Musculoskeletal: Autoimmune; Inflammatory; Metabolic; Infectious DisordersRheumatoid Arthritis; SLE; Pagets Disease; Gout; OsteomyelitisCarolyn Morse Jacobs, RN, MSN, ONC 10/ 24/ 04

Autoimmune and Inflammatory Disorders:Rheumatoid Arthritis

Chronic systemic, inflammatory disease characterized by recurrent inflammation of connective tissue, primarily of joints (diarthroidal) and related structures.

Pathophysiology Rheumatoid Arthritis

Normal antibodies (immunoglobulins) become autoantibodies and attack host tissues (RF)Neutrophils, T cells synovial fluid cells acitavted;Cystokines, interleukin-1 and TNR (tumor necrosing factor) alpha; chrondroytes attack cartilage;Synovium digests cartilage; inflammatory molecules released containing interleukin-1 and TNF alpha

Pathophysiology: Rheumatoid Arthritis

Pathophysiology

IgG/RF (HLA)= antigen-antibody complexPrecipitates in synovial fluidInflammatory responseCartilage connective tissue primarily affected!

Rheumatoid arthritis: assessment: manifestations and complicationsFatigue, weakness, painJoint deformityRheumatic nodulesMultisystem involvement

How does Rheumatoid Arthritis Compare to Osteoarthritis?Definition: *wear and tear, progressive, non-systemic, Degenerative Joint Disease (DJD)

Pathophysiology

Identify which joints are primarily affected with osteoarthritis.What factors contribute to the development of osteoarthritis?Osteoarthritis (top slide only)Ankylosing spondylitis (what type of arthritis is this?)

Structural changes with OsteoarthritisEarly Cartilage softens, pits, fraysProgressiveCartilage thinner, bone ends hypertrophy, bone spurs develop and fissures formAdvancedSecondary inflammation of synovial membrane; tissue and cartilage destruction; late ankylosis

Normal Knee structureModerately advanced osteoarthritisAdvanced osteoarthritisWhat signs and symptoms does the person with osteoarthritis experience?

What symptoms/assessment for the patient with osteoarthritis? Onset of pain is insidious, individual is healthy!Pain is aching in nature; relieved by rest!.Local signs and symptoms: swelling, crepitation of joint and joint instability, asymmetrical joint involvement

Deformities with OsteoarthritisGenuvarusHerberdens nodesCarpometacarpocarpal joint of thumb with subluxation of the first MCP

Osteoarthritis (review only)Diagnostic TestsNone specificLate joint changes, boney sclerosis, spur formationSynovial fluid inc., minimal inflammationGait analysisNursing diagnosisInterventions determined by complicationsSupportive devicesMedications (no systemic treatment with steroids)Dietary to dec. wt.Surgical Intervention (joint replacement)Teaching

Comparison of RA and OA

RACause unknown; auto-immune factorOnset suddenRemissions*Body parts affected, systemic, small joints, symmetricalCauses redness, warmth, swelling of jointsFemales, age 20-30; 3-1 ratioOACause wear and tear, develops slowly Non-systemic, weight bearing jointsMiddle-aged and elderly, males 2-1 affectedDoes not cause malaiseBegins after 40

Manifestations of RASystemically illHematologicPulmonary/CVNeurologicOcular symptoms (Sjorgens)SkinMusculoskeletal deformity, painPain!Pain!Pain

Joints changes with RA Early PannusGranulation, inflammation at synovial membrane, invades joint, softens and destroys cartilage

Mod advanced Pannusjoint cartilage disappears, underlying bone destroyed, joint surfaces collapse

Fibrous AnkylosisFibrous connective tissue replaces pannus; loss of joint otion

Bony AnkylosisEventual tissue and joint calcificationRA

Joint Changes RABilateral, symmetrical, PIPs, MCPsThumb instabilitySwan neck, boutonniere deformityTensynovitisMultans deformitySubcutaneous nodulesGenu valgumPes plano valgusProminent metatarsal headsHammer toes

Assessment RADeformities that may occur with RASynotenovitisUlnar driftSwan neck deformityBoutonniere deformity

