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Autoimmune Disorders That Affect the Musculoskeletal System Rheumatoid Arthritis Rheumatoid Arthritis (RA) Chronic, systemic autoimmune disease Inflammation of connective tissue in diarthrodial (synovial) joints Periods of remission and exacerbation Frequently accompanied by extra-articular manifestations Incidence Occurs globally, affecting all ethnic groups Occurs at any time of life Incidence increases with age Peaks between 30s and 50s Nearly 2.1 million Americans affected Women have incidences three times higher than men Etiology Cause of RA is unknown No infectious agent found Two etiologies Autoimmune etiology • Most widely accepted Genetic factor etiology Pathophysiology Chronic inflammation of the joints leads to: Scar tissue (pannus) & joint cartilage destruction Joint laxity, subluxation (dislocation), & contracture Pathophysiology Fig. 65-3 As the synovitis expands inside and outside of the joint, it can damage the bone and cartilage of the joint and the surrounding tissues, such as ligaments, tendons, nerves, and blood vessels. With articular cartilage destruction, vascular granulation tissue grows across the surface of the cartilage (pannas) with loss of cartilage beneath the expanding pannas Etiology and Pathophysiology of Rheumatoid Arthritis Inflammatory pannus causes destruction of bone. This leads to joint deformities. Pathophysiology If unarrested, RA progresses in 4 stages Stage 1: Early • No destructive changes on x-ray, possible x-ray evidence of osteoporosis Stage 2: Moderate • X-ray evidence of osteoporosis, with or without slight bone or cartilage destruction • No joint deformities, adjacent muscle atrophy, possibly presence of extra-articular soft tissue lesions Etiology and Pathophysiology RA progresses in four stages (cont'd) Stage 3: Severe • X-ray evidence of cartilage and bone destruction in addition to osteoporosis; joint deformity; extensive muscle atrophy; possible presence of extraarticular soft tissue lesions Stage 4: Terminal • Fibrous or bony ankylosis, stage III criteria Clinical Manifestations Onset Onset is typically insidious Nonspecific manifestations may precede onset of arthritic complaints Some report a history of precipitating events Clinical Manifestations Joints Specific articular involvement Symptoms occur symmetrically Frequently affect small joints of hands and feet Larger peripheral joints may also be involved Typical Deformities of Rheumatoid Arthritis Fig. 65-4 Clinical Manifestations Joints Patient experiences joint stiffness after periods of __________ Morning stiffness may last from 60 minutes to several hours or more MCP and PIP joints typically swollen Fingers may become spindle shaped from synovial hypertrophy and thickening of joint capsule Clinical Manifestations Joints Joints become tender, painful, and warm Joint pain Increases with ______ Varies in intensity May not be proportional to degree of inflammation Tenosynovitis frequently affects extensor and flexor tendons near wrists RA progresses Extraarticular Manifestations of Rheumatoid Arthritis Fig. 65-5 Clinical Manifestations Extraarticular Manifestations Three most common Rheumatoid nodules develop in up to 25% of all patients with RA Those affected usually have high RF titers Clinical Manifestations Extraarticular Manifestations Sjögren’s syndrome Seen in 10% to 15% of patients with RA Can occur as a disease by itself or in conjunction with other arthritic disorders • RA and systemic lupus erythematosus (SLE) Complaints of burning, gritty, itchy eyes Decreased tearing, photosensitivity Clinical Manifestations Extraarticular Manifestations Felty’s syndrome Most commonly in patients with severe, nodule-forming RA Characterized by • • • • • Inflammatory eye disorder Splenomegaly Lymphadenopathy Pulmonary disease Blood dyscrasias Complications Joint destruction begins as early as first year of disease without treatment Flexion contractures and hand deformities Cause diminished grasp strength Affect patient’s ability to perform self-care tasks Cataract development and loss of vision can result from scleral nodules Complications Rheumatoid nodules On the skin can ulcerate, similar to pressure ulcers On vocal cords leads to progressive hoarseness In vertebral bodies can cause bone destruction Complications Cardiopulmonary effects may occur later in RA Pleurisy, pleural effusion, pericarditis, pericardial effusion, cardiomyopathy Carpal tunnel syndrome can result from swelling of synovial membrane Diagnostic Studies RA is defined as having at least 4 of the following seven criteria. Following must be present for at least 6 wks: Morning stiffness that lasts ≥1 hour Swelling in three or more joints Swelling in hand joints Symmetrical joint swelling Erosions or decalcification seen on hand x-rays 6. Rheumatoid nodules 7. Presence of serum RF 1. 2. 3. 4. 