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1Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Chapter 41
Connective Tissue Disorders
2Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Learning Objectives
• Define connective tissue.• Describe the function of connective tissue.• Describe the characteristics and prevalence of connective tissue diseases.• Describe the diagnostic tests and procedures used for
assessing connective tissue diseases.• Discuss the drugs used to treat connective tissue diseases.• Describe the pathophysiology and treatment of osteoarthritis (degenerative
joint disease), rheumatoid arthritis, osteoporosis, gout, progressive systemic sclerosis, polymyositis, bursitis, carpal tunnel syndrome, ankylosing spondylitis, polymyalgia rheumatica, Reiter’s syndrome, Behçet’s syndrome, and Sjögren’s syndrome.
• Identify the data to be collected in the nursing assessmentof a patient with a connective tissue disorder.
• Assist in developing a nursing care plan for a patientwhose life has been affected by a connective tissue disease.
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Anatomy and Physiology of Connective Tissues
• Bind structures together, providing support for individual organs and a framework for the body
• Store fat, transport substances, provide protection, and play a role in repair of damaged tissue
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Anatomy and Physiology of Connective Tissues
• Types of connective tissue• Loose (areolar, adipose, reticular) • Dense (tendons, fascia, dermis, gastrointestinal
tract submucosa, fibrous joint capsules) • Elastic (aortic walls, vocal cords, parts of trachea
and bronchi, some ligaments) • Hematopoietic (blood) • Strong supportive (cartilage, bone, ligaments)
5Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Anatomy and Physiology of Connective Tissues
• Bone • Hard tissue: makes up most of skeletal system • Functions: support, protection, movement, storage
of calcium and other ions, and manufacture of blood cells
6Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Anatomy and Physiology of Connective Tissues
• Cartilage• Specialized fibrous connective tissue • Provides firm but flexible support for the embryonic
skeleton and part of the adult skeleton • Cartilage cells are called chondrocytes
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Anatomy and Physiology of Connective Tissues
• Ligaments • Strong and flexible fibrous bands of connective
tissue that connect bones and cartilage and support muscles
• Yellow ligaments, located in the vertebral column, are elastic and allow for stretching
• White ligaments, found in the knee, do not stretch but provide stability
8Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Anatomy and Physiology of Connective Tissues
• Tendons• Composed of very strong and dense fibrous
connective tissue • They are in the shape of heavy cords and anchor
muscles firmly to bones
9Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Joint Structure and Function
• Connective tissue disorders: manifested as joint disorders since joint mobility depends on functional connective tissue
• Joint: site where two or more bones are joined; permit motion and flexibility of the rigid skeleton
• Classification• Synarthroses (fixed joints)• Amphiarthroses (slightly movable joints)• Diarthroses (freely movable joints)
• Encased in a fibrous capsule made of strong cartilage and lined with synovial membrane
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Age-Related Changes
• Loss of bone mass and bone strength• Osteoporosis common in women but affects men • Put the older patient at risk for fractures
• Cartilage gradually loses elasticity; becomes soft and frayed • Water content decreases, and cartilage may ulcerate, leaving
bony joint surfaces unprotected and promoting growth of osteophytes (bony spurs)
• Result in pain and limited mobility
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Nursing Assessment of Connective Tissue Structures
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Chief Complaint and History of Present Illness
• Complaints that suggest possible problems related to connective tissue disorders are aches, pain, joint swelling or stiffness, generalized weakness, a change in ability to work or to enjoy leisure activities, a change in appearance that is significant to the patient, and a change in ability to carry out activities of daily living
13Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Past Medical History
• Major childhood and adult illnesses, operations, and current medications and allergies
• History of tuberculosis, poliomyelitis, diabetes mellitus, gout, arthritis, rickets, infection of bones or joints, autoimmune diseases, and neuromuscular disabilities
• Accidents and injuries • Current medications
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Family History
• Osteoporosis, osteoarthritis, rheumatoid arthritis, gout, or scoliosis may have some genetic basis • Autoimmune diseases, e.g., thyroid disorders
• Review of systems • General health status; determines patient’s
perception of well-being • Fatigue, malaise, anorexia, weight loss, pain,
stiffness, dysphagia, or dyspnea
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Physical Examination
• Vital signs, height, and weight • Skin color, rashes, lesions, scars, or any signs of
injuries • Palpate skin for warmth, edema, and moisture • Palpate lymph nodes for enlargement and tenderness • Inspect joints for swelling and deformity, and palpate
