Systemic Lupus Erythematosus (SLE)

Systemic Lupus Erythematosus (SLE)Systemic Lupus Erythematosus (SLE)Chronic multisystem inflammatory disease Berhubungan dengan abnormalitas sistem imunDihasilkan dari interaksi genetic, hormonal, environmental, and immunologic factors Systemic Lupus ErythematosusTargetKulitSendiMembran serosaSistem renalSistem hematologiSistem neurologiSystemic Lupus ErythematosusVariasi penyakitChronicUnpredictableCharacterized by exacerbations & remissionsIncidenceSLE affects 2 to 8 persons per 100,000 in United StatesMost cases occur in women of childbearing yearsAfrican, Asian, Hispanic, and Native Americans three times more likely to develop than whitesEtiologyEtiology is unknownMost probable causesGenetic influenceHormonesEnvironmental factorsCertain medicationsEtiologyUnknownAt least 10 gene loci known to riskGenetic predisposition (10x more in monozygotic twins)Human leukocyte Ag: HLA-DR2, HLA-DR3 + HLA-B8Null complement alleles + congenital complement (esp C4, C2 etc)PathophysiologyAutoimmune reactions directed against constituents of cell nucleus, DNAAntibody response related to B and T cell hyperactivityPathophysiologyProposed mechanism for autoantibodies:Defect in apoptosiscell death disturbance in immune tolerancePlasma + nuclear antigens displayed on cell surfaceDysregulated lymphocytes target Ag (normally intracellular)Immune complexes form in microvasculature complement activation + inflammation Ag-Ab complexes deposit in basement membranes of skin and kidneysFig 65-9

Clinical ManifestationsClinical ManifestationsDermatologic Cutaneous vascular lesionsButterfly rashOral/nasopharyngeal ulcersAlopeciaDermatologic ManifestationsFig 65-10

Clinical ManifestationsMusculoskeletalPolyarthralgia with morning stiffnessArthritisSwan neck fingersUlnar deviationSubluxation with hyperlaxity of joints

Swan Neck DeformityFig. 65-4 D

Clinical ManifestationsCardiopulmonaryTachypneaPleurisyDysrhythmiasAccelerated CADPericarditisClinical ManifestationsRenalLupus nephritisRanging from mild proteinuria to glomerulonephritisPrimary goal in treatment is slowing the progressionClinical ManifestationsNervous systemGeneralized/focal seizuresPeripheral neuropathyCognitive dysfunctionDisorientationMemory deficitsPsychiatric symptomsClinical ManifestationsHematologicFormation of antibodies against blood cellsAnemiaLeukopeniaClinical ManifestationsHematologic (contd)ThrombocytopeniaCoagulopathyAnti-phospholipid antibody syndromeClinical ManifestationsInfectionIncreased susceptibility to infectionsFever should be considered seriousInfections such as pneumonia are a common cause of deathDiagnostic StudiesNo specific testSLE is diagnosed primarily on criteria relating to patient history, physical examination, and laboratory findingsDiagnostic Studies

HistoryConstitutional fatigue, fever, weight lossSkin malar rash, photosensitive, discoid lupus, alopecia, Raynaud phenomenon, livido reticularisMusculoskeletal arthralgia, myalgia, arthritisRenalNeuropsychiatric headache, mood disorders, cognitive disorders, psychosis, seizures, TIA/ stroke, movement disorders, mononeuritisPulmonary chest pain, dyspnoeaGastrointestinal Abdominal pain, jaundiceCardiac heart failure/chest painHaematological multiple cytopeniasOther miscarriages, family history of autoimmune diseaseOn examinationConstitutional lymphadenopathy, hepatosplenomegalyMusculoskeletal Jaccoud arthropathyDermatologic - capillaroscopyRenalNeuropsychiatricCardiopulmonary friction rubs, pulmonary embolism, Libman-Sacks endocarditisGIT peritonitis, pancreatitis, mesenteric vasculitisDiagnostic criteriaAmerican College of Rheumatology4/11 criteria (sens 85%, specif 95%)SOAP BRAIN MDSerositis heart, lung, peritoneumOral ulcers painless esp palateArthritis non-erosivePhotosensitivity

Diagnostic criteria continuedBlood disorders - RBC (Coombs +), PLT, WCC, LymphocytesRenal involvement proteinuria / castsANA titer > 1:160Immunologic phenomena LE cells, anti-dsDNA Ab, anti-Sm Ab, antiphospholipid Ab, false WR +Neurological disorders seizures/ psychosisMalar rash cheeks + nasal bridgeDiscoid rash rimmed with scaling, follicular pluggingDiscoid rash

