Autoimmune Disorders That Affect the Musculoskeletal System

Autoimmune Disorders Autoimmune Disorders That Affect the That Affect the

Musculoskeletal SystemMusculoskeletal System

Rheumatoid ArthritisRheumatoid Arthritis

Rheumatoid Arthritis (RA)Rheumatoid Arthritis (RA)

Chronic, systemic autoimmune disease Chronic, systemic autoimmune disease Inflammation of connective tissue in Inflammation of connective tissue in

diarthrodial (synovial) joints diarthrodial (synovial) joints Periods of remission and exacerbationPeriods of remission and exacerbation Frequently accompanied by extra-articular Frequently accompanied by extra-articular

manifestations manifestations

IncidenceIncidence

Occurs globally, affecting all ethnic groupsOccurs globally, affecting all ethnic groups Occurs at any time of lifeOccurs at any time of life Incidence Incidence increases with ageincreases with age

Peaks between 30s and 50sPeaks between 30s and 50s Nearly 2.1 million Americans affectedNearly 2.1 million Americans affected Women have incidences three times Women have incidences three times

higher than menhigher than men

EtiologyEtiology

Cause of RA is unknownCause of RA is unknown No infectious agent foundNo infectious agent found Two etiologies Two etiologies

Autoimmune etiologyAutoimmune etiology• Most widely accepted Most widely accepted

Genetic factor etiologyGenetic factor etiology

Etiology and Pathophysiology of Rheumatoid Etiology and Pathophysiology of Rheumatoid ArthritisArthritis

Main Pathology is chronic inflammation leading to scar Main Pathology is chronic inflammation leading to scar tissue (pannus) & joint cartilage destructiontissue (pannus) & joint cartilage destruction

Etiology and Pathophysiology of Etiology and Pathophysiology of Rheumatoid ArthritisRheumatoid Arthritis

Inflammatory pannus causes destruction Inflammatory pannus causes destruction of bone.of bone.

This leads to joint deformities. This leads to joint deformities.

PathophysiologyPathophysiology

If unarrested, RA progresses in 4 stagesIf unarrested, RA progresses in 4 stages Stage 1: Stage 1: EarlyEarly

• No destructive changes on x-ray, possible x-ray No destructive changes on x-ray, possible x-ray evidence of osteoporosisevidence of osteoporosis

Stage 2: Stage 2: ModerateModerate• X-ray evidence of osteoporosis, with or without slight X-ray evidence of osteoporosis, with or without slight

bone or cartilage destructionbone or cartilage destruction• No joint deformities, adjacent muscle atrophy, No joint deformities, adjacent muscle atrophy,

possibly presence of extra-articular soft tissue lesionspossibly presence of extra-articular soft tissue lesions

Etiology and PathophysiologyEtiology and Pathophysiology

RA progresses in four stages (cont'd)RA progresses in four stages (cont'd) Stage 3: Stage 3: SevereSevere

• X-ray evidence of cartilage and bone destruction in X-ray evidence of cartilage and bone destruction in addition to osteoporosis; joint deformity; extensive addition to osteoporosis; joint deformity; extensive muscle atrophy; possible presence of extra-muscle atrophy; possible presence of extra-articular soft tissue lesionsarticular soft tissue lesions

Stage 4: Stage 4: TerminalTerminal• Fibrous or bony ankylosis, stage III criteria Fibrous or bony ankylosis, stage III criteria

Clinical ManifestationsClinical ManifestationsOnsetOnset

Onset is typically insidiousOnset is typically insidious Nonspecific manifestations may precede Nonspecific manifestations may precede

onset of arthritic complaintsonset of arthritic complaints

Some report a history of precipitating Some report a history of precipitating eventsevents

Clinical ManifestationsClinical Manifestations JointsJoints

Specific articular Specific articular involvementinvolvement

Symptoms occur Symptoms occur symmetricallysymmetrically

Frequently affect small Frequently affect small joints of hands and feetjoints of hands and feet

Larger peripheral joints Larger peripheral joints may also be involvedmay also be involved

Typical Deformities of Typical Deformities of Rheumatoid Arthritis Rheumatoid Arthritis

Fig. 65-4Fig. 65-4


Patient experiences joint stiffness after Patient experiences joint stiffness after periods of __________periods of __________

Morning stiffness may last from 60 Morning stiffness may last from 60 minutes to several hours or moreminutes to several hours or more

MCP and PIP joints typically swollenMCP and PIP joints typically swollen Fingers may become spindle shaped from Fingers may become spindle shaped from

synovial hypertrophy and thickening of synovial hypertrophy and thickening of joint capsule joint capsule


