Connective Tissue Disorders Notes

Chapter 44: Connective Tissue Disorders

Osteoarthritis (Degenerative Joint Disease)

• Pathophysiology:
  • Degeneration of articular cartilage with hypertrophy of underlying/adjacent bone.
  • New bone growth is stimulated on exposed bone surfaces, causing bone spurs.
  • Types: Primary or Secondary.
• Risk Factors:
  • Increasing age (>50)
  • Trauma (exercise-induced)
  • Infection
  • Congenital deformities
  • Long-term corticosteroid therapy
  • Certain conditions (Diabetes Mellitus & obesity)
  • Family History
• Signs & Symptoms:
  • Assess by comparing sides.
  • Pain in affected joint with activity, relieved with rest (usually not bilateral).
  • Stiffness
  • Limitation in Range of Motion (ROM)
  • Swelling (not always)
  • Deformity or enlargement of joint
  • Bouchard nodes (proximal interphalangeal)
  • Heberden nodes (distal interphalangeal)
  • Crepitation
  • Difficulty with Activities of Daily Living (ADLs)
• Diagnostic Tests:
  • Health history
  • Radiographic studies
  • Arthroscopy
  • MRI
  • Synovial fluid aspiration
  • Labs (Rule out Rheumatoid Arthritis):
    • ESR = normal
    • RF assay = negative
    • Antinuclear antibody (ANA) = negative
    • Synovial fluid = few/no leukocytes
• Treatment:
  • Medications:
    • Acetaminophen
    • NSAIDs
    • COX-2 Inhibitors
    • Low-dose salicylates
    • Injections (joint): Hyaluronic acid or corticosteroid
  • Arthroscopic surgery or arthroplasty
  • Physical therapy
  • Education:
    • Exercise with periods of rest
    • Glucosamine not proven to help
    • Moist heat or occasional cold (for pain); heat is contraindicated with metal implants
  • Transcutaneous electrical nerve stimulator (TENS) - good for back pain
• Complications:
  • No specific complications.
• Patient Problems / Interventions:
  • Chronic Pain:
    • Administer pain medications and anti-inflammatories.
    • Heat/cold treatments.
    • Monitor and record response to treatments and report ineffective treatments.
  • Impaired Physical Mobility:
    • Regular exercise program to maintain muscle mass.
    • Weight loss if overweight.
    • Balance rest and activity to avoid becoming overly tired.
    • If hand affected, use clothes with Velcro closures, elastic waistband pants, & slip-on shoes.
    • If hip mobility is an issue, use grab bars in the bathroom, shower seat, raised toilet seat.
  • Ineffective Coping:
    • Allow patient time to discuss concerns about osteoarthritis and its effects on lifestyle.
  • Ineffective Self-Health Management:
    • Assess patient knowledge about the disease and correct any misconceptions.
    • Provide patient education.
• Patient Education (cont.):
  • To reduce joint strain and pain:
    • Maintain proper posture and body alignment.
    • Attain and maintain a healthy body weight.
    • Identify activities that take a long time to do or for which you need assistance.
    • Plan activities when help is available or when time is not a major concern and take periodic rest breaks.
    • Wear splints or support devices that rest or relieve painful, unstable joints.
    • Push or slide heavy objects rather than pull them.
    • Wear shoes with low heels and shock-absorbent soles to help decrease stress on the knee joints.
    • Avoid stairs whenever possible.
    • Sit rather than stand.
    • Use high stools when sitting at a counter.
    • Use higher chairs rather than low sofas.
    • When rising from a chair, inch to the edge of the seat and then use the armrests to push up from the seat.
    • Use large-diameter pencils and pens and use eating utensils with large, round handles.
  • Self-medication:
    • Analgesics are usually more effective if taken routinely as prescribed rather than only when you have pain.
    • Identify the side effects of your medications and notify your physician if they occur (provide a specific list).
  • Resource: Arthritis Foundation.
• Surgical Management:
  • Reserved for People with persistent pain and disability despite conservative treatment.
  • Arthroscopic surgery:
    • Remove loose bodies and repair defects.
  • Arthroplasty (Total joint replacement):
    • Indicated for intractable pain that disrupts sleep and daily activities.
• Arthroplasty Nursing Care:
  • Vital Signs, include pain
  • Level of consciousness
  • Intake & Output
  • Respiratory status
  • Neurovascular status
  • Urinary function
  • Bowel elimination
  • Surgical incision
  • Preventing complications of immobility
• Hip Replacement Patient Education:
  • Do not flex hip more than 90 degrees.
  • Avoid flexion, adduction, and internal rotation.
  • Place a large pillow between the patient's legs when turning the patient, when the patient is supine, and when the patient is lying on the unaffected side.
  • Advise the patient not to cross the legs or feet and not to put on his or her own shoes, socks, or stockings for 6 weeks to 2 months, as directed by the surgeon.
  • Apply leg abductor pillow as ordered.
  • Do not turn onto the operative side unless specifically ordered to do so by the surgeon.
  • Have the patient sit in a chair that has arms to facilitate rising without extreme hip flexion.
  • Arrange for a raised toilet seat to allow toileting without extreme hip flexion.
  • Permit weight-bearing as ordered, depending on type of prosthesis used and whether cement was used.
  • Encourage the patient to exercise the unaffected extremities to maintain strength.
• Knee Replacement Patient Education:
  • Encourage quadriceps-setting exercises and straight leg lifts beginning on postoperative day 2 to 5, as ordered.
  • Use a continuous passive motion (CPM) machine as ordered; check the alignment and settings.
  • Monitor weight-bearing with walker or crutches as ordered.

