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Rheumatic and Autoimmune DisordersRheumatic DiseasesDictionary: Disorders of connective tissue, especially the joints and related structures, characterized by inflammation, degeneration, or metabolic derangement.Affect: Skeletal muscles Bones Cartilage Ligaments Tendons JointsRheumatic DiseasesInflammatory v. non-inflammatoryMonoarticular v. polyarticular-these disorder affect mutiple jointsPathophysiology Synovial Joint Function = movementArticular cartilage = friction free movementSynovial fluid = shock absorberSynovitis = Primary or SecondaryInflammation Antigen stimulusActivation of monocytes “leukocyte that functions in the ingestion of bacteria” and… Activation of T cells “specific kind of white blood cell, active in controlling the immune response and attacking infections” Antibodies form immune complexes with antigens “substance that induces an immune response” Phagocytosis “Ingestion and digestion of bacteria” Inflammatory reaction- joint effusion, pain, edema, swellingInflammationPhagocytosis Leukotrienes & Prostaglandins ↓ Collagenase ↓ Breaks down collagen ↓ Synovial membrane damage, Pannus formation, destruction of cartilage, & erosion of bone. Pannus- forms in the joint affected by the disease, causing loss of bone and cartilagePannus FormationA growth composed of thickened synovium. Forms due leak of fluid. Pannus = “Granulation tissue”Composed of inflammatory cells Erodes articular cartilageLeads to bone destructionOvergrowth of bearing surfaceBreakdown of joint surfaces Steps of Pannus formation:RA begins with synovitis of the affected jointInflammatory chemicals are inappropriately releasedInflammatory blood cells migrate to the joint, causing swellingInflamed synovial membrane thickens into a pannus Pannus erodes cartilage, scar tissue forms, articulating bone ends connectThe end result, ankylosis, produces bent, deformed fingersSystemic InflammationDiffuse Connective Tissue Diseases:Systemic Lupus Erythrematosus- a chronic inflammatory disease affecting many systems of the body. Rheumatoid Arthritis- a chronic, inflammatory, destructive, and deforming collagen disease that has an autoimmune component. It is characterized by inflammation and increased synovial exudate, leading to thickening of the membranes and swelling of the joints. Scleroderma- chronic hardening and thickening of the skin caused by new collagen formation. Polymyalgia rheumatica- a chronic, episodic, inflammatory disease of the large arteries. Joint DegradationSeveral theoriesGenetic or hormonal- strongly link to estrogenMechanical factors- obesityPrior joint damage- trauma to joints are more prone to have problems laterBone stiffening- Joint EffusionsFriction at bone ends damage tiny blood and lymphatic vessels. Blood particles, lymph, and chemical mediators accumulate in the synovial fluid.AKA Synovitis Clinical Manifestations of Rheumatic DisordersPAINSwellingLimited movementStiffnessWeakness/ FatigueAssessmentAssessment 5-6Qs & Allergy 9-10Qs & HIV 10Qs Health historyOnset & evolution of sx FamilyPast hx Contributing factorsHow does it affect lifestyleTreatments attempted… Successful?Physical ExamFunctional assessment (Limp, balance support)Other Systemic AssessmentsSee chart pg 1609 “systemic assessments w/ RA Skin- buising, inflamm of skin, thinning of skin (prednisone), warmth/infected, photo-sensitivityHair- allopecia Eye- grittiness or dryness (lacrimal gland decr)Ear- ringing of the ears, tinnitus, hearing loss, poos from bone stiffening or due to medsMouth- ulcerations, jaw stiffness due to inflam process diff eating/chewing Chest- pleuralitis, inflam of tissueCardiovascular system- pericarditis, Reynauld’s diseaseNeurological- HAs, depression, psycosies Diagnostic StudiesArthrocentesis- joint aspiration, goal is to sample fluid for analysis. This will also decr pain. W/ inflam diseases it’ll look milky, pus-like, bloody. X-raysArthrography- move joint around in diff positions to take a look at it. Giving a functional picture of joint.Bone & Joint scans- bone density and overgrowthTissue Biopsy Blood StudiesESR- Erythrocyte Sedimentation Rate: the rate at which red blood cells settle out in a tube of blood under standardized conditionsCreatinine- incr (due to wide spread inflam indicative of nephritis) because of systemic complicationsRBC, H/H, WBC- blood due to meds (some meds can cause ulcerations)Uric acidANA (Antinuclear Antibody)- very specific to Rheumatic diseases. added:Present in 30% of patients with RA. Rheumatoid factor- An antibody that reacts with