Joints become tender, painful, and warmJoints become tender, painful, and warm Joint painJoint pain

Increases with ______Increases with ______ Varies in intensityVaries in intensity May not be proportional to degree of May not be proportional to degree of

inflammationinflammation Tenosynovitis frequently affects extensor and Tenosynovitis frequently affects extensor and

flexor tendons near wrists flexor tendons near wrists RA progressesRA progresses

Extraarticular Manifestations of Extraarticular Manifestations of Rheumatoid ArthritisRheumatoid Arthritis

Fig. 65-5Fig. 65-5

Clinical ManifestationsClinical Manifestations Extraarticular ManifestationsExtraarticular Manifestations

Three most commonThree most common

Rheumatoid nodules develop in up to 25% Rheumatoid nodules develop in up to 25% of all patients with RAof all patients with RA

Those affected usually have high RF titersThose affected usually have high RF titers


SjSjöögren’s syndrome gren’s syndrome Seen in 10% to 15% of patients with RASeen in 10% to 15% of patients with RA Can occur as a disease by itself or in Can occur as a disease by itself or in

conjunction with other arthritic disorders conjunction with other arthritic disorders • RA and systemic lupus erythematosus (SLE)RA and systemic lupus erythematosus (SLE)

Complaints of burning, gritty, itchy eyesComplaints of burning, gritty, itchy eyes Decreased tearing, photosensitivity Decreased tearing, photosensitivity


Felty’s syndromeFelty’s syndrome Most commonly in patients with severe, Most commonly in patients with severe,

nodule-forming RA nodule-forming RA Characterized by Characterized by

• Inflammatory eye disorderInflammatory eye disorder• SplenomegalySplenomegaly• Lymphadenopathy Lymphadenopathy • Pulmonary diseasePulmonary disease• Blood dyscrasias Blood dyscrasias

ComplicationsComplications

Joint destruction begins as early as first Joint destruction begins as early as first year of disease without treatmentyear of disease without treatment

Flexion contractures and hand deformities Flexion contractures and hand deformities Cause diminished grasp strengthCause diminished grasp strength Affect patient’s ability to perform Affect patient’s ability to perform

self-care tasksself-care tasks Cataract development and loss of vision Cataract development and loss of vision

can result from scleral nodulescan result from scleral nodules


Rheumatoid nodules Rheumatoid nodules On the skin can ulcerate, similar to On the skin can ulcerate, similar to

pressure ulcers pressure ulcers On vocal cords leads to progressive On vocal cords leads to progressive

hoarseness hoarseness In vertebral bodies can cause bone In vertebral bodies can cause bone

destructiondestruction


Cardiopulmonary effects may occur later Cardiopulmonary effects may occur later in RAin RA Pleurisy, pleural effusion, pericarditis, Pleurisy, pleural effusion, pericarditis,

pericardial effusion, cardiomyopathypericardial effusion, cardiomyopathy Carpal tunnel syndrome can result from Carpal tunnel syndrome can result from

swelling of synovial membraneswelling of synovial membrane

Diagnostic StudiesDiagnostic Studies

RA is defined as having at least 4 of the RA is defined as having at least 4 of the following seven criteria. following seven criteria. Following must be Following must be present for at least 6 wks:present for at least 6 wks:

1.1. Morning stiffness that lasts ≥1 hourMorning stiffness that lasts ≥1 hour

2.2. Swelling in three or more jointsSwelling in three or more joints

3.3. Swelling in hand jointsSwelling in hand joints

4.4. Symmetrical joint swellingSymmetrical joint swelling

5.5. Erosions or decalcification seen on hand Erosions or decalcification seen on hand x-rays x-rays

6.6. Rheumatoid nodulesRheumatoid nodules

7.7. Presence of serum RFPresence of serum RF

Diagnostic TestingDiagnostic Testing History & PE Blood Studies

Rheumatoid factor (RF) Erythrocyte sedimentation rate (ESR) C-reactive protein (CRP) Antinuclear antibodies (ANA) titers are seen in some

Synovial fluid analysis Straw-colored fluid with fibrin flecks WBC is elevated to >25,000/μl

X-rays Will not confirm diagnosis – only show bone changes

Collaborative CareCollaborative Care

Care begins with a comprehensive Care begins with a comprehensive program of education and drug therapyprogram of education and drug therapy Education of drug therapyEducation of drug therapy

Patient and family educated about disease Patient and family educated about disease process and home management strategies process and home management strategies

Involve PT and OTInvolve PT and OT

Drug TherapyDrug Therapy

Drugs remain cornerstone of treatment Drugs remain cornerstone of treatment DMARDs can lessen permanent effects of DMARDs can lessen permanent effects of

RA RA Choice of drug is based on patient’s Choice of drug is based on patient’s

disease activitydisease activity level of functionlevel of function lifestyle considerations: e.g; choices for a lifestyle considerations: e.g; choices for a

woman of childbearing age? woman of childbearing age?