Rheumatoid Arthritis (RA)

• Pathophysiology:
  • Chronic, progressive inflammatory disease.
  • Inflammation of the synovial tissue.
  • Synovium thickens; fluid accumulates in the joint space.
  • Vascular granulation tissue (pannus) forms in the joint capsule and breaks down cartilage and bone.
  • Fibrous tissue invades pannus, converting it first to rigid scar tissue and finally to bony tissue.
  • These changes result in ankylosis.
• Signs and symptoms:
  • Pain in affected joints aggravated by movement.
  • Morning stiffness lasting more than 1 hour (unlikely osteoarthritis: relieved within minutes).
  • Weakness, easy fatigability, anorexia, weight loss, muscle aches and tenderness, and warmth and swelling of the affected joints.
  • Joint changes are usually symmetric.
  • Rheumatoid nodules (subcutaneous, over bony prominences).
  • Any organ may be affected.
    • Inflammation in tissues of the heart, lungs, kidneys, eyes, blood vessels (vasculitis).
  • Clusters of symptoms:
    • Sjögren, Felty, or Caplan syndromes.
• Medical diagnosis:
  • Health history and physical examination.
  • Laboratory studies:
    • RF (rheumatoid factor), ESR (erythrocyte sedimentation rate), and CRP (C-reactive protein).
  • Synovial fluid:
    • Viscosity, WBCs, glucose, and mucin clot test.
  • Imaging:
    • MRI, bone scans, and dual-energy x-ray absorptiometry (DEXA) scans.
• Medical treatment:
  • Drug therapy (focused on inflammation/symptom relief):
    • Combinations of NSAIDs, glucocorticoids, DMARDs (including BRMs), and JAK inhibitors.
    • DMARDs slow progression of the disease.
  • Joint injections of corticosteroids.
  • Supportive treatment.
  • Surgery:
    • Arthroplasty, synovectomy, tenosynovectomy, and arthrodesis.
• Assessment:
  • Pain, joint swelling, tenderness, joint deformities and limitation of movement, fatigue, and decreased ability to perform activities of daily living.
• Interventions:
  • Pain
  • Impaired mobility
  • Inadequate coping
  • Decreased socialization
  • Inability to Manage Treatment Program
• Patent education:
  • Take your medications exactly as prescribed and notify your physician of any adverse effects (provide drug names, dosage, schedule, side and adverse effects).
  • Keep follow-up appointments; the effects of many drugs need to be monitored with periodic blood studies.
  • Balance activity and rest.
  • Avoid prolonged bed rest, which can lead to further loss of function.
  • Use assistive devices as needed to maintain safe mobility.
  • Support your joints in functional positions to reduce the risk of contractures.
  • Do as much as you can for yourself but do not hesitate to ask for help with difficult tasks.
  • Avoid heavy lifting that may cause strain on your joints.
  • Use artificial tears, vaginal moisturizer, etc. for dryness (Sjögren syndrome)

