1. Connective Tissue Disorders
Chapter 41
Connective Tissue Disorders 1

2. 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.

3. 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

4. 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

5. 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

6. 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

7. 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

8. 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

9. 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

10. 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

11. Nursing Assessment of Connective Tissue Structures11

12. 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

13. 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

14. 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

15. 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

16. 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

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

18. 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

19. 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

20. Disorders of Connective Tissue Structures20

21. 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

22. Figure 41-1 22

23. 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

24. Figure 41-2 24

25. 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

26. Figure 41-3 26

27. 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

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

29. 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

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

31. Figure 41-4 31

32. 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

33. Figure 41-6 33

34. 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

35. Figure 41-7 35

36. 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

37. 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

38. Figure 41-5 38

39. 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

40. 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

41. 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

42. 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

43. 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

44. 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

45. 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

46. 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

47. 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

48. 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

49. Figure 41-8 49

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

51. 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

52. 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

53. 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

54. Figure 41-9 54

55. 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

56. 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

57. 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

58. 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

59. 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

60. 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

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