Treatment and Nursing Care for Treatment and Nursing Care for Rheumatoid ArthritisRheumatoid Arthritis

See Table 65-3 for meds used for arthritis p. See Table 65-3 for meds used for arthritis p. 1698-1700. 1698-1700. SalicylatesSalicylates NSAIDsNSAIDs AntibioticsAntibiotics Topical analgesicsTopical analgesics CorticosteroidsCorticosteroids DMARDsDMARDs Gold compoundsGold compounds AntimalarialsAntimalarials ImmunosupressantsImmunosupressants Biologic/Targeted therapyBiologic/Targeted therapy

Drug TherapyDrug TherapyDMARDDMARD

Methotrexate (Rheumatrex) is drug of Methotrexate (Rheumatrex) is drug of choicechoice Rapid antiinflammatory effect Rapid antiinflammatory effect decreases decreases

clinical symptoms in days to weeksclinical symptoms in days to weeks InexpensiveInexpensive Lower toxicity compared to other drugsLower toxicity compared to other drugs Side effects: bone marrow suppression, Side effects: bone marrow suppression,

hepatotoxicityhepatotoxicity

Drug TherapyDrug TherapyDMARDDMARD

Other DMARDs for mild to moderate Other DMARDs for mild to moderate diseasedisease Sulfasalazine (Azulfidine)Sulfasalazine (Azulfidine) Antimalarial drug hydroxychloroquineAntimalarial drug hydroxychloroquine

Leflunomide (Arava) - Leflunomide (Arava) - a newer synthetica newer synthetic: : DMARD that blocks immune cell overproduction DMARD that blocks immune cell overproduction


Biologic/targeted drug therapies can also Biologic/targeted drug therapies can also slow disease progression in RAslow disease progression in RA

Can be used in patients with moderate to Can be used in patients with moderate to severe disease who have not responded severe disease who have not responded to DMARDs or in combination therapy with to DMARDs or in combination therapy with an established DMARDan established DMARD


Corticosteroid therapy can aid in symptom Corticosteroid therapy can aid in symptom controlcontrol Intraarticular injections may relieve pain and Intraarticular injections may relieve pain and

inflammation associated with flare-upsinflammation associated with flare-ups• Long-term use should not be a mainstayLong-term use should not be a mainstay

Risk osteoporosis, avascular necrosis Risk osteoporosis, avascular necrosis

Low-dose prednisone for a limited time to Low-dose prednisone for a limited time to decrease disease activity until DMARD effect is decrease disease activity until DMARD effect is seenseen


Various NSAIDs and salicylates are used Various NSAIDs and salicylates are used to treat pain and inflammationto treat pain and inflammation

Aspirin is often used in high dosages of 4 to Aspirin is often used in high dosages of 4 to 6 g/day (10 to 18 tablets) 6 g/day (10 to 18 tablets)

NSAIDs have antiinflammatory, analgesic, NSAIDs have antiinflammatory, analgesic, and antipyretic properties; fand antipyretic properties; full effectiveness ull effectiveness may take 2 to 3 weeksmay take 2 to 3 weeks

NSAIDS may be used when patient cannot NSAIDS may be used when patient cannot tolerate high doses of aspirin tolerate high doses of aspirin

Combination Drug TherapyCombination Drug Therapy

Use of two more drugs simultaneouslyUse of two more drugs simultaneously Can slow symptoms and joint damage Can slow symptoms and joint damage

while improving functionwhile improving function Often include a DMARD, an NSAID, and a Often include a DMARD, an NSAID, and a

corticosteroid corticosteroid

Relieve PainRelieve Pain NSAIDsNSAIDs

DMARDsDMARDs

Non-PharmacologicalNon-Pharmacological Heat or Cold applications Heat or Cold applications RestRest Relaxation techniquesRelaxation techniques

