Connective Tissue Disease

Peggy D. Johndrow

2009

Connective Tissue DiseasesCommon disorders such as Rheumatoid Arthritis (RA), Osteoarthritis (OA) or more rare conditions such as systemic lupus erythematosum (SLE) & SclerodermaCommonly referred to as Autoimmune DiseasesCan be life threatening or merely inconvenientProblems cause limitations in mobility and activities of daily living as well as systemic effects that can lead to organ failure and death

Autoimmune Disease

May be primary or secondary health problems May be acute or chronic with remission and exacerbationsPermanent changes may result from these conditions

Rheumatology Connective tissue disease (CTD) major focus of rheumatologyRheumatic disease any disease or condition involving musculoskeletal systemArthritis means inflammation of one or more jointsNon-inflammatory arthritis not systemicInflammatory arthritis– Rheumatoid arthritis– Systemic lupus erythematosus

Diagnostic Studies

Arthrocentesis: needle aspiration of synovial fluid to obtain fluid & relieve pain; fluid cloudy instead of clearX-rays: diagnose & monitor disease; show cartilage abnormalities, joint erosion, abnormal bone growth & osteopenia (decreased bone mineralization)

Arthrography: detect connective tissue disorders; radiopaque substance injected into joint cavity, outline joint contourBone and joint scans: reflect crystal lattice of bone “takes up” radioactive isotope; uptake considered abnormal

Diagnostic Studies

Biopsy: muscle biopsy myositis, arterial biopsy arteritis/vasculitis, skin biopsy confirm inflammatory connective tissue disease lupus and scleroderma

Serum labsNo one test used in isolation sufficiently diagnoses a rheumatic disease

Serum Laboratory StudiesErythrocyte sedimentation rate (ESR) : increase CTD; indicates rising inflammation; used to monitor long-term inflammatory disease process; higher ESR greater inflammatory activityHematocrit: decrease in chronic inflammation RBC: decrease seen in RA, SLE

WBC: decrease in SLESerum Immunology– ANA Titer: positive with SLE,

RA, Scleroderma, Raynaud’s disease, Sjogren’s syndrome, necrotizing arteritis; higher titer greater inflammation

– Complement levels: decrease in RA and SLE[ indicates autoimmune/inflammatory activity

Serum Laboratory StudiesC-Reactive Protein: positive indicates active inflammation; often positive for RA, disseminated lupus erythematosumTissue Typing: HLA antigen; measures presences of HLA Antigen used for tissue recognition; found in 80-90% of clients ankylosing spondylitis and Reiter’s syndrome

Immunoglobulin Electrophoresis: increased levels with autoimmune disordersRheumatoid Factor (RF): determines presence of abnormal antibodies seen in CTD; positive in 80% with RA; positive may also suggest CTD; higher titer greater inflammation

Implications

Diagnostic studies not clear-cutObserve signs and symptoms over time to diagnoseReferred to rheumatologist for management

Gerontological Considerations

May be primary or secondary Clinical course can be differentPharmacological treatment in older clients increased risk for ototoxicity, cumulative effect, altered metabolism of certain medicationMore prone to side effects

Assessment

Health historyPhysical ExamFunctional Assessment of MusculoskeletalDiagnostic studies

Pharmacological ManagementSalicylates: action anti-inflammatory, analgesic, antipyretic, platelet aggregation inhibitor; first line treatmentNonsteroidal Anti-inflammatory Drugs (NSAIDs): action anti-inflammatory, analgesic, antipyretic, platelet aggregation inhibitor; alternate to salicylates for first-line therapy in several rheumatic diseases

Cox-2 Inhibitors: action inhibit cyclooxygenase-2 (COX-2) enzymes, produced during inflammation & spare COX-1 enzymes, protective stomach & kidneysDisease Modifying Antirheumatic Drugs (DMARDs): anti-inflammatory, inhibit lysosomal enzymes; onset 2-4 monthsAdditionally: short-term low dose anti-depressants prescribed to re-establish sleep patterns, provide better pain management.

Degenerative Joint Disease Osteoarthritis (OA) Most common disability joint disorderIdiopathic, seen with increasing age; increasing age directly related to degenerative changes, as ability of articular cartilage unable to resist microfracture with repetitive low loads diminishes Begins in 30’s and peaks in 50’s and 60’s

Osteoarthritis (OA)

Affects articular cartilage, subchondral bone & synoviumHereditary, congenital & developmental disorders predisposes to OA of hipObesity associated with OAMechanical factors such as joint trauma, sports activities, and occupationsIW Consider older adults; genetic considerations p 323

AssessmentHistory (IW Bullets p 324)Physical assessment and clinical manifestations– Joint involvement– Heberden's nodes– Bouchard’s nodes– Joint effusions– Atrophy of skeletal muscle