for warmth, swelling, and tenderness • Joint pain and range of motion • Measure limb length and muscle strength
16Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Diagnostic Tests and Procedures
• Blood studies • Complete blood cell count, erythrocyte
sedimentation rate (ESR), and C-reactive protein determination
• Venereal Disease Research Laboratory (VDRL), rheumatoid factor (RF), creatinine, and antinuclear antibody (ANA) tests
• Urine studies• Creatinine and uric acid levels
17Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Diagnostic Tests and Procedures
• Radiologic imaging studies • Radiography, ultrasonography, arthrography,
nuclear scintigraphy, magnetic resonance imaging, diskography, tomography, and computed tomography
18Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Therapeutic Measures
• Physical and occupational therapy• Physical therapy: exercise and positioning to help preserve
functional capability and minimize disability • Occupational therapy helps patient gain maximal function in
work and personal life
• Education and support• Education: how treatment plan will benefit the patient • Patients and their families need information about community
support groups that can offer encouragement, information, and resources
19Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Therapeutic Measures
• Drug therapy• Glucocorticoids• Nonsteroidal anti-inflammatory drugs (NSAIDs)• Biologic response modifiers (BRMs)• Disease-modifying antirheumatic drugs (DMARDs)• Cyclooxygenase-2 (COX-2) inhibitors
• Surgical treatment• Indicated in some musculoskeletal disorders, such as
degenerative joint disease and arthritis• Continuous passive motion (CPM) machine
20Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Disorders of Connective Tissue Structures
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Osteoarthritis
• Pathophysiology • Degeneration of articular cartilage with hypertrophy of the
underlying and adjacent bone • Normally, articular cartilage provides a smooth surface for one
bone to glide over another• Cartilage transfers the weight of one bone to another so the
bones do not shatter• Osteoarthritis: shock-absorbing protection lost • New bone growth is stimulated by exposed bone surfaces,
causing bone spurs
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Figure 41-1
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Osteoarthritis
• Signs and symptoms • Pain in affected joint, stiffness, limitation of
movement, mild tenderness, swelling, and deformity or enlargement of the joint
• Heberden nodes and Bouchard nodes
• Medical diagnosis • Health history and radiographic studies• Arthroscopy and MRI • Synovial fluid aspiration
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Figure 41-2
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Osteoarthritis
• Medical treatment • Drug therapy
• Acetaminophen, NSAIDs, DMARDs, COX-2 inhibitors, or low dose of salicylates (aspirin)
• Surgery• Arthroscopic surgery and arthroplasty
• Physical therapy• Improve range of motion; maintain muscle mass and
strength
• Education
26Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Figure 41-3
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Osteoarthritis
• Assessment• Joint pain or tenderness• Examine joints for crepitus, enlargement, deformity,
and decreased range of motion • Compare affected and unaffected joints to detect
abnormalities• Determine how the disease affects the patient’s
mobility and ability to perform activities of daily living
28Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Osteoarthritis
• Interventions• Chronic Pain • Impaired Physical Mobility • Ineffective Coping• Ineffective Therapeutic Regimen Management
29Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Osteoarthritis
• Care following total joint replacement• Assessment
• Vital signs, level of consciousness, intake and output, respiratory and neurovascular status, urinary function, bowel elimination, wound condition, and comfort
• Circulation and sensation in the affected extremity
30Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Osteoarthritis
• Interventions• Acute Pain • Risk for Injury • Impaired Physical Mobility • Impaired Tissue Perfusion • Risk for Infection • Anxiety or Fear • Deficient Knowledge
31Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Figure 41-4
32Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Rheumatoid Arthritis
• Pathophysiology• Chronic, progressive inflammatory disease• Inflammation of the synovial tissue• Synovium thickens; fluid accumulates in 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
33Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Figure 41-6
34Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Rheumatoid Arthritis
• Signs and symptoms • Pain in affected joints aggravated by movement • Morning stiffness lasting more than 1 hour• 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 heart, lungs, kidneys, eyes • Clusters of symptoms
• Sjögren’s, Felty’s, or Caplan’s syndromes
35Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Figure 41-7
36Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Rheumatoid Arthritis
• Medical diagnosis • Health history and physical examination• Laboratory studies
• RF (rheumatoid factor), ESR (erythrocyte sedimentation rate), and CRP (C-reactive protein)
• MRI, bone scans, and DEXA scans
37Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Rheumatoid Arthritis
• Medical treatment • Drug therapy