Nursing ImplementationNursing ImplementationAcute InterventionAcute Intervention

Usually treated on an outpatient basisUsually treated on an outpatient basis Hospitalization may be necessary for Hospitalization may be necessary for

patients with extraarticular complications patients with extraarticular complications or advancing disease or advancing disease Reconstructive surgery for disabling Reconstructive surgery for disabling

deformities deformities Nursing intervention begins with a careful Nursing intervention begins with a careful

physical assessment physical assessment

Nursing ManagementNursing ManagementAssessmentAssessment

Nurse must alsoNurse must also Evaluate psychosocial needs and Evaluate psychosocial needs and

environmental concernsenvironmental concerns After problem identification, coordinate a After problem identification, coordinate a

carefully planned program for rehabilitation carefully planned program for rehabilitation and education for interdisciplinary health care and education for interdisciplinary health care teamteam

Nursing ManagementNursing ManagementProblemsProblems

Chronic painChronic pain Impaired physical mobility Impaired physical mobility Activity intoleranceActivity intolerance Self-care deficit Self-care deficit Ineffective therapeutic regimen Ineffective therapeutic regimen

managementmanagement Disturbed body imageDisturbed body image

Nursing ManagementNursing ManagementPlanningPlanning

Overall goalsOverall goals Satisfactory pain reliefSatisfactory pain relief Minimal loss of functional ability of affected Minimal loss of functional ability of affected

jointsjoints Perform self-carePerform self-care Participate in planning and carrying out Participate in planning and carrying out

therapeutic regimentherapeutic regimen Maintain a positive self-imageMaintain a positive self-image

Nursing ManagementNursing ManagementInterventionsInterventions

Discuss drug actions, side effects, need Discuss drug actions, side effects, need for timely administrations to sustain drug for timely administrations to sustain drug levels and lab monitoringlevels and lab monitoring

Many patients take several different drugs Many patients take several different drugs so the nurse must make the drug regimen so the nurse must make the drug regimen as understandable as possibleas understandable as possible


Nonpharmacologic relief of painNonpharmacologic relief of pain Therapeutic heat and coldTherapeutic heat and cold RestRest Relaxation techniquesRelaxation techniques Joint protectionJoint protection BiofeedbackBiofeedback Transcutaneous electrical stimulation Transcutaneous electrical stimulation HypnosisHypnosis


Lightweight splints may be prescribed to Lightweight splints may be prescribed to rest an inflamed joint & prevent deformityrest an inflamed joint & prevent deformity Should be removed regularly to perform skin Should be removed regularly to perform skin

care and ROM exercises and reapplied as care and ROM exercises and reapplied as prescribed prescribed

OT may help identify additional self-help OT may help identify additional self-help devices to assist in activities of daily livingdevices to assist in activities of daily living

AM care and procedures should be AM care and procedures should be planned around morning stiffnessplanned around morning stiffness

Nursing Management Nursing Management InterventionsInterventions

Morning care and procedures should be Morning care and procedures should be planned around patient’s morning stiffnessplanned around patient’s morning stiffness

Assist with activities to help relieve joint Assist with activities to help relieve joint stiffness: warm shower, warm towels stiffness: warm shower, warm towels around shoulder, etc.around shoulder, etc.

Gentle skin care if bedridden Gentle skin care if bedridden

Ambulatory and Home CareAmbulatory and Home CareRestRest

Alternate scheduled rest periods with Alternate scheduled rest periods with activity throughout dayactivity throughout day Helps relieve pain and fatigueHelps relieve pain and fatigue Amount of rest varies Amount of rest varies

Total bed rest Total bed rest Rarely necessary Rarely necessary Should be avoided to prevent stiffness and Should be avoided to prevent stiffness and

immobilityimmobility

Ambulatory and Home CareAmbulatory and Home CareRestRest

Good body alignment while resting can be Good body alignment while resting can be maintained through use of a firm mattress maintained through use of a firm mattress or bed boardor bed board

Encourage positions of extension Encourage positions of extension Avoid flexion positionsAvoid flexion positions

Splints and casts can help maintain proper Splints and casts can help maintain proper alignment and promote rest alignment and promote rest

Ambulatory and Home CareAmbulatory and Home CareHeat and Cold TherapyHeat and Cold Therapy

Help relieve pain, stiffness, and muscle Help relieve pain, stiffness, and muscle spasmspasm

IceIce

Superficial heat sourcesSuperficial heat sources

Moist heatMoist heat

Ambulatory and Home CareAmbulatory and Home CareExerciseExercise

Inadequate joint movement can result in Inadequate joint movement can result in progressive joint immobility and muscle progressive joint immobility and muscle weaknessweakness

Overaggressive exercise can result in Overaggressive exercise can result in increased pain, inflammation, and joint increased pain, inflammation, and joint damagedamage

Gentle ROM exercises are usually done Gentle ROM exercises are usually done daily to keep joints functionaldaily to keep joints functional