Osteoporosis

• Pathophysiology:
  • Bone constantly formed and absorbed
  • Until adolescence, bone formation exceeds bone absorption so that bones grow and strengthen
  • Around age 30, bone absorption surpasses formation
  • Loss of trabecular bone, innermost layer, occurs first
  • Loss of cortical bone, hard outer shell, begins later
  • Begins earlier and progresses faster in women than in men
  • Result is loss of bone mass
• Risk factors:
  • Older women who have small frames, who are white or of northern European heritage, and who have fair skin and blond or red hair
  • Estrogen deficiency; physical inactivity; low body weight; inadequate calcium, protein, or vitamin D intake; corticosteroid therapy over more than 6 months; and excessive use of cigarettes, caffeine, and alcohol
  • Disorders associated include:
    • Cushing disease, hyperparathyroidism, hypogonadism, cirrhosis, leukemia, and diabetes mellitus
• Signs and symptoms:
  • Back pain, fractures, loss of height due to vertebral compression, and kyphosis
  • Bone deterioration in the jaw can cause dentures to fit poorly
  • Collapsed vertebrae can cause chronic pain
• Medical diagnosis:
  • Dual-energy x-ray absorptiometry (DEXA) scans
  • Radiographs
  • Blood studies
    • Rule out secondary cause (Calcium, Vitamin D, etc.)
• Medical treatment:
  • Calcium supplementation & Estrogen replacement (postmenopausal)
  • Bisphosphonates (Fosamax, Boniva, etc.)
  • Selective estrogen receptor modulators (SERMs) (Evista. Etc.)
  • Regular exercise
  • Percutaneous vertebroplasty (fractures)
• Assessment:
  • Diet, calcium intake, and exercise plan
  • Note whether the patient is menopausal or has had an oophorectomy
  • Compare height with previous measurements
  • Posture; note the presence and degree of deformity
• Interventions:
  • Risk for trauma
  • Chronic pain
  • Ineffective self-health management
• Patient Teaching:
  • Women need 1000 mg of calcium daily before menopause and while on hormone replacements.
  • Women need 1200 mg of calcium daily after 50 years of age.
  • Men require 1000 mg every day up to 71 years of age, when the amount should be increased to 1200 mg per day.
  • Approximately 300 mg of calcium are found in each of the following: 1 cup of milk, 1 cup of yogurt, 1 oz of Swiss cheese.
  • Nonfat and skim milk have as much calcium as whole milk.
  • Increase your fluid intake if you are taking calcium supplements unless advised not to do so by your health care provider.
  • You need at least 600 IU of vitamin D every day up to 71 years of age, when your physician may advise an increase.
  • Taking excessive amounts of calcium and/or vitamin D can cause kidney stones.
  • Limit your intake of alcohol and caffeine.
  • If Fosamax is prescribed, take it in the morning with a full glass of water on an empty stomach; sit or stand for 30 minutes after taking it.
  • Regular weight-bearing exercise helps to maintain bone strength.
  • Avoid activities that might lead to falls and fractures.