Mutlans deformity (rapidly progressing RA)Hitch-hiker thumbGenu valgus

Subcutaneous nodules (disappear and appear without warning)

Hammer toes

Diagnostic Tests RAESR elevated

+ RA, ^ RA titer

Sed rate increased

CBC

C-reactive protein

Dec. serum complement

Synovial fluid inflammation

Joint and bone

Swelling,inflammation

Interventions RANursing DiagnosisComfortPhysical mobilitySelf imageGoalsTeam ApproachPain managementExerciseSurgeryTeaching

Medications RAASA & NSAIDSCorticosteroids; low doseDMARDs (diverse group) of remitting agents: including antimalarial (hydroxychloroquine:plaquenil) *eye effects; Penicillamine (empty stomach); Gold (Auranofin) *dermatitis, blood dyscrasia; and Immunosuppressive agents as methotrexate and cyclosporineBiologic response modifiers Adalimumab (Humira)Infliximab (Remicade)

Joint Protection: Dos and Donts

Case Presentation; Mrs. Michaels with Rheumatoid Arthritis(PDS: Adult Health: Musculoskeletal Health: Mrs. Michaels)Comparison to usual courseDiagnostic testsNursing diagnosisTherapiesMedications usedExerciseJoint ProtectionResources on the Web

Systemic Lupus Erythematous (SLE)Chronic multisystem disease involving vascular and connective tissueLupus Foundation

Etiology and Pathophysiology SLEExact etiology unknown: genes (HLA), hormones, environment involvedFormation auto-antibodies; immune complexes depositedInflammatory response triggered by deposition of immune complexes (kidney, brain)Drug induced syndrome similar to SLE (Procan-SR, hydralazine, isonaiazid)Pneumonia, infections, *renal, CNS involvement!; Course of disease variesMildEpisodicRapidly fatal

Manifestations/Complications of SLETypes: Discoid, SLEIncidence: 1:2000Women 1-9, child-bearing age, african americans Periods remission and exacerbationStress Environmental factors AssessmentLow grade feverIntegumentary MS involvementCVRespiratoryUrinary Renal failureNeurologic CNSGI HematologicEndocrineReproductive

Characteristic butterfly rash associated with SLE, especially discoid lupus erythematousBarrys lupus

SLE characterized by periods of remission and exacerbation. Stimulated by sunlight, stress, pregnancy, infections like strep and some drugs. Some drugs like apresoline, pronestyl, dilantin, tetracycline, phenobarbital may cause a lupus-like reaction which disappears when drug is stopped.

Diagnostic TestsLE cellAnt-DNAANA, titerAnti-DNAComplement fixation decreasedESROther (and CBC, UA)Kidney biopsyCriteria to Dx.malar, discoid rashphotosensitivityarthritisrenal disorderimmunological disorderDNA, ANA

Therapeutic Interventions/Management SLENursing diagnosisSee RAImpaired skin integrityIneffective protectionImpaired health maintenance

Goal: control inflammation

Emotional support

Life Planning

Required ReviewMedicationsNSAIDS(Disease modifying agents) Antimalarial drugsCorticosteroidsImmunsuppressive therapy

Antineoplastic drugs such as Imuran, cytoxan, cyclosporine

Avoid UV Reduce stressMonitor/manage to prevent complications

Case StudyClinical Background:

18 year old patient admitted with recent onset (3 months earlier) of malar rash and constitutional symptoms (weakness and malaise), now symptoms of renal failure. She reported having a 5-year history of Raynaud's phenomenon and arthralgia. Abnormal results of laboratory studies included the presence of ANA, anti-nDNA, anti-SS-A autoantibodies and a proteinuria of approximately 10 g/d. Renal biopsy revealed a Class IV lupus glomerulonephritis.

What assessment data is priority: what additional date should you collect?

What are the priority nursing problems? What are the priority interventions?What medications are typically used and why?