PsychosocialDiagnostic Studies

Clinical Manifestations

Pain, stiffness, and functional impairmentStiffness in morning or after awakening usually last less than 30 minutes & decrease with movement Affects weight bearing joints hips, knees, cervical, lumbar spine, proximal and distal fingers Characteristic bony nodes may be present; are usually painlessCompare to RA (I&W Table 20-1 p 325)

Diagnosis

Tender enlarged joints, inflammation not destructive typeCharacterized by progressive loss of joint cartilage, appears on x-ray as narrowing joint spaces, osteophytes, when combined with narrowing joint spaces are sensitive and specific findingsSerum studies not useful in diagnosis of disorder

Management

Preventive measures– Weight reduction, prevention of injuries– Health Promotion (I&W Bullets p 324; Chart 20-7 p

336)

Conservative Treatment– Heat, weight reduction, joint rest and avoidance of

overuse, orthotic devices to splint and brace, OT, PT, isometric and postural exercises

– I&W Chart 20-1 p 327; Bullets p 327; Chart 20-6 p 336

Pharmacological Therapy

Tylenol if not relieved, add NSAIDs, ongoing reassess and reduce dose of NSAIDs and use only intermittently with joint pain exacerbation Intra-articular injections of corticosteroids for immediate short term reliefIW Chart 20-2 p 328

Surgical Management

Only when pain is intractable and function has been lost (IW Bullets p 328)Arthroplasty: replacement of affected joints– I&W Table 20-2 p 330– Knee replacement: continuous passive motion

machine (CPM) I&W Fig 20-3 p 333; Chart 20-5 p 334

Total Hip Arthroplasty

Preoperative careOperative proceduresPostoperative care– Prevention of dislocation, infection & thromboembolic

complications– Assessment of bleeding– Management of anemia

Care of Total Hip Arthroplasty

Assessment for neurovascular compromiseManagement of painProgression of activityPromotion of self-care

– I&W Chart 20-3 p 330; Fig 20-2; Chart 20-4 p 332

Rheumatoid Arthritis

A most common connective tissue disease & most destructive to jointsChronic, progressive, systemic inflammatory autoimmune disease primarily affecting synovial jointsAutoantibodies (rheumatoid factors) formed that attack healthy tissueAffects synovial tissue of any organ or body system

Rheumatoid Arthritis Pathophysiology

Occurs in synovial tissuePhagocytosis produces enzymes within joint, enzymes breakdown collagen, causes edema, proliferation of synovial membrane, and ultimately pannus formationPannus destroys cartilage and boneResults in loss of articular surface, joint motion, muscle elasticity and contractile power lost Genetics (I&W p 337)

Clinical Manifestations Classic: edema, pain, swelling, warmth, erythema & lack of function classicInitially involves small joints as disease progresses involve larger joints; bilateral and symmetricalClassic sign joint stiffness in morning lasting more than 30 minutes; stiffness improves later in dayJoint deformities (I&W Fig 20-4 p 338)I&W Chart 20-8 p 337

Other ManifestationsImmobility for extended periods leads to contractures leading to deformity; deformities common in rheumatoid arthritis Systemic: fever, weight loss, fatigue, anemia, lymph node enlargement, arteritis, neuropathy, scleritis, pericarditis, splenomegaly, Sjogren syndrome (dry eyes and dry mucous membranes) and Raynaud’s phenomenon (IW Bullets p 338)Nodules non-tender and movable in subcutaneous tissue; appear over bony prominences I&W Chart 20-8 p 337

Diagnostic Studies

Rheumatoid factor present in 80% of clients, ESR elevated, RBC & C4 complement component decreased; CRP & ANA may be positive Arthrocentesis fluid cloudy; X ray reveals bony erosion & narrowed joint spacesIW Chart 20-9 p 339

AssessmentPhysical assessment and clinical manifestations– Early disease manifestations– Late disease manifestations– Joint involvement– Systemic complications– Associated syndromes

Psychosocial assessmentDiagnostic studies

Management Mild SymptomsBegin with salicylates or first generation NSAIDs; dosage to maintain consistent blood levelSecond generation NSAIDs: COX-2 inhibitor blocks enzyme involved in inflammation without block enzyme involved in protecting stomach lining; less toxic than first generationI&W Chart 20-10 p 341-342

Management Moderate Symptoms

DMARDs initiated early in treatment within 2 years improves symptoms control and managementMethotrexate used successfully Physical therapy and add cyclosporine (immunomodulator) to enhance effects of methotrexate

Management Severe Symptoms

Erosive effectsReconstructive surgery, corticosteroids Biological response modifiers such as etanercept, infiximab, adalimumab, anakinraImmunosuppressive agents; high dose methotrexate, cyclophosphamide, and azathioprineHighly toxic and can produce bone marrow suppression