Ig (an immunoglobulin) found particularly in patients with rheumatoid arthritis. C-reactive protein- byproduct of inflammation Immunoglobulin electrophoresisDifficult Diagnosis…..Not a clear-cut diagnosis, because s/s are vagueDon’t make diagnostic assumptionsRheumatology Consult, a specialist to help make a better dx Gerontological considerations“Older person’s disease”Secondary concern- arthritis may not be the number one concernDiagnosis difficultiesIncreased risk osteoporosis- due to lack of support for jointsQuality of life – lifestyle changes- laying awake at night worrying, sleeplessnessMedical ManagementPharmacologic:Salicylates- aspirin, not as commonNSAIDs- big help (give w/ food & monitor GI function)DMARDs (Disease Modifying AntiRheumatic Drugs)Will inhibit breakdown of cells. Antimalarials (Plaquenil)- antiarthritic, and lupus erythematosus suppressant agent. Monitor visual changes and skin rashSulfasalazine (Azulfidine)- antibacterial to treat ulcerative colitis and rheumatoid arthritisAntidepressantsManagementNon-pharmacologicalPain Hot, cold (compresses)Devices to protect & support joints (Cane, walker)Exercise, ActivityPassive exercises will be neededNeed to keep moving, even when pain is a factorGoals of ManagementSuppress inflammationSuppress Autoimmune responseControl PainMaintain/improve joint mobility (splints, compresses) Maintain/improve functional status (devices: walker, canes) Increase knowledgePromote self-management (see what works best for them) Nursing DiagnosisAcute & chronic painFatigueDisturbed sleep patternImpaired physical mobilityDisturbed body imageSelf – care deficitsIneffective copingNursing InterventionsRelieving Pain & discomfortPromoting restorative sleepIncreasing mobilityImproving body image & copingMonitoring & managing potential complications- watch out for injury, neuro problemsDischarge PlanningMaintain independencePt teachingMedications (Monitor reaction to meds)Required monitoring for medsSafety/mobilityTeaching self–injection medsDiffuse Connective Tissue DiseasesGroup of Chronic disordersExacerbation “incr in seriousness of disease” ? Remission “partial/complete disappearance”Etiology Unknown Diseases:Rheumatoid Arthritis- chronic inflammation of joints & surrounding tissue Lupus (SLE)- chronic inflammatory disease Scleroderma- chronic hardening and thickening of the skin Polymyositis- inflammation of muscles, accompanied by deformity, edema, insomnia, pain, sweating, and tension Polymyalgia- Many muscle pain Rheumatoid Arthritis (RA)Clinical ManifestationsMore common in womenJoint Pain- usually bil & begins in smaller joints SwellingWarmth & erythemaLoss of functionSpongy or boggy joint tissueBegins with Small Joints!!Acute onsetDeformity hands, feet Extra-articular featuresFatigueFeverWeight lossAnemiaLymph node enlargementRaynaud’s phenomenon- strong link to immune problemsRheumatic nodulesAssociated DisordersArthritis- inflam of arteries (can lead to HAs)Neuropathy- ex: decr sensation in toesPericarditis- tissue surrounding the heart (pericardium) becomes inflamedSplenomegaly- abn enlargement of the spleen Sj?gren’s syndrome- autoimmune disorder in which immune cells attack and destroy the exocrine glands that produce tears and saliva Physical Assessment of RA Bilateral & symmetric stiffnessJoint TendernessJoint SwellingTemperature changes in jointsAssess for extra-articular symptomsDiagnostic Findings with RAElevated Rheumatiod factor- happens in 80% of patients RBC & C4 complement- goes w/ inflammation C-reactive protein- Produced by the liver - present during episodes of acute inflammation. ANA Synovial fluid changes- cloudy, dark X-ray changes- joint space will narrow Stages of Rheumatoid ArthritisEarly- catch early & treat (2yr window for symptom control)Methotrexate- Main med, anti-inflammatory drug w/ immune suppressing effect. Will see alopecia, N/V, wt loss, fatigue.Moderate- erosiveEducate.Persistent- erosiveProvide support, corticoid steroids to prevent inflam. Reconstructive Surgery for repair may be required.Advanced- unremittingImmunosuppressive meds. Pts at this stage may be wheelchair bound.Nursing ManagementDietary assessment- high vitamin/protein/ironManage…WtPainSleep disturbanceFatigueAltered moodLimited mobilityEducate – disease, meds, copingSupport groups may be neededSystemic Lupus Erythematosus (SLE) Immune Regulation Problem: production of autoantibodies.10 times more in women3 times more common in African-AmericansPatho of LupusAbnormal Suppressor T-cell Function ↓ Immune Complex Deposition & Tissue Damage ↓ Inflammation ↓Antigen