Musculoskeletal: Autoimmune; Inflammatory; Metabolic; Infectious Disorders

1. Musculoskeletal: Autoimmune; Inflammatory; Metabolic; Infectious Disorders Rheumatoid Arthritis; SLE; Paget’s Disease; Gout; Osteomyelitis

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

3. Pathophysiology: Rheumatoid Arthritis Cause unknown - Autoimmune Theory • Exposure to unidentified antigen • Triggers formation of an abnormal immunoglobulin G (IgG) • Autoantibodies called rheumatoid factor (RF) develop in response to IgG • ( Rheumatoid factor (RF) is diagnostic for RA) • IgG + RF = IgG/RF called immune complexes • Precipitates in synovial fluid & on articular cartilage • Triggers Inflammatory response • Cartilage and connective tissue primarily affected!

4. Pathophysiology: Rheumatoid Arthritis Inflammatory response • Immune complexes activate complement • Neutrophils release proteolytic enzymes = damage or thickening of synovial lining and damaged cartilage • T helper CD4 cells stimulate release of cytokines such as interleukin-1 and TNR (tumor necrosis factor alpha) cause chondrocytes to attack cartilage. Primary drivers of immune response in RA • Chronic inflammation = hypertrophy of synovial membranes, pannus formation, scarring, cartilage destruction, disrupts tendons, ligaments

5. Early Pannus Immune complexes enter joint Granulation, inflammation at synovial membranes Cartilage softens and begins process of destroying joint Joints changes with RA

6. RA • Mod advanced Pannus • joint cartilage disappears, underlying bone destroyed, joint surfaces collapse • Fibrous Ankylosis • Fibrous connective tissue replaces pannus = loss of joint • motion • Bony Ankylosis • Eventual tissue and joint calcification

7. Rheumatoid arthritis: assessment: manifestations and complications • Fatigue, weakness, pain • Joint deformity • Rheumatic nodules • Multi-system involvement

8. Bilateral & symmetrical PIP’s (hands) MTP’s (feet) Thumb instability Swan neck deformity Boutonniere deformity Tensynovitis Subcutaneous nodules Genu valgum (knock-knee) Pes plano (flat foot) Gneu valgus Hallux valgus Prominent metatarsal heads Hammer toes Joint Changes RA

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

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

11. Hammer toes

12. Subcutaneous nodules (disappear and appear without warning)

13. Manifestations of RA • Systemically ill • Hematologic • Pulmonary/CV • Neurologic • Ocular symptoms (Sjogren’s & Felty) • Skin • Musculoskeletal deformity, pain Pain! Pain! Pain!

14. Extra-articular Manifestations • Can affect almost all systems • Sjogren’s syndrome: 10-15% of patients. Decreased lacrimal and salivary gland secretions • Felty Syndrome: most common in pts with nodule forming type - Inflammatory eye disorders, splenomegaly, lymphadenopathy, pulmonary disease, blood dyscrasias

15. Diagnostic Tests RA + RF RF titer – 80% ESR elevated C-reactive protein – general indicators of inflammation CBC Synovial fluid – WBC’s present bone scans – early detection Swelling,inflammation X-rays only useful in late stages

16. How does Rheumatoid Arthritis Compare to Osteoarthritis? • Definition: *wear and tear, progressive, non-systemic, Degenerative Joint Disease (DJD) • Pathophysiology – loss of cartilage, exposed bone, bone spurs, inflammation

17. Comparison of RA and OA RA Cause unknown; auto-immune factor, genetic? Onset sudden with Remissions *Body parts affected: systemic, small joints, symmetrical Causes redness, warmth, swelling of joints Females, begins at any age; 2-3:1 ratio RF is positive, pain increases with movement OA Cause “wear and tear”, develops slowly Non-systemic, weight bearing joints Middle-aged, elderly, males 2-1 affected, Begins after 40 Does not cause malaise RF is negative, pain decreases with movement

18. Osteoarthritis (top slide only) Identify which joints are primarily affected with osteoarthritis. What factors contribute to the development of osteoarthritis?