• Aspirin and other NSAIDs for several months, with the addition of gold compounds, d-penicillamine, antimalarials, or sulfasalazine if needed
• Physical and occupational therapy• Surgery
• Arthroplasty, synovectomy, tenosynovectomy, and arthrodesis
38Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Figure 41-5
39Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Rheumatoid Arthritis
• Assessment• Pain, joint swelling, tenderness, joint deformities and limitation
of movement, fatigue, and decreased ability to perform activities of daily living
• Interventions• Chronic Pain• Activity Intolerance • Ineffective Coping • Social Isolation • Ineffective Therapeutic Regimen Management
40Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
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
41Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Osteoporosis
• 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
42Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Osteoporosis
• 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
43Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Osteoporosis
• Medical diagnosis • Absorptiometry • Radiographs • Bone specimen
• Medical treatment • Calcium supplementation and estrogen replacement • Bisphosphonates and selective estrogen receptor modulators
(SERMs) • Regular exercise • Percutaneous vertebroplasty
44Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Osteoporosis
• 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 Therapeutic Regimen Management
45Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Gout
• Pathophysiology • Characterized by hyperuricemia• Related to excessive uric acid production or
decreased uric acid excretion by the kidneys • Four stages
• Asymptomatic hyperuricemia• Acute gouty arthritis• Asymptomatic intercritical period• Chronic tophaceous gout
46Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Gout
• Signs and symptoms• Asymptomatic hyperuricemia
• Blood uric acid level is elevated, but no other symptoms • Many people with asymptomatic hyperuricemia never
progress to the next stage
47Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Gout
• Signs and symptoms• Acute gouty arthritis
• Onset is abrupt, usually occurs at night • The patient is suddenly afflicted with severe, crushing pain
and cannot bear even the light touch of bed sheets on the affected joint
• Joint commonly affected is the great toe• Symptoms usually disappear within a few days
48Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Gout
• Signs and symptoms • Asymptomatic intercritical period
• No symptoms
• Chronic tophaceous gout• Advanced gout• Tophi: deposits of sodium urate crystals that are visible as
small white nodules under the skin
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Figure 41-8
50Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Gout
• Medical diagnosis • History and physical examination • Urate crystals in synovial fluid • Urinary uric acid • Blood uric acid
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Gout
• Medical treatment • Asymptomatic hyperuricemia requires no medical
treatment • NSAID alone or with colchicine for acute gouty
arthritis • For subsequent attacks: indomethacin,
corticosteroids, and corticotrophin • Avoid foods high in purines
52Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Gout
• Assessment• Pain, joint swelling, tophi, uric acid stones, fever,
and a history of trauma, injury, or surgery
• Interventions• Acute Pain• Impaired Physical Mobility • Altered Urinary Elimination • Ineffective Therapeutic Regimen Management
53Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Progressive Systemic Sclerosis
• Pathophysiology• Primary vessel injury/dysfunction of immune system • Manifestations: from inflammation to degeneration of tissues,
that results in decreased elasticity, stenosis, and occlusion of vessels
• Signs and symptoms • Raynaud’s 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
54Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Figure 41-9
55Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Progressive Systemic Sclerosis
• Medical diagnosis • History and physical examination may lead the
physician 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
56Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Progressive Systemic Sclerosis
• Medical treatment• No cure • High doses of steroids or other
immunosuppressants may bring about remission • Physical therapy • d-Penicillamine; antihypertensives
57Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Progressive Systemic Sclerosis
• Assessment• Pain and stiffness in the fingers; 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
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Progressive Systemic Sclerosis
• Interventions• Impaired Skin Integrity • Self-Care Deficit • Chronic Pain • Social Isolation • Imbalanced Nutrition: Less Than Body
Requirements • Ineffective Therapeutic Regimen Management
59Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
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’s phenomenon, and
joint pain and inflammation• Dermatomyositis: periorbital edema as well
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Dermatomyositis/Polymyositis
• 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
61Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier, Inc.
Other Connective Tissue Disorders
• Bursitis• Carpal tunnel syndrome• Ankylosing spondylitis• Polymyalgia rheumatica • Reiter’s syndrome• Behçet’s syndrome• Sjögren’s syndrome• Periarteritis nodosa