Ambulatory and Home CareAmbulatory and Home CarePsychologic SupportPsychologic Support

Patient’s value system and perception of Patient’s value system and perception of disease must be considereddisease must be considered

Patient is constantly challenged by Patient is constantly challenged by problems of problems of Limited function and fatigueLimited function and fatigue Loss of self-esteemLoss of self-esteem Altered body imageAltered body image Fear of disability or deformityFear of disability or deformity

GoutGout

GoutGout Deposits of sodium urate crystals in Deposits of sodium urate crystals in

articular, periarticular, and subcutaneous articular, periarticular, and subcutaneous tissuestissues

May be primary or May be primary or

secondarysecondary Primary – hereditary error Primary – hereditary error

of purine metabolismof purine metabolism Secondary – drugs that inhibit uric acid Secondary – drugs that inhibit uric acid

excretion or increase rate of cell death or excretion or increase rate of cell death or another acquired disorderanother acquired disorder

Incidence and Risk FactorsIncidence and Risk Factors

Primary gout accounts for 90% of casesPrimary gout accounts for 90% of cases

Affects primarily middle aged menAffects primarily middle aged men

Risk factors: obesity, HTN, thiazide Risk factors: obesity, HTN, thiazide diuretics, excess alcohol usediuretics, excess alcohol use

PathophysiologyPathophysiology

Uric acid is end product of purine Uric acid is end product of purine metabolism and is excreted by the kidneysmetabolism and is excreted by the kidneys

Causes of hyperuricemiaCauses of hyperuricemia

Diet high in purines will not cause gout, Diet high in purines will not cause gout, but may trigger an attack in a susceptible but may trigger an attack in a susceptible personperson

Clinical ManifestationsClinical Manifestations

Gouty arthritis in one or more joints (but Gouty arthritis in one or more joints (but less than four)less than four)

Great toe joint most common first Great toe joint most common first manifestation; other joints may be the foot, manifestation; other joints may be the foot, ankle, knee, or wristankle, knee, or wrist

Joints are tender & cyanoticJoints are tender & cyanotic May be precipitated by trauma, surgery, May be precipitated by trauma, surgery,

alcohol ingestion, or infectionalcohol ingestion, or infection


Onset usually nocturnal, with sudden Onset usually nocturnal, with sudden swelling and excruciating painswelling and excruciating pain

May have low grade feverMay have low grade fever Usually subsides within 2-10 daysUsually subsides within 2-10 days Joints are normal, with no symptoms Joints are normal, with no symptoms

between attacksbetween attacks


Joint deformityJoint deformity

OsteoarthritisOsteoarthritis

Tophi may produce draining sinuses that may Tophi may produce draining sinuses that may become infectedbecome infected

Renal stones, pyelonephritis, obstructive renal Renal stones, pyelonephritis, obstructive renal diseasedisease

DiagnosisDiagnosis

History & physical examinationHistory & physical examination

Clinical symptoms alone are Clinical symptoms alone are sufficient to sufficient to

make accurate DX in most cases make accurate DX in most cases

Family history of goutFamily history of gout

Diagnostic studiesDiagnostic studies


Serum uric acid levelsSerum uric acid levels May be caused by other factorsMay be caused by other factors

WBC elevated during acute attackWBC elevated during acute attack ESR ESR 24 hour urine uric acid levels 24 hour urine uric acid levels Synovial fluid aspiration contains uric acid crystalsSynovial fluid aspiration contains uric acid crystals

Seldom necessarySeldom necessary

X-rays appear normal in early stages; tophi appear X-rays appear normal in early stages; tophi appear as eroded areas of boneas eroded areas of bone


Acute attackAcute attack Colchicine produces dramatic antiiflammatory Colchicine produces dramatic antiiflammatory

effects with relief within 24-48 hourseffects with relief within 24-48 hours NSAIDs for additional pain reliefNSAIDs for additional pain relief Corticosteroids (po or intraarticular)Corticosteroids (po or intraarticular) Adrenocorticotropic hormone (ACTH)Adrenocorticotropic hormone (ACTH) Joint aspiration to decompressJoint aspiration to decompress


Prevention of acute attacksPrevention of acute attacks

Colchicine combined with: Colchicine combined with: • allopurinol (Zyloprim, Alloprim) – blocks production allopurinol (Zyloprim, Alloprim) – blocks production

of uric acid of uric acid • probenecid (Benemid), sulfinpyrazone (Anturane) probenecid (Benemid), sulfinpyrazone (Anturane)