Nonpharmacological Treatment of RA

PlasmapheresisComplementary and alternative therapies (I&W Bullets p 345)Promotion of self-careManagement of fatigue (I&W Bullets p 346; Chart 20-11 p 346)Enhancement of body imageHealth teaching

Nursing Care

I&W Plan of Care p 359Number 1&2; Bullets p 325

Systemic Lupus Erythematosus

Chronic, progressive, inflammatory connective tissue disorder can cause major body organs and systems to failMany clients with SLE have some degree of kidney involvement; increasing renal involvement has poor prognosis (I&W Bullets p 347)

AssessmentHistory (IW Culture & Genetic p 348)Physical assessment and clinical manifestations– Skin involvement– Musculoskeletal changes– Systemic manifestations including pleural effusions or

pneumonia and Raynaud’s phenomenon

Psychosocial results can be devastatingDiagnostic studies

Clinical Manifestations

Insidious or acuteInvolves multiple body systemsI&W Chart 20-12 p 349

Musculoskeletal

Arthralgia and arthritis (synovitis)Joint swelling, tendernessPain on movementMorning stiffness

Integument

Polycystic lesionsChronic rashButterfly rash (“wolf mask”)– IW Fig 20-7 p 349

Oral ulcers occurring in cropsI&W Chart 20-13 p 350

Vascular and Lymphatic

CV; pericarditis Inflammation of arteriolesPapular, erythematous, purpuric lesions on the fingertips, elbow toes, forearms, and hands May progress to necrosisLymphadenopathy

Renal

Renal involvement affecting glomeruli Can result in renal failurePrognosis poor if this occurs

Neurological and Behavioral

Widespread Neurological involvementChanges in behavior and cognitive functionDepression and psychosis

Management

Control disease activity or exacerbationsPrevent progressive loss of organ function

Pharmacological therapyNSAIDs for minor clinical manifestationsCorticosteroids: single most important medication available for treatmentUse topically for cutaneous; low oral doses; high doses for major disease activity IV corticosteroidsAntimalarials used for cutaneous, musculoskeletal & mild systemic featuresImmunosuppressive agents with serious forms unresponsive to conservative therapies

Progressive Systemic SclerosisSystemic scleroderma, meaning hardening of the skinDiffuse cutaneous sclerodermaLimited cutaneous sclerodermaPharmacotherapy slows disease progression but often unsuccessful

Scleroderma PathophysiologyBegin skin involvement, mononuclear cells cluster on skin, stimulate lymphokines to stimulate pre-collagen; insoluble collagen formed & accumulates excessively in tissue Initial inflammatory response, edema formation, results in taunt, smooth & shiny skin Skin undergoes fibrotic changes, leads to loss of elasticity & movement; eventually tissue degenerates, becomes nonfunctional Occur in major blood vessels, major organs & body systems potentially resulting in death IW Bullets p 351

Clinical manifestations

Starts insidiously with Raynaud’s phenomenon and swelling in hand Skin and subcutaneous tissue hard & rigidSkin dry: secretion suppressedExtremities stiffen & lose mobility; IW Fig 20- p 351)Condition spreads slowlyFace mask like appearance, immobile & expressionless, mouth becomes rigid

Other Manifestations

Left ventricle with heart failureEsophagus hardens interfering with swallowingLung scarring impeding respirationsDigestive disturbances because of hardening of intestinal mucosaProgressive renal failure occursI&W Bullets p 351-352

CREST

Calcinous (calcium deposits in the skin)Raynaud’s phenomenonEsophageal hardeningSclerodactyly (scleroderma of the digits)Telangiectasis (capillary dilation that forms a vascular lesion)

Assessment and Diagnosis

Skin changesSkin biopsyAbnormal ventilation perfusion studiesAbnormal esophageal studiesPositive ANA

Client Education

Medication instructionsEncourage independence as possibleInformation about the disease processTherapeutic regimen

Management

Penicillamine decreases skin thickening & reduces rate of new visceral organ involvementCapoten (captopril) effective hypertension control Anti-inflammatory to control arthralgia, stiffness, & general musculoskeletal discomfort

Nursing Interventions

Common problems impaired skin integrity; self care deficits, altered nutrition, and body image disturbanceFocus on impaired gas exchange, decreased cardiac output, impaired swallowing and constipation I&W Chart 20-14 p 352

GoutAlso called gouty arthritis, a systemic disease in which urate crystals deposit in joints and other body tissues, causing inflammationPrimary gout: most common type; cause by several errors of metabolism; uric acid production greater than ability of kidneys to excreteSecondary gout: excess uric acid caused by another disease/medication (renal disease, diuretics, chemotherapy)

Clinical Manifestations

Acute gout: joint inflammation caused by uric acid depositsChronic gout: presence of tophi (deposits of sodium urate crystals; IW Fig 20-9 354); ear, joints of arms, fingers; hard to palpation, irregular shape; renal calculi, renal dysfunctionDiagnosis: serial measurements serum uric acid levels; urine uric acid levels