5. Diagnostic Studies Accurate diagnosis is essential to initiation of appropriate treatment and prevention of unnecessary disability Diagnosis is often made Based on history and physical findings Some laboratory tests are useful for confirmation and to monitor disease progression Diagnostic Studies Positive RF Titers rise during active disease Antinuclear antibody (ANA) titers Indicators of active inflammation ESR C-reactive protein (CRP) Diagnostic Testing Blood Studies Rheumatoid factor (RF) Erythrocyte sedimentation rate (ESR) C-reactive protein (CRP) Antinuclear antibodies (ANA) titers are seen in some Synovial fluid analysis Straw-colored fluid with fibrin flecks WBC is elevated to >25,000/μl X-rays Will not diagnosis – only show bone changes Collaborative Care Care begins with a comprehensive program of education and drug therapy Education of drug therapy Patient and family educated about disease process and home management strategies NSAIDs are prescribed to provide comfort Collaborative Care Physical therapy helps maintain joint motion and muscle strength Occupational therapy develops extremity function and encourages joint protection Drug Therapy Drugs remain cornerstone of treatment DMARDs can lessen permanent effects of RA Choice of drug is based on Treatment and Nursing Care for Rheumatoid Arthritis See Table 65-3 for meds used for arthritis p. 1698-1700. Salicylates NSAIDs Antibiotics Topical analgesics Corticosteroids DMARDs Gold compounds Antimalarials Immunosupressants Biologic/Targeted therapy Drug Therapy DMARD Many of the drugs used to treat RA are expensive Methotrexate (Rheumatrex) is drug of choice Rapid antiinflammatory effect decreases clinical symptoms in days to weeks Inexpensive Lower toxicity compared to other drugs Drug Therapy Effective DMARDs for mild to moderate disease Sulfasalazine (Azulfidine) Antimalarial drug hydroxychloroquine Leflunomide (Arava) is a newer synthetic DMARD that blocks immune cell overproduction Drug Therapy Biologic/targeted drug therapies can also slow disease progression in RA Can be used in patients with moderate to severe disease who have not responded to DMARDs or in combination therapy with an established DMARD Drug Therapy Corticosteroid therapy can aid in symptom control Intraarticular injections may relieve pain and inflammation associated with flare-ups • Long-term use should not be a mainstay Risk osteoporosis, avascular necrosis Low-dose prednisone for a limited time to decrease disease activity until DMARD effect is seen Drug Therapy Various NSAIDs and salicylates to treat arthritis pain and inflammation Aspirin is often used in high dosages of 4 to 6 g/day (10 to 18 tablets) NSAIDs have antiinflammatory, analgesic, and antipyretic properties Drug Therapy NSAIDs Do not alter natural history of RA Full effectiveness may take 2 to 3 weeks • Some relief may be noted within days May be used when patient cannot tolerate high doses of aspirin Relieve Pain NSAIDs DMARDs Non-Pharmacological Heat or Cold applications Rest Relaxation techniques Nursing Implementation Acute Intervention Usually treated on an outpatient basis Hospitalization may be necessary for patients with extraarticular complications or advancing disease Reconstructive surgery for disabling deformities Nursing intervention begins with a careful physical assessment Nursing Management Assessment Nurse must also Evaluate psychosocial needs and environmental concerns After problem identification, coordinate a carefully planned program for rehabilitation and education for interdisciplinary health care team Nursing Management Problems Chronic pain Impaired physical mobility Activity intolerance Self-care deficit Ineffective therapeutic regimen management Disturbed body image Nursing Management Planning Overall goals Satisfactory pain relief Minimal loss of functional ability of affected joints Perform self-care Participate in planning and carrying out therapeutic regimen Maintain a positive self-image Nursing Management Interventions Discuss with patient Many patients take several different drugs so the nurse must make the drug regimen as understandable as possible Careful attention to timing is critical to Nursing Management Interventions Nonpharmacologic relief of pain Therapeutic heat and cold Rest Relaxation techniques Joint protection Biofeedback Transcutaneous electrical stimulation Hypnosis Nursing Management Interventions Lightweight splints may be prescribed to rest an inflamed joint and prevent deformity Should be removed regularly to perform skin care and ROM exercises Should be reapplied as prescribed Occupational therapist may help identify additional self-help devices to assist in activities of daily living Nursing Management Interventions Morning care and procedures should be planned around morning stiffness To relieve joint stiffness and increase comfort Ambulatory and Home Care Rest Alternate scheduled rest periods with activity throughout day Helps relieve pain and fatigue Amount of rest varies Total bed rest Rarely necessary Should be avoided to prevent stiffness and immobility Ambulatory and Home Care Rest Good body alignment while resting can be maintained through use of a firm mattress or bed board Encourage positions of extension Avoid flexion positions Splints and casts can help maintain proper alignment and promote rest Ambulatory and