– inhibit tubular reabsorption of uric acid– inhibit tubular reabsorption of uric acid• febuxostat (Uloric) – inhibits xanthine oxidase, febuxostat (Uloric) – inhibits xanthine oxidase,

recently shown to reduce serum uric acid levelsrecently shown to reduce serum uric acid levels


Uricosuric Agents Probenecid (Benemid)Probenecid (Benemid)

PrecautionsPrecautions Sulfinprazone (anturan) block resorption uric acidSulfinprazone (anturan) block resorption uric acid High fluid intake, alkaline urineHigh fluid intake, alkaline urine Not effective if creatinine clearance is elevatedNot effective if creatinine clearance is elevated

Cozaar Angiotension II receptor angonistAngiotension II receptor angonist

• MonitoringMonitoring

Force Fluids while on these medications!!Force Fluids while on these medications!!


Dietary measuresDietary measures Weight reductionWeight reduction Avoidance of alcoholAvoidance of alcohol Avoidance of foods high in purinesAvoidance of foods high in purines

• High: Sardines, anchovies, herring, mussels, liver, High: Sardines, anchovies, herring, mussels, liver, kidney, goose, venison, meat soups, sweetbreads, kidney, goose, venison, meat soups, sweetbreads, beer & winebeer & wine

• Moderate: Chicken, salmon, crab, veal, mutton, Moderate: Chicken, salmon, crab, veal, mutton, bacon, pork, beef, hambacon, pork, beef, ham

Treatment and Nursing CareTreatment and Nursing Care

Bedrest and position for comfortBedrest and position for comfort

Joint immobilization and protect joint from pressureJoint immobilization and protect joint from pressure

Local application of heat or coldLocal application of heat or cold

Assess for complications Assess for complications Formation of kidney stonesFormation of kidney stones HypertriglyceridemiaHypertriglyceridemia HypertensionHypertension


Prevention of renal stonesPrevention of renal stones Increase fluid intake to maintain adequate Increase fluid intake to maintain adequate

urine outputurine output AllopurinolAllopurinol ACE inhibitor losartin (Cozaar) – promotes ACE inhibitor losartin (Cozaar) – promotes

urate diuresisurate diuresis

Nursing CareNursing Care

Acute gouty arthritis – pain controlAcute gouty arthritis – pain control Gentle, supportive care of affected jointsGentle, supportive care of affected joints Immobilize and rest affected joints – bed rest Immobilize and rest affected joints – bed rest

or NWBor NWB Cradle or footboard to prevent pressure from Cradle or footboard to prevent pressure from

bedcoversbedcovers Monitor ROM and degree of painMonitor ROM and degree of pain


Patient/Family teachingPatient/Family teaching Gout is a chronic diseaseGout is a chronic disease

Drug teachingDrug teaching

Need to monitor serum uric acid levelsNeed to monitor serum uric acid levels

Precipitating factorsPrecipitating factors

Systemic Lupus Systemic Lupus Erythematosus (SLE)Erythematosus (SLE)

Chronic multisystem disease involving Chronic multisystem disease involving vascular and connective tissuevascular and connective tissue

IncidenceIncidence

SLE affects 2 to 8 persons per 100,000 in SLE affects 2 to 8 persons per 100,000 in United StatesUnited States

Most cases occur in women of Most cases occur in women of childbearing yearschildbearing years

African, Asian, Hispanic, and Native African, Asian, Hispanic, and Native Americans three times more likely to Americans three times more likely to develop than Whitesdevelop than Whites

EtiologyEtiology

Etiology is unknownEtiology is unknown Most probable causesMost probable causes

Genetic influenceGenetic influence HormonesHormones Environmental factorsEnvironmental factors Certain medicationsCertain medications

SLESLEPathophysiologyPathophysiology

Chronic multisystem inflammatory diseaseChronic multisystem inflammatory disease Associated with abnormalities of immune Associated with abnormalities of immune

systemsystem Results from interactions among genetic, Results from interactions among genetic,

hormonal, environmental, and immunologic hormonal, environmental, and immunologic factors factors

Because the antibodies and accompanying Because the antibodies and accompanying cells of inflammation can affect tissues cells of inflammation can affect tissues anywhere in the body, lupus can affect a anywhere in the body, lupus can affect a variety of areasvariety of areas

Systemic Lupus ErythematosusSystemic Lupus Erythematosus

A variable diseaseA variable disease ChronicChronic UnpredictableUnpredictable Characterized by exacerbations & remissionsCharacterized by exacerbations & remissions


Ranges from a relatively mild disorder to Ranges from a relatively mild disorder to rapidly progressing, affecting many body rapidly progressing, affecting many body systemssystems