Gout

• Pathophysiology:
  • Characterized by hyperuricemia (i.e., excess uric acid in the blood) and is related either to an excessive rate of uric acid production or to decreased uric acid excretion by the kidneys.
• Risk Factors:
  • Men > Women (40-50 yo)
  • Obesity
  • High intake of foods high in purines:
    • ETOH
    • Red meat
    • Organ meats
    • Seafood
    • Fructose
• Signs & Symptoms:
  • Asymptomatic hyperuricemia
    • Blood uric acid elevated -> Usually do not progress
  • Acute gouty arthritis
    • Great toe commonly affected
    • Abrupt onset, usually at night
    • Severe, crushing pain and cannot even bear the light touch of sheets
    • Symptoms usually disappear within a couple of days
  • Chronic tophaceous gout
    • Advanced gout with Tophi: Visible sodium urate crystals
• Diagnostic Tests:
  • History and physical exam
  • Urate crystals in synovial fluid
  • Urinary uric acid
  • Blood uric acid and creatinine
  • Imaging
    • Ultrasound
    • Joint x-ray (r/o other causes)
• Treatment:
  • Asymptomatic: no treatment needed
  • Acute gouty arthritis
    • Prednisone inject (if single joint)
    • NSAID alone or with Colchicine (treat inflammation)
    • Subsequent attacks:
      • Indomethacin, Corticosteroids, Corticotrophin
  • Chronic Management:
    • Allopurinol/febuxostat
    • Probenecid
    • Pigloticase
    • Avoid foods high in purines; limit animal-derived foods, ETOH, and fructose
• Complications:
  • Kidney stones
    • S/S:
      • Flank, lower abdominal, or genital pain
      • Hematuria
      • Decreased urine output
• Pain:
  • Nursing care to decrease discomfort includes elevating the affected extremity, administering prescribed medications, encouraging rest, and avoiding pressure on the area.
  • Assess effectiveness of medications
  • Because even bed sheets may cause pain, a foot cradle/foot board should be used.
  • Hot or cold packs may be ordered.
  • Splints or bandages may be used to immobilize the affected joint
• Impaired mobility:
  • Bed rest is usually recommended during the acute period.
  • Provide assistance with ADL as needed.
• Impaired fluid balance/Altered urinary elimination:
  • Uric acid stones may form and can obstruct urine flow from the kidney, causing renal damage. To prevent this complication, advise the patient to drink eight to sixteen 8-oz cups of fluid daily unless contraindicated.
• Ineffective self-health management:
  • Instruct the patient in measures to prevent or decrease future attacks.
• Patient education:
  • Your diet should limit animal protein, alcohol, and fructose. Avoid foods that are very high in purines.
  • Maintain a fluid intake of eight to sixteen 8-oz glasses daily to reduce the risk of uric acid stone formation in the urinary tract.
  • Take your drugs as prescribed (provide specific dosage, schedule, list of side effects).
  • To prevent severe attacks, report early joint or urinary symptoms to the physician. Severe attacks may be averted if treatment is begun soon after symptoms develop.
  • If you are overweight, weight loss may help by reducing stress on joints.

