Urinary Renal failure Neurologic CNS GI Hematologic Endocrine Reproductive

30. Characteristic butterfly rash associated with SLE, especially discoid lupus erythematous Barry’s lupus

31. SLE characterized by periods of remission and exacerbation. Stimulated by sunlight, stress, pregnancy, infections like strep and some drugs. Some drugs like apresoline, pronestyl, dilantin, tetracycline, phenobarbital may cause a lupus-like reaction which disappears when drug is stopped.

32. Diagnostic Tests LE cell Ant-DNA ANA, titer Anti-DNA Complement fixation decreased ESR Other (and CBC, UA) Kidney biopsy Criteria to Dx. malar, discoid rash photosensitivity arthritis renal disorder immunological disorder DNA, ANA

33. Therapeutic Interventions/Management SLE Nursing diagnosis See RA Impaired skin integrity Ineffective protection Impaired health maintenance Goal: control inflammation Emotional support Life Planning Required Review Medications NSAIDS (Disease modifying agents) Antimalarial drugs Corticosteroids Immunsuppressive therapy Antineoplastic drugs such as Imuran, cytoxan, cyclosporine Avoid UV Reduce stress Monitor/manage to prevent complications

34. Case Study Clinical Background: 18 year old patient admitted with recent onset (3 months earlier) of malar rash and constitutional symptoms (weakness and malaise), now symptoms of renal failure. She reported having a 5-year history of Raynaud's phenomenon and arthralgia. Abnormal results of laboratory studies included the presence of ANA, anti- nDNA, anti-SS-A autoantibodies and a proteinuria of approximately 10 g/d. Renal biopsy revealed a Class IV lupus glomerulonephritis. What assessment data is priority: what additional date should you collect? What are the priority nursing problems? What are the priority interventions? What medications are typically used and why?medications

35. SclerodermaScleroderma (Systemic sclerosis) Definition: progressive sclerosis of skin and connective tissue; fibrous and vascular changes in skin, blood vessels, muscles, synovium, internal organs. become “hide bound” Immune-mediated disorder; genetic component

36. Scleroderma (Systemic sclerosis) Abnormal amounts of fibrous connective tissue deposited in skin, blood vissels, lungs, kidneys, other organs Can be systemic or localized (CREST) syndrome

37. CREST Syndrome Calcinosis Raynaud’s phenomena Esophageal hypomotility Sclerodactyl (skin changes of fingers) Telangiectasia (macula-like angioma of skin) More on CREST

38. CREST Syndrome & scleroderma Sclerodactyl (localized scleroderma of fingers) Raynaud’s disease with ischemia

39. Typical “hide- bound” face of person with scleroderma Tissue hardens; claw-like fingers; fibrosis

40. Manifestations & Complications (systemic) Female 4:1 Pain, stiffness, polyartheritis Nausea, vomiting Cough Hypertension Raynauld’s syndrome Skin atrophy, hyperpigmented

41. Scleroderma cont. Esophageal hypomotility leads to frequent reflux GI complaints common Lung-pleural thickening and pulmonary fibrosis Renal disease...leading cause of death!

42. Diagnosis/Treatment Scleroderma R/O autoimmune disease Radiological: pulmonary fibrosis, bone resorption, subcutaneous calcification, distal esophageal hypomotility ESR elevated CBC anemia Gammaglobulin lelels elevaed; RA present Skin biopsy to confirm What are the KEY components of care for the individual with Scleroderma?

43. Scleroderma: Patient Care Do’s Avoid cold Provide small, frequent feedings Protect fingers Sit upright post meals No fingersticks Daily oral hygiene Resources

44. Scleroderma: Patient Care Medications: based upon symptoms : Immunosuppressive agents & steroids & remitting agents Ca channels blockers & alpha-adrenergic blockers H2 receptor blockers ACE inhibitors Broad spectrum antibiotics

45. Ankylosing Spondylitis Definitions: chronic inflammatory polyarteritis of spine Affects mostly young men Associated with HLA-B27 antiget positive antigen (90%) Pathophysiology & Manifestations Like arthritis have inflammatory changes; erosion of cartilage, ossification of joint margins; scar tissue replaces Morning backache, flexion of spine, decreased chest expansion Diagnosis ESR elevation Positive HLA-B27 antigen Vertebral changes

46. Ankylosing Spondylitis Insidious onset Morning backache Inflammation of spine; later spine ossification Oh my back hurts!

47. Comparison of changes with ospeoporosis and Ankylosing spondylitis Identify a PRIORITY nursing concern related to ankylosing spondylitis

48. Management Ankylosing Spondilitis Do’s Maintain spine mobility Pain management Proper positioning Meds for pain, inflammation

49. Other Collagen Diseases Reiter’s Syndrome Reactive arthritis associated with enteric disease Lyme Disease Lyme Disease (mimics rheumatoid disease) Caused by spirochete, borrelia burgdorferi (tick) Inflammatory disorder 3 stages Initial rash (target) Disseminated (arthitic like symptoms) Late (neurologic symptoms) Diagnosis Culture (difficult) Antibody detection Treatment: antibiotics (amoxicillin, vibramycin, tetracycline, etc); NSAID) Prevention

50. Other Collagen Diseases Polymyositis Systemic connective tissue disorder characterized by inflamation of connective tissue and muscle fibers Autoimmune; affecting women 2:1 If muscle fiber inflammation is accompanied by skin lesion disease known as dermatomyositis Manifestations & complications Muscle pain, tenderness, rash; arthralgias; fatigue; fever and weight loss; Skeletal muscle weakness most prominent Dusky red rash over face Raynaud’s phenomenon Malignancy with dermatomyositis Diagnosis None specific Elevated CK

51. Other Collagen Diseases cont Reiters syndrome Self-limited disease of reactive arthritis such as shingles, venereal diseas, associated with HLA— B27 antigen Polyarteritis Nodosa Collegan; diffuse inflammation and necrosis of wall of small to medium sized arteries especially in muscles, kidneys, heart liver, GI and peripheral nerves like SLE Juvenile Rheumatoid Arthritis Juvenile Rheumatoid Arthritis