Most commonly affects the skin/muscles, Most commonly affects the skin/muscles, lining of lungs, heart, nervous tissue, and lining of lungs, heart, nervous tissue, and kidneyskidneys

Systematic Lupus Systematic Lupus ErythematosusErythematosus

Affects theAffects the SkinSkin JointsJoints Serous membranes Serous membranes Renal systemRenal system Hematologic Hematologic

systemsystem Neurologic systemNeurologic system


Dermatologic Dermatologic Cutaneous vascular lesionsCutaneous vascular lesions Butterfly rashButterfly rash Oral/nasopharyngeal ulcersOral/nasopharyngeal ulcers AlopeciaAlopecia


MusculoskeletalMusculoskeletal Polyarthralgia with morning stiffnessPolyarthralgia with morning stiffness ArthritisArthritis

• Swan neck fingersSwan neck fingers• Ulnar deviationUlnar deviation• Subluxation with hyperlaxity of jointsSubluxation with hyperlaxity of joints


CardiopulmonaryCardiopulmonary TachypneaTachypnea PleurisyPleurisy DysrhythmiasDysrhythmias Accelerated CADAccelerated CAD PericarditisPericarditis


RenalRenal Lupus nephritisLupus nephritis

• Ranging from mild proteinuria to Ranging from mild proteinuria to glomerulonephritisglomerulonephritis

• Primary goal in treatment is slowing the Primary goal in treatment is slowing the progressionprogression


Nervous systemNervous system Generalized/focal seizuresGeneralized/focal seizures Peripheral neuropathyPeripheral neuropathy Cognitive dysfunctionCognitive dysfunction

• DisorientationDisorientation• Memory deficitsMemory deficits• Psychiatric symptomsPsychiatric symptoms


HematologicHematologic Formation of antibodies against blood cellsFormation of antibodies against blood cells AnemiaAnemia LeukopeniaLeukopenia ThrombocytopeniaThrombocytopenia CoagulopathyCoagulopathy Anti-phospholipid antibody syndromeAnti-phospholipid antibody syndrome


InfectionInfection SSusceptibility to infectionsusceptibility to infections Fever should be considered seriousFever should be considered serious Infections such as pneumonia are a common Infections such as pneumonia are a common

cause of deathcause of death


No specific testNo specific test SLE is diagnosed primarily on criteria relating to SLE is diagnosed primarily on criteria relating to

patient history, physical examination, and patient history, physical examination, and laboratory findingslaboratory findings

DiagnosisDiagnosisThese tests are not diagnostic for SLE, but These tests are not diagnostic for SLE, but

help in the diagnosis help in the diagnosis

Autoantibody Testing:Autoantibody Testing: Anti-DNA- specificAnti-DNA- specific Anti- Smith antibody (Anti-Sm) Anti- Smith antibody (Anti-Sm)

These 2 tests ar emost specific to SLEThese 2 tests ar emost specific to SLE Anti nuclear antibody (ANA) – establishes Anti nuclear antibody (ANA) – establishes

presence of autoimmune diseasepresence of autoimmune disease

DiagnosisDiagnosis

Inflammatory Activity:Inflammatory Activity:

* ESR * ESR

* C-reactive protein * C-reactive protein

These tests are helpful in monitoring the These tests are helpful in monitoring the disease activity.disease activity.

Diagnostic TestsDiagnostic Tests

CBCCBC UAUA X-raysX-rays Chest x-rayChest x-ray ECGECG


Prognosis is improved withPrognosis is improved with

Earlier diagnosisEarlier diagnosis Earlier and better treatment regimensEarlier and better treatment regimens Careful monitoring for organ Careful monitoring for organ

involvementinvolvement


Drug therapyDrug therapy NSAIDs - for mild diseaseNSAIDs - for mild disease Antimalarial drugs – for fatigue/skin/joint Antimalarial drugs – for fatigue/skin/joint

problemsproblems Steroid-sparing drugs - Exs: MethotrexateSteroid-sparing drugs - Exs: Methotrexate Corticosteroidsdoes - for exacerbations and Corticosteroidsdoes - for exacerbations and

severe diseasesevere disease Immunosuppressive drugs – Exs: Cytoxan, Immunosuppressive drugs – Exs: Cytoxan,

Imuran; to reduce long term corticosteroid useImuran; to reduce long term corticosteroid use

Nursing ManagementNursing ManagementNursing AssessmentNursing Assessment

Assess patient’s physical, psychologic, Assess patient’s physical, psychologic, and sociocultural problems with long-term and sociocultural problems with long-term management of SLEmanagement of SLE

