Connective Tissue Disorders Chapter 41 Connective Tissue Disorders 1

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 assessment of a patient with a connective tissue disorder. Assist in developing a nursing care plan for a patient whose life has been affected by a connective tissue disease.

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 More than 66 million people in the United States have arthritis or chronic joint symptoms; their primary effects are on connective tissue. What is the cause of rheumatoid disorders? 3

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) 4

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 5

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 6

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 7

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 8

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 What bone in the body does not articulate with at least one other bone? The synovial membrane is very smooth, thus permitting structures to move without friction. The bursae permit tendons to slide easily with movement of the bones. 9

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 What is caused by the progressive loss of bone density? 10

Nursing Assessment of Connective Tissue Structures 11

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 12

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 Accidents and injuries, even in the distant past, may be significant because they could be related to the patient’s current problem. Self-medication is common with musculoskeletal pain, and patients may fail to report it unless specifically asked. Why is it important to ask about the use of alternative therapies? 13

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 14

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 What is crepitus? A goniometer measures the range of movement of the joints. 15

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 What tests may be done on fluid aspirated from the joints? Most laboratory tests are not diagnostic for a single condition—that is, many factors could cause measures of inflammation to increase. 16

Diagnostic Tests and Procedures Radiologic imaging studies Radiography, ultrasonography, arthrography, nuclear scintigraphy, magnetic resonance imaging, diskography, tomography, and computed tomography 17

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 Team members must be sensitive to the inconvenience and discomfort the disease has caused and must also help the patient identify ways to cope with the condition. How can a patient’s attitude affect the outcome of a chronic disease? 18

Therapeutic Measures Drug therapy Surgical treatment 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 19

Disorders of Connective Tissue Structures 20

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 Osteoarthritis may be classified as primary or secondary, depending on the cause. Osteoarthritis that occurs with aging is generally considered to be primary and may have a genetic basis. Secondary osteoarthritis may be associated with trauma, infection, congenital deformities, or corticosteroid therapy. What type of joints does osteoarthritis generally affect? 21

Figure 41-1 22

Osteoarthritis Signs and symptoms Medical diagnosis 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 Many people with osteoarthritis have no symptoms, but others have pain that ranges from mild to severe. What is the pain of osteoarthritis usually associated with? 23

Figure 41-2 24

Osteoarthritis Medical treatment Drug therapy Surgery Physical 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 What are the goals of patient therapy? Moist heat and, occasionally, cold can be used to help relieve pain. Transcutaneous nerve stimulation (TENS) devices are especially effective for treating back pain. 25

Figure 41-3 26

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 27

Osteoarthritis Interventions Chronic Pain Impaired Physical Mobility Ineffective Coping Ineffective Therapeutic Regimen Management 28

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 29

Osteoarthritis Interventions Acute Pain Risk for Injury Impaired Physical Mobility Impaired Tissue Perfusion Risk for Infection Anxiety or Fear Deficient Knowledge 30

Figure 41-4 31

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 In what population is rheumatoid arthritis commonly seen? There is no single known cause for RA, but it is considered an autoimmune disorder. 32

Figure 41-6 33

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 What is vasculitis? Sjögren’s syndrome is characterized by dryness of the mouth, eyes, and vagina. Felty’s syndrome, characterized by liver and spleen enlargement and neutropenia, is less common. Caplan’s syndrome, which is marked by rheumatoid nodules in the lungs, occurs most often in coal miners and asbestos workers. 34

Figure 41-7 35

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 36

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 What is the goal of drug therapy? In addition to drug therapy, supportive treatments may be used—including rest, splinting of joints to reduce the motion that aggravates the inflammation, orthotic devices to support deformed joints, and assistance in modifying activities of daily living. Surgical management of RA is reserved for those patients with severe pain and deformities. 37

Figure 41-5 38

Rheumatoid Arthritis Assessment Interventions 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 39

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 What sites are commonly fractured as a result of osteoporosis? Age-related loss of bone mass without apparent underlying medical causes is called primary osteoporosis. Secondary osteoporosis is the loss of bone mass due to factors other than age, such as hyperparathyroidism or long-term therapy with corticosteroids or heparin. 40

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 41

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 42

Osteoporosis Medical diagnosis Medical treatment Absorptiometry Radiographs Bone specimen Medical treatment Calcium supplementation and estrogen replacement Bisphosphonates and selective estrogen receptor modulators (SERMs) Regular exercise Percutaneous vertebroplasty Absorptiometry is a quick, painless radiologic procedure that measures the amount of bone tissue in the hip and spine. When will radiographs show decreased bone density? 43

Osteoporosis Assessment Interventions 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 44

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 There are two forms of gout: primary gout, in which uric acid is elevated because of a metabolic disorder; and secondary gout, in which uric acid is elevated owing to another disease process. What can increase the risk of gout? 45

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 46

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 47

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 48

Figure 41-8 49

Gout Medical diagnosis History and physical examination Urate crystals in synovial fluid Urinary uric acid Blood uric acid 50

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 If the patient does not respond to colchicine or NSAIDs, parenteral glucocorticoids or adrenocorticotropin may be prescribed. What drugs are used to inhibit uric acid synthesis? 51

Gout Assessment Interventions 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 52

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 Progressive systemic sclerosis is commonly called scleroderma. What organs are commonly affected by PSS? 53

Figure 41-9 54

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 55

Progressive Systemic Sclerosis Medical treatment No cure High doses of steroids or other immunosuppressants may bring about remission Physical therapy d-Penicillamine; antihypertensives Esophageal reflux may be treated with drugs to decrease the acidity of gastric secretions and with periodic dilation of the esophagus and other measures, such as small, frequent feedings and elevation of the head of the bed. How is Raynaud’s phenomenon managed? Antihypertensives are used to control hypertensive crisis. 56

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 57

Progressive Systemic Sclerosis Interventions Impaired Skin Integrity Self-Care Deficit Chronic Pain Social Isolation Imbalanced Nutrition: Less Than Body Requirements Ineffective Therapeutic Regimen Management 58

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 Dermatomyositis and polymyositis are relatively rare acute or chronic inflammatory diseases that primarily affect the skeletal muscle. The term polymyositis is applied to the condition when there is no skin involvement, and dermatomyositis is used when there is a characteristic skin rash. What disorders are commonly associated with dermatomyositis/polymyositis? 59

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 60

Other Connective Tissue Disorders Bursitis Carpal tunnel syndrome Ankylosing spondylitis Polymyalgia rheumatica Reiter’s syndrome Behçet’s syndrome Sjögren’s syndrome Periarteritis nodosa 61