Stimulation ↓ Antibody Synthesis & Cyclical Pattern Systemic Lupus Erythematosus Cause?? Thought to be due to a combination of ...GeneticHormonalEnvironmental factorsSun exposure can trigger lupus!Certain Meds Pronestyl, hydralazine, isoniazid, Thorazine, some anti-seizure meds.Systemic Lupus Erythematosus Clinical ManifestationsLupus MusculoskeletalArthralgias, arthritis (synovitis)- joint painJoint swelling, tenderness, pain with movement- morning stiffness… big prob w/ these pts.Morning stiffnessClinical ManifestationsLupus Skin lesionsAnnular polycyclic- reddish spots… <image> Discoid lupus lesions- tuff on black women <image> Butterfly rashOral ulcers-Alopecia- hair lossSystemic ManifestationsLupus Pericarditis- the most common disorder in LupusRenal- Creatine levelHypertensionCNS DiseasesNeuro-psychiatric- signs of dementia or mood alterationsDiagnostic Tests for SLESystemic Lupus Erythematosus No one test confirms DiagnosisModerate to severe anemiaThrombocytopeniaLeukocytosis Positive ANAMedical ManagementLupus NSAIDsCorticosteroids- most important med for lupusAntimalarial Meds Immunosuppressive agentsNursing ManagementFatigueImpaired skin integrityBody image concernsLack of knowledge Self managementSclerodermaNo known cure “Hard skin”- excess collagen production under skinEdema (Hard, tight, causing diff of movement) Rare Connective Tissue DiseaseMore common in womenSclerodermaStarts with Raynaud’s & swelling of handsHard, taut, shiny skinDry skinMoves slowly abd can be isolated to one area for yearsLimited mobilityTerm- calcinosis- calcium deposits in the tussues, charateristic sign of SclerodermaAssessment of SclerodermaGeneral physical assessmentSkin changesBody systems assessmentsBig problems Hardening of the esophagusMay see scarring of lungs and stuff intestinal mucosa (causing persistant diarrhea)No conclusive diagnostic testsCRESTCalcinosis, Reynauld’s, Esophageal hardening, Screla of digits, Telangiectasis Treatment of SclerodermaMain goal: decr pain and limit disability Continual assessment of progressionTreat symptomsModerate exercise- to decr stiffnessAvoid temperature extremes- mainly cause of Reynauld’s Use lotion for skin dryness- moist and movableMedications for SclerodermaNONE to delay its progressionCa++ Blockers for Raynaud’s Anti-inflammatories Nursing Diagnoses: SclerodermaImpaired Swallow/Airway is a main focus!Impaired skin integrity- excellent skin care is must! Creams, ointments to keep skin soft, protect from temp extremes Self-care deficitsImbalanced nutrition- Due to swallowing impairmentDisturbed body imagePolymyalgia Rheumatica Severe muscle stiffnessMost common in shoulders, upper arms, neck, hips, & thighsAuto-immune processGiant cell arteritis Spondyloarthropathies Inflammation at sites of bone attachmentTendonsJoint capsulesLigamentsOccurs more commonly in malesArthritis of the sacroiliac joints is commonSpondyloarthropathies Ankylosing spondylitis- spine fixatesAnkylosis = fixationMost common feature: Back painBreathing problems!!!Reactive arthritis- other probs Psoriatic arthritis- other probs Gout Hyperuricemia- over secretion of ***uric acid in bloodIncidence: (picture overwt elderly man) ↑ Age↑ BMIMore common in malesSuspected Cause?Primary hyperuricemia Severe dieting/starvation (***high protein intake)High purine intake (shellfish, red meat)HereditarySecondary hyperuricemia LeukemiaMultiple myelomaAnemiaPsoriasisPatho of GoutIncreases in uric acid levels ↓Urate crystals gather in joints ↓Tophi “lrg gouty nodules due to uric acid accumulation” and kidney stones develop (***Tophi most commonly found in toes) What does this have to do with immune function????Urate crystals obtained during synovial fluid analysis are often covered with IgG antibodies!There is an immunologic link to gout formation.Manifestations of GoutPain and inflammation of jointsTophi Renal impairmentUric acid urinary calculiTreatment of Gout***Decr protein and ETOH consumptionColchicine NSAIDsAllopurinol- ***common medLow purine dietDecrease alcohol consumptionFibromyalgia Chronic fatigueGeneralized muscle achesStiffnessManagement of FibromyalgiaNSAIDsTricyclic antidepressants- for pain and to promote sleepIndividualized exercise plansSupport/encouragementInfectious Arthritis***Infection in the joint space- joint can “shed” and get into blood system Med emergency. Stabilize joint. Common cause:Neisseria gonorrhoeae Staph Aureus StreptococciAcute onset Warm, swollen, painful jointC&S of Synovial fluidIV antibioticsJoint drainage ................
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