Progressive Systemic Sclerosis

• Pathophysiology:
  • Primary vessel injury/dysfunction of immune system as a result of environmental factors and genetic susceptibility
  • Manifestations: from inflammation to degeneration of tissues, that results in decreased elasticity, stenosis, and occlusion of vessels
• Signs and symptoms:
  • Raynaud phenomenon, symmetric painless swelling or thickening of the skin, taut and shiny skin, morning stiffness, frequent reflux of gastric acid, difficulty swallowing, weight loss, dyspnea, pericarditis, and renal insufficiency
• Limited symptoms (CREST):
  • Calcinosis - calcium deposits in the skin
  • Raynaud's phenomenon - spasm of blood vessels in response to cold or stress
  • Esophageal dysfunction - acid reflux and decrease in motility of the esophagus
  • Sclerodactyly - thickening and tightening of the skin on the fingers and hands
  • Telangiectasias - dilation of capillaries causing red marks on the surface of the skin
• Medical diagnosis:
  • History and physical examination may lead the provider to suspect fibrotic changes typical of PSS in the skin, lungs, heart, or esophagus
  • Positive ANA assay result, elevated ESR, and increased serum muscle enzyme levels
• Medical treatment:
  • No cure
  • High doses of steroids or other immunosuppressants may bring about remission
  • Physical therapy
  • d-Penicillamine (Antifibrotic drug)
  • Corticosteroid (Short term)
  • Antihypertensives (Control hypertensive crisis & sclerodermal renal crisis)
  • Management of Raynaud phenomenon (vasospasms)
    • Avoid smoking cold environmental temperature, and vasoconstricting drugs
    • Angiotension II receptor blockers
• Assessment:
  • Pain and stiffness in the fingers; color changes or lesions in the fingertips; intolerance for cold
  • Signs and symptoms suggestive of cardiovascular, respiratory, renal, and gastrointestinal problems
  • Skin rash, loss of wrinkles on the face, limitations of joint range of motion, muscle weakness, and dry mucous membranes
  • Examine the hands for contractures of the fingers and for color changes or lesions on the fingertips
  • Palpate the fingers to determine warmth
• Interventions:
  • Inadequate Tissue Integrity
  • Impaired Ability to Carry Out ADLs (Bathing, Feeding, Toileting)
  • Pain
  • Decreased Socialization
  • Inadequate Nutrition
  • Potential for Fluid and Electrolyte Imbalance
  • Decreased ability to manage treatment program
• Patient Teaching:
  • To prevent vasospasm, keep your hands warm and reduce stress and exhaustion.
  • Take drugs as prescribed and report adverse effects to your physician (provide specific information about drug names, dosage, schedule, and adverse effects).
  • Esophageal reflux can be managed with drug therapy; relaxing meals; avoiding spicy foods, caffeine, and alcohol; and maintaining an upright position for 1 to 2 hours after eating.

Dermatomyositis/Polymyositis

• Pathophysiology:
  • Polymyositis: infiltration of inflammatory cells, causing destruction of muscle fibers
  • Inflammation of tissues surrounding blood vessels is an outstanding pathologic feature of the disease
  • Condition is sometimes associated with malignancy
• Signs and symptoms:
  • Polymyositis: muscle weakness, Raynaud phenomenon, and joint pain and inflammation
  • Dermatomyositis: periorbital edema as well
• Medical diagnosis:
  • Proximal muscle weakness, a muscle biopsy positive for muscle degeneration, elevated muscle enzymes, and myopathic electromyographic changes
• Medical treatment:
  • Drug therapy
    • High-dose glucocorticoids, such as prednisone, and chemotherapeutic agents, such as methotrexate
  • Supportive treatment: balancing rest and exercise to prevent contractures

Nutrition Considerations for Connective Tissue Disorders

• A daily intake of 1000 to 1200 mg/day of calcium and 600 IU/day of vitamin D is important for the prevention of osteoporosis.
• Eight ounces of milk (whole, low fat, or skim) provides 300 mg of calcium.
• A well-balanced diet, including foods high in vitamin E and zinc, is recommended for persons with rheumatoid arthritis (RA).
• A weight loss program for persons with arthritis reduces stress on weight-bearing joints.
• Patients with gout are advised to limit intake of animal foods, alcohol, and fructose.
• The patient with esophageal involvement of systemic sclerosis (SSc) needs small, frequent meals and limited spicy foods, alcohol, and caffeine.

Other Connective Tissue Disorders

• Bursitis
• Carpal tunnel syndrome
• Ankylosing spondylitis
• Polymyalgia rheumatica
• Reiter syndrome
• Behçet syndrome
• Sjögren syndrome
• Periarteritis nodosa