Home Care Heat and Cold Therapy Help relieve pain, stiffness, and muscle spasm Ice Superficial Moist heat heat sources Ambulatory and Home Care Exercise Inadequate joint movement can result in progressive joint immobility and muscle weakness Overaggressive exercise can result in increased pain, inflammation, and joint damage Gentle ROM exercises are usually done daily to keep joints functional Ambulatory and Home Care Psychologic Support Patient’s value system and perception of disease must be considered Patient is constantly challenged by problems of Limited function and fatigue Loss of self-esteem Altered body image Fear of disability or deformity Gout Gout Deposits of sodium urate crystals in articular, periarticular, and subcutaneous tissues May be primary or secondary Primary – hereditary error of purine metabolism Secondary – drugs that inhibit uric acid excretion or another acquired disorder Incidence and Risk Factors Primary Affects Risk gout accounts for 90% of cases primarily middle aged men factors: obesity, HTN, thiazide diuretics, excess alcohol use Pathophysiology Uric acid is end product of purine metabolism and is excreted by the kidneys Causes Diet of hyperuricemia high in purines will not cause gout, but may trigger an attack in a susceptible person Clinical Manifestations Gouty arthritis in one or more joints (but less than four) Great toe joint most common first manifestation; other joints may be the foot, ankle, knee, or wrist Joints are tender & cyanotic May be precipitated by trauma, surgery, alcohol ingestion, or infection Clinical Manifestations Onset usually nocturnal, with sudden swelling and excruciating pain May have low grade fever Usually subsides within 2-10 days Joints are normal, with no symptoms between attacks Complications Joint deformity Osteoarthritis Tophi may produce draining sinuses that may become infected Renal stones, pyelonephritis, obstructive renal disease Diagnosis History & physical examination Family history of gout Diagnostic studies Diagnostic Studies Serum uric acid levels May be caused by other factors WBC elevated during acute attack ESR 24 hour urine uric acid levels Synovial fluid aspiration contains uric acid crystals Seldom necessary X-rays appear normal in early stages; tophi appear as eroded areas of bone Collaborative Care Acute attack Colchicine produces dramatic antiiflammatory effects with relief within 24-48 hours NSAIDs for additional pain relief Corticosteroids (po or intraarticular) Adrenocorticotropic hormone (ACTH) Joint aspiration to decompress Collaborative Care Prevention of acute attacks Colchicine combined with: • allopurinol (Zyloprim, Alloprim) – blocks production of uric acid • probenecid (Benemid), sulfinpyrazone (Anturane) – inhibit tubular reabsorption of uric acid • febuxostat (Uloric) – inhibits xanthine oxidase, recently shown to reduce serum uric acid levels Collaborative Care Uricosuric Agents Probenecid (Benemid) Precautions Sulfinprazone (anturan) block resorption uric acid High fluid intake, alkaline urine Not effective if creatinine clearance is elevated Cozaar Angiotension II receptor angonist • Monitoring Force Fluids while on these medications!! Collaborative Care Dietary measures Weight reduction Avoidance of alcohol Avoidance of foods high in purines • High: Sardines, anchovies, herring, mussels, liver, kidney, goose, venison, meat soups, sweetbreads, beer & wine • Moderate: Chicken, salmon, crab, veal, mutton, bacon, pork, beef, ham Treatment and Nursing Care Bedrest and position for comfort Joint immobilization and protect joint from pressure Local application of heat or cold Assess for complications Formation of kidney stones Hypertriglyceridemia Hypertension Collaborative Care Prevention of renal stones Increase fluid intake to maintain adequate urine output Allopurinol ACE inhibitor losartin (Cozaar) – promotes urate diuresis Nursing Care Acute gouty arthritis – pain control Gentle, supportive care of affected joints Immobilize and rest affected joints – bed rest or NWB Cradle or footboard to prevent pressure from bedcovers Monitor ROM and degree of pain Nursing Care Patient/Family teaching Gout is a chronic disease Drug teaching Need to monitor serum uric acid levels Precipitating factors Systemic Lupus Erythematosus (SLE) Chronic multisystem disease involving vascular and connective tissue Incidence SLE affects 2 to 8 persons per 100,000 in United States Most cases occur in women of childbearing years African, Asian, Hispanic, and Native Americans three times more likely to develop than Whites Etiology Etiology is unknown Most probable causes Genetic influence Hormones Environmental factors Certain medications SLE Pathophysiology Chronic multisystem inflammatory disease Associated with abnormalities of immune system Results from interactions among genetic, hormonal, environmental, and immunologic factors Because the antibodies and accompanying cells of inflammation can affect tissues anywhere in the body, lupus can affect a variety of areas Systemic Lupus Erythematosus A variable disease Chronic Unpredictable Characterized by exacerbations & remissions Clinical Manifestations Ranges from a relatively mild disorder to rapidly progressing, affecting many body systems Most commonly affects the skin/muscles, lining of lungs, heart, nervous tissue, and kidneys Systematic Lupus Erythematosus Affects the Skin Joints Serous membranes Renal system Hematologic system Neurologic system Clinical Manifestations Dermatologic Cutaneous vascular lesions Butterfly rash Oral/nasopharyngeal ulcers Alopecia Clinical Manifestations Musculoskeletal Polyarthralgia with morning stiffness Arthritis • Swan neck fingers • Ulnar deviation • Subluxation with hyperlaxity of joints Clinical Manifestations Cardiopulmonary Tachypnea Pleurisy Dysrhythmias Accelerated CAD Pericarditis Clinical Manifestations Renal Lupus nephritis • Ranging from mild proteinuria to glomerulonephritis • Primary goal in treatment is slowing the progression Clinical Manifestations Nervous system Generalized/focal seizures Peripheral neuropathy Cognitive dysfunction • Disorientation • Memory deficits • Psychiatric symptoms Clinical Manifestations Hematologic Formation of antibodies against blood cells Anemia Leukopenia Thrombocytopenia Coagulopathy Anti-phospholipid antibody syndrome Clinical Manifestations Infection Susceptibility to infections Fever should be considered serious Infections such as pneumonia are a common cause of _____ Diagnostic Studies No specific test SLE is diagnosed primarily on criteria relating to patient history, physical examination, and laboratory findings Diagnosis Autoantibody Anti-DNA- specific Anti- Smith antibody (Anti-Sm) Anti nuclear antibody (ANA) Inflammatory Testing: Activity: ESR C-reactive protein These are not diagnostic for SLE, but help in the diagnosis Diagnostic Tests CBC UA X-rays Chest ECG x-ray Collaborative Care Prognosis is improved with Earlier diagnosis Earlier and better treatment regimens Careful monitoring for organ involvement Collaborative Care Drug therapy NSAIDs Antimalarial drugs Steroid-sparing drugs Corticosteroids Immunosuppressive drugs Treatment and Nursing Care What is the single most important medication used in the treatment of SLE? Other Medications: Anitmalarial Immunosuppressive agents Nursing Management Nursing Assessment Assess patient’s physical, psychologic, and sociocultural problems with long-term management of SLE Assess pain and fatigue daily Obtain subjective and objective data Educate and counsel on expected issues Nursing Management Nursing Diagnoses Fatigue Acute pain Impaired skin integrity Ineffective therapeutic regimen management Body image disturbance Nursing Management Nursing Implementation Health promotion Prevention of SLE is not possible Promote early diagnosis and treatment Relieve pain and discomfort and fatigue Application of heat and cold Encourage to alternate rest and activity Support devices – braces, splints, firm mattress Analgesics and anti-inflammatory medications Nursing Care Maintain Skin Integrity Apply topical antiinflammatory Avoid direct sunlight Increase Mobility ROM Assistive devices Nursing Management Nursing Implementation Acute intervention During exacerbation, patient will become abruptly, dramatically ill Record severity of symptoms and response to therapy Observe for • • • • • Fever pattern Joint inflammation Limitation of motion Location and degree of discomfort Fatigability Nursing Management Nursing Implementation Acute intervention (cont’d) Monitor ______ & _ _ _ Collect 24-hour urine sample Assess neurological status Explain nature of disease Provide support Nursing Management Nursing Implementation Ambulatory and home care Reiterate that adherence to treatment does not necessarily halt progression Minimize exposure to precipitating factors – Teach energy conservation and relaxation exercises For joint problems, all the teaching for RA related to joint protection, ROM, and positioning to prevent contractures Treatment and Nursing Care Facilitate self care Improve body image Provide adaptive equipment for eating, bathing, toileting, dressing Allow patient extra time to complete care Encourage patient to verbalize feelings, perceptions, and fears Monitor for complications Assess for serious adverse effects of medications used in treatment Avoid ____________ ____ ____ _______ Nursing Management Nursing Implementation Lupus and pregnancy Infertility SLE is associated with complications of pregnancy Pregnancy & post partum Women with serious SLE Nursing Management Nursing Implementation Psychosocial issues Counsel patient and family that SLE has good prognosis Physical effects can lead to isolation, selfesteem, and body image disturbances Assist patient in developing goals Nursing Management Evaluation Expected outcomes Completion of priority activities Verbalization of having more energy Expression of satisfaction with pain relief measures Performance of activities of daily living without pain Limitation of direct exposure to sun No open skin lesions Expression of satisfaction with activity level Pacing of activities to match level of tolerance Expression of confidence in ability to manage SLE over time and in home environment