ManagementAcute gout: combination of Novocolchicine (colchicine), NSAID, Motrin (ibuprofen); IV colchinicine effect in 12 hours, take orally for 4-7 daysChronic gout: Zyloprim (allopurinol), Benemid (probenecid), ColBenemid (probenecid/colchicine)Diet Therapy: usually avoid foods that cause gouty attacks; increase fluid intakeAvoid aspirin & diuretics; extreme stress, excessive physical exercise

Lyme Disease

Reportable systemic infectious disease caused by spirochete Borrelia burgdorferi, resulting from bite of infected deer tickStages I, II and IIIIf not treated in early stages, chronic complications such as arthralgias, fatigue, memory & thinking problems present in later stages; permanent damage to joints & nervous system

Clinical Manifestations

Stage I: Large “bulls eye” circular rash, flu-like symptoms (malaise, fever, H/A, joint/muscle pain); symptoms within 3-32 days of tick biteStage II: occur 2-12 weeks after tick bite; carditis, dysrhythmias, dyspnea, dizziness, palpitations; meningitis, facial paralysis (www.chiroweb.com/archives/10/20/05.html ) , peripheral neuritisStage III (chronic persistent): occur weeks to years after tick bite; only sign may be arthritis, disease not respond to antibiotics, permanent damage to joints and nervous system

Pharmacological Therapy

Stage I: Vibramycin (doxycycline), Amoxil (amoxicillin), Ceftin (cefuroxime) taken for 10-21 daysStage II: if severe, IV antibiotics, Rocephin (ceftriaxone), Claforan (cefotaxime)Stage III: treat symptoms; damage may be permanent Prevention best therapy (I&W Chart 20-16 p 356)

Fibromyalgia Syndrome

Chronic pain syndrome, not an inflammatory disease (I&W Fig 20-10 p 357)Pain typically located at trigger points (neck, upper chest, trunk, low back, extremities)Other symptoms (I&W Bullets p 358)Exacerbated by stress, increased activity, change weather conditionsSecondary disease may develop r/t RA, SLE

Management

Physical therapy treatmentPharmacotherapy with NSAIDs, muscle relaxants, sedatives for sleepHome exercises, including walking, swimming, rowing, biking, and water exerciseStress management

Other Related Disorders

Polymyositis/DermatomyosisSystemic Necrotizing VasculitisPolymyalgia Rheumatica; Temporal Arteritis(I&Wchart 20-15 p355)Ankylosing SpondylitisReiter’s SyndromeSjogren’s SyndromeAnkylosing Spondylitis (IW Genetics p 355

Marfan SyndromeInfectious ArthritisPseudogoutPsoriatic ArthritisChronic Fatigue Syndrome(I&W Bullets p 358)Local inflammatory Mixed Connective tissue diseasePsoriatic arthritis (IW Table 20-3 p 357

Generalized Nursing Interventions

Pain Relief

Medications for short-term acute pain– Non-opioid analgesics– NSAIDS

Disease modifying medications

Non-Pharmacological Pain ReliefSuperficial heat applied form of warm tub baths, showers, warm moist compressesParaffin baths (dips) for concentrated heat for wrist and small-joint involvement Maximum benefit with 20 minutes of applicationHeat may increase painIf inflammatory process acute may try cold compressMuscle relaxation techniques, self-hypnosis, and distraction

Joint Support

Use of braces, splints, and assistive mobility devices to ease pain from weight bearing Splints may be applied to rest inflamed joints, support the joint and relieve spasms

Decrease Fatigue and Promote Sleep

Acute or chronic Modify and reduce fatiguePeriods of rest, splints, conditioning exercise to build endurance; recommended walking, swimming, bicyclingSleep-inducing routine, medications, and comfort measures

Improve Mobility

Proper body positing to prevent deformity Support joints Firm mattress supine position with a footboard and one pillow under the head.

Lie prone several times a day Active range of motion; passive ROM if unable to perform activeAssistive devices

Exercise and Activity

Maintain and improve joint functionActive inflammatory process: passive ROMSubacute inflammatory process: active assistive ROM or active ROM within pain toleranceInactive, remission: active ROM, isometrics

Facilitate Self Care and Self-Concept

Adaptive equipment to increase independenceDemonstrate use and positive attitude Encourage verbalization of feelings, perceptions, and outlook Active listeningAcceptance

Monitor and Manage Potential

Complications Avoid drug-induced complicationsRecognize and deal with side effects – Side Effects: GI bleeding or irritation, bone marrow

depression, mouth sores, rashes, changes in vision, bruising, breathing problem, dizziness, jaundice, dark urine, black or tarry stools, diarrhea, nausea and vomiting and headaches

Corticosteroids can mask systemic and local infections