Scleroderma (Systemic sclerosis)Definition: progressive sclerosis of skin and connective tissue; fibrous and vascular changes in skin, blood vessels, muscles, synovium, internal organs. become hide boundImmune-mediated disorder; genetic component

Scleroderma (Systemic sclerosis)Abnormal amounts of fibrous connective tissue deposited in skin, blood vissels, lungs, kidneys, other organsCan be systemic or localized (CREST) syndrome

CREST SyndromeCalcinosisRaynauds phenomenaEsophageal hypomotilitySclerodactyl (skin changes of fingers)Telangiectasia (macula-like angioma of skin)More on CREST

Crest Syndrome

CREST Syndrome & sclerodermaSclerodactyl (localized scleroderma of fingers)Raynauds disease with ischemia

Typical hide-bound face of person with sclerodermaTissue hardens; claw-like fingers; fibrosis

Manifestations & Complications (systemic)Female 4:1Pain, stiffness, polyartheritisNausea, vomitingCoughHypertensionRaynaulds syndrome Skin atrophy, hyperpigmented

Scleroderma cont.Esophageal hypomotility leads to frequent refluxGI complaints commonLung-pleural thickening and pulmonary fibrosisRenal disease...leading cause of death!

Diagnosis/Treatment SclerodermaR/O autoimmune diseaseRadiological: pulmonary fibrosis, bone resorption, subcutaneous calcification, distal esophageal hypomotilityESR elevatedCBC anemiaGammaglobulin lelels elevaed; RA presentSkin biopsy to confirmWhat are the KEY components of care for the individual with Scleroderma?

Scleroderma: Patient CareDosAvoid coldProvide small, frequent feedingsProtect fingersSit upright post mealsNo fingersticksDaily oral hygieneResources

Scleroderma: Patient CareMedications: based upon symptoms:Immunosuppressive agents & steroids & remitting agentsCa channels blockers & alpha-adrenergic blockersH2 receptor blockersACE inhibitorsBroad spectrum antibiotics

Ankylosing SpondylitisDefinitions: chronic inflammatory polyarteritis of spineAffects mostly young men Associated with HLA-B27 antiget positive antigen (90%)Pathophysiology & ManifestationsLike arthritis have inflammatory changes; erosion of cartilage, ossification of joint margins; scar tissue replacesMorning backache, flexion of spine, decreased chest expansionDiagnosisESR elevationPositive HLA-B27 antigenVertebral changes

Ankylosing SpondylitisInsidious onsetMorning backacheInflammation of spine; later spine ossification

Oh my back hurts!

Comparison of changes with ospeoporosis and Ankylosing spondylitisIdentify a PRIORITY nursing concern related to ankylosing spondylitis

Management Ankylosing Spondilitis

DosMaintain spine mobilityPain managementProper positioningMeds for pain, inflammation

Other Collagen DiseasesReiters SyndromeReactive arthritis associated with enteric disease

Lyme Disease (mimics rheumatoid disease)Caused by spirochete, borrelia burgdorferi (tick)Inflammatory disorder3 stagesInitial rash (target)Disseminated (arthitic like symptoms)Late (neurologic symptoms)DiagnosisCulture (difficult)Antibody detection

Treatment: antibiotics (amoxicillin, vibramycin, tetracycline, etc); NSAID)Prevention

Other Collagen DiseasesPolymyositis

Systemic connective tissue disorder characterized by inflamation of connective tissue and muscle fibersAutoimmune; affecting women 2:1If muscle fiber inflammation is accompanied by skin lesion disease known as dermatomyositisManifestations & complicationsMuscle pain, tenderness, rash; arthralgias; fatigue; fever and weight loss;Skeletal muscle weakness most prominentDusky red rash over faceRaynauds phenomenonMalignancy with dermatomyositisDiagnosisNone specificElevated CK

Other Collagen Diseases contReiters syndromeSelf-limited disease of reactive arthritis such as shingles, venereal diseas, associated with HLAB27 antigen

Polyarteritis NodosaCollegan; diffuse inflammation and necrosis of wall of small to medium sized arteries especially in muscles , kidneys, heart liver, GI and peripheral nerves like SLEJuvenile Rheumatoid Arthritis