Assess pain and fatigue dailyAssess pain and fatigue daily Obtain subjective and objective dataObtain subjective and objective data Educate and counsel on expected issuesEducate and counsel on expected issues

Nursing ManagementNursing ManagementNursing DiagnosesNursing Diagnoses

FatigueFatigue Acute painAcute pain Impaired skin integrityImpaired skin integrity Ineffective therapeutic regimen Ineffective therapeutic regimen

managementmanagement Body image disturbance Body image disturbance

Nursing ManagementNursing ManagementNursing ImplementationNursing Implementation

Health promotionHealth promotion Promote early diagnosis and treatmentPromote early diagnosis and treatment

Relieve pain and discomfort and fatigueRelieve pain and discomfort and fatigue Application of heat and coldApplication of heat and cold Encourage to alternate rest and activityEncourage to alternate rest and activity Support devices – braces, splints, firm Support devices – braces, splints, firm

mattressmattress Analgesics and anti-inflammatory medicationsAnalgesics and anti-inflammatory medications


Maintain Skin IntegrityMaintain Skin Integrity Apply topical antiinflammatoryApply topical antiinflammatory Avoid direct sunlightAvoid direct sunlight

Increase MobilityIncrease Mobility ROMROM Assistive devices Assistive devices


Acute interventionAcute intervention During exacerbation, patient will become During exacerbation, patient will become

abruptly, dramatically illabruptly, dramatically ill Record severity of symptoms and response to Record severity of symptoms and response to

therapy therapy Observe forObserve for

• Fever patternFever pattern• Joint inflammationJoint inflammation• Limitation of motionLimitation of motion• Location and degree of discomfortLocation and degree of discomfort• Fatigability Fatigability


Acute intervention (cont’d)Acute intervention (cont’d) Monitor weight and I & O Monitor weight and I & O Collect 24-hour urine sample for protein and Collect 24-hour urine sample for protein and

creatinine clearance creatinine clearance Assess neurological statusAssess neurological status Explain nature of diseaseExplain nature of disease Provide supportProvide support


Ambulatory and home careAmbulatory and home care Reiterate that adherence to treatment does not Reiterate that adherence to treatment does not

necessarily halt progressionnecessarily halt progression Minimize exposure to precipitating factors – Minimize exposure to precipitating factors –

fatigue, sun, stress, infectionfatigue, sun, stress, infection Teach energy conservation and relaxation Teach energy conservation and relaxation

exercisesexercises For joint problems, all the teaching for RA For joint problems, all the teaching for RA

related to joint protection, ROM, and positioning related to joint protection, ROM, and positioning to prevent contracturesto prevent contractures

Treatment and Nursing CareTreatment and Nursing Care

Facilitate self careFacilitate self care Provide adaptive equipment for eating, bathing, Provide adaptive equipment for eating, bathing,

toileting, dressingtoileting, dressing Allow patient extra time to complete careAllow patient extra time to complete care

Improve body imageImprove body image Encourage patient to verbalize feelings, perceptions, Encourage patient to verbalize feelings, perceptions,

and fearsand fears

Monitor for complicationsMonitor for complications Assess for serious adverse effects of medications Assess for serious adverse effects of medications

used in treatmentused in treatment


Lupus and pregnancyLupus and pregnancy InfertilityInfertility

SLE is associated with complications of SLE is associated with complications of pregnancypregnancy

Pregnancy & post partumPregnancy & post partum

Women with serious SLEWomen with serious SLE


Psychosocial issuesPsychosocial issues Counsel patient and family that SLE has good Counsel patient and family that SLE has good

prognosisprognosis Physical effects can lead to isolation, self-Physical effects can lead to isolation, self-

esteem, and body image disturbancesesteem, and body image disturbances Assist patient in developing goalsAssist patient in developing goals

Nursing ManagementNursing ManagementEvaluationEvaluation

Expected outcomesExpected outcomes Completion of priority activitiesCompletion of priority activities Verbalization of having more energyVerbalization of having more energy Expression of satisfaction with pain relief measuresExpression of satisfaction with pain relief measures Performance of activities of daily living without painPerformance of activities of daily living without pain Limitation of direct exposure to sunLimitation of direct exposure to sun No open skin lesionsNo open skin lesions Expression of satisfaction with activity levelExpression of satisfaction with activity level Pacing of activities to match level of tolerancePacing of activities to match level of tolerance Expression of confidence in ability to manage SLE Expression of confidence in ability to manage SLE

over time and in home environmentover time and in home environment

Documents

Autoimmune Disorders That Affect the Musculoskeletal System