19. 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

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

21. 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

22. Deformities with Osteoarthritis Carpometacarpocarpal joint of thumb with subluxation of the first MCP Genu varus Herberden’s nodes

23. 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 Osteoarthritis (review only)

24. Nursing Diagnosis Comfort Physical mobility Self image Goals Team Approach Pain management Exercise Surgery Synovectomy Joint fusion Athrodesis Joint replacement Arthroplasty Teaching Interventions RA

25. Medications RA • ASA & NSAIDS, Cox-2 inhibitors • Corticosteroids; low dose • DMARDs (diverse group) of remitting agents: including antimalarial (hydroxychloroquine: plaquenil) *loss of vision Penicillamine (empty stomach); bone marrow/kidney issues Gold (Auranofin) *dermatitis, blood dyscrasias, renal toxicity • Immunosuppressive agents as methotrexate and cyclosporine • Biologic response modifiers • Adalimumab (Humira)- Sub-Q binds with tumor necrosis factor to decrease inflammatory process – report infections STAT • Infliximab (Remicade)- IV similar to above

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

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

28. SystemicLupus Erythematous (SLE) Chronic multisystem disease involving vascular and connective tissue Lupus Foundation

29. Exact etiology unknown: genes (HLA), hormones, environment involved Formation auto-antibodies to DNA; immune complexes deposited Inflammatory response triggered by deposition of immune complexes in skin kidney, heart, joints, brain, lung, spleen, GI Drug induced syndrome similar to SLE (Procan-SR, Pronestal, (Apresoline) hydralazine, isonaiazid, siezure meds) Course of disease varies Mild Episodic Rapidly fatal Etiology and Pathophysiology SLE

30. Manifestations and Complications • Dermatologic – 50% have butterfly rash. Skin, nasal, oral lesions, hair loss. • Musculoskeletal – polyarthralgia, arthritis, swelling, pain, deformity • Cardiopulmonary – Tachypnea, cough, fibrosis of nodes = arrhythmias, accelerates CAD • Renal – 50% of patients within 1 year of onset. Protienurea to rapid glomerulonephritis • CNS – focal seizures, peripheral neuropathy, organic brain syndrome • Hematologic – antibodies form against blood cells • Infection – major cause of death

31. Incidence: 1:2000 Women 9:1, child-bearing age, african american, native american, asian 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 Manifestations/Complications of SLE

32. Characteristic butterfly rash associated with SLE, especially discoid lupus erythematous

33. 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.

34. LE cell prep; + in other rheumatoid diseases Anti-DNA- specific Anti nuclear antibody, titer Serum Complement levels decreased ESR - elevated CBC- leuko /lympho cytopenia, anemia UA –RBC’s, protien Kidney biopsy Criteria to Dx. malar, discoid rash; oral ulcers photosensitivity arthritis renal disorder immunological disorder DNA:ANA Diagnostic Tests

35. 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 light Reduce stress Monitor/manage to prevent complications Therapeutic Interventions/Management SLE

36. 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? Case Study What are the priority interventions? What medications are typically used and why?

37. Scleroderma(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

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

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

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

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

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

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

44. R/O autoimmune disease Radiological: pulmonary fibrosis, bone resorption, subcutaneous calcification, distal esophageal hypomotility ESR elevated CBC anemia Gammaglobulin levels elevated; RF in 30%& SCL-70 in 35% Skin biopsy to confirm What are the KEY components of care for the individual with Scleroderma? Diagnosis/Treatment Scleroderma

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

46. 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 • Capsaicin

47. Definitions: chronic inflammatory polyarthritis of spine Affects mostly young men Associated with HLA-B27 antigen 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 Ankylosing Spondylitis

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

49. Ankylosing spondylitis Identify a PRIORITY nursing concern related to ankylosing spondylitis

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