Malar rashFig 65-10

Laboratory studies High clinical suspicion/ high ANA titresSLE Screen:FBC and diffS-creatinineUrinalysis with microscopyBasic inflammatory markersAntibodies to dsDNAComplement ANA subtypes (anti-Sm, Ro, La, RNP Abs)Autoantibody tests used in SLEANA screening test (95% sensitivity)Anti-dsDNA (high specifcity, sens 70%)Anti-Sm (most specific Ab for SLE, 30% sens)Anti-Ro/anti-La (15% in SLE patients, neonatal disease)Anti-ribosomal P (uncommon, assoc lupus cerebritis)Anti-RNP (overlap)Anticardiolipin (antiphospholipid Ab syndrome)Lupus Anticoagulant (antiphospholipid Ab syndrome)Coombs test (Ab on RBCs)Anti-histone (drug-induced lupus)Radiological studiesJoint x-rays: no erosions, periarticular osteopenia + soft tissue swellingCXR/CT chest: interstitial lung disease, pneumonitis, pulmonary emboli, alveolar hemorrhageCTBrain or Brain MRI angiography: lupus white matter changes, vasculitis or strokeEcho: pericardial effusion, pulmonary hypertension or Libman-Sacks endocarditisInvasive proceduresLP nonspecific cells + protein, glucoseRenal biopsy prognosis and RxSkin biopsyLupus nephritis

Class IMinimal mesangialNormal light microscopy; abnormal electron microscopyClass II

Mesangial proliferativeHypercellular on light microscopyClass IIIFocal proliferative50% glomeruli involved; segmental/globalClass V

MembranousPredominantly nephrotic diseaseClass VI

Advanced sclerosingChronic lesions and sclerosisDifferential diagnosisDrug induced lupus erythematosisVasculitisLeukemiaHIVMultiple sclerosisParvovirus or other viral infectionsTreatment principlesDepends on disease severityFever, skin, musculoskeletal and serositis = milder diseaseCNS and renal involvement aggressive RxEmergencies: - severe CNS involvement - systemic vasculitis - profound thrombocytopenia (TTP-like syndrome) - rapidly progressive nephritis - diffuse alveolar hemorrhageMedications usedNSAIDSChloroquineSteroidsCyclophosphamideAzathioprineMycophenolate(Rituximab)Plasma exchange/ IVIGPreventive careMedication-related (steroid) complications (Ca, vit D, bisphosphonates)Aggressive BP and lipid control Immunization (complement deficient)Stress-dose steroid protocols for patients on maintenance corticosteroids (surgery/ infection)Avoid UV exposureAvoid estrogen therapiesAvoid sulfa-containing medicationsPregnancy planning

PrognosisBenign to rapidly progressiveBetter for isolated skin + musculoskeletal disease vs renal and CNSDeath rate 3X age-comparable general population

Mortality

Nephritis (most within 5 yrs of symptoms)Infectious (active SLE + Rx most common)CVS disease (50X more MI than other woman)Malignancy (chronic inflammation + Rx)Collaborative CareDrug therapyNSAIDsAntimalarial drugsSteroid-sparing drugsCorticosteroidsImmunosuppressive drugsNursing ManagementNursing AssessmentAssess patients physical, psychologic, and sociocultural problems with long-term management of SLEAssess pain and fatigue dailyNursing ManagementNursing AssessmentObtain subjective and objective dataEducate and counsel on expected issuesNursing ManagementNursing DiagnosesFatigueAcute painImpaired skin integrityIneffective therapeutic regimen managementBody image disturbance Nursing ManagementPlanningOverall goalsHave satisfactory pain reliefComply with therapeutic regimen to achieve maximum symptom management

Nursing ManagementPlanningOverall goals (contd)Demonstrate awareness of, and avoid activities that cause disease exacerbation (triggers)Maintain optimal role function and a positive self-imageNursing ManagementNursing ImplementationHealth promotionPrevention of SLE is not possiblePromote early diagnosis and treatmentNursing ManagementNursing ImplementationAcute interventionDuring exacerbation, patient will become abruptly, dramatically illRecord severity of symptoms and response to therapy Nursing ManagementNursing ImplementationAcute intervention (contd)Observe forFever patternJoint inflammationLimitation of motionLocation and degree of discomfortFatigability Nursing ManagementNursing ImplementationAcute intervention (contd)Monitor weightCollect 24-hour urine sampleAssess neurological statusExplain nature of diseaseProvide supportNursing ManagementNursing ImplementationAmbulatory and home careReiterate that adherence to treatment does not necessarily halt progressionMinimize exposure to precipitating factors fatigue, sun, stress, infection, drugsNursing ManagementNursing ImplementationAmbulatory and home careTeach energy conservation and relaxation exercisesFor joint problems, all the teaching for RA related to joint protection, ROM, and positioning to prevent contracturesNursing ManagementNursing Implementation

Nursing ManagementNursing ImplementationLupus and pregnancyInfertility can result from SLE treatment regimenSLE is associated with complications of pregnancyPregnancy & post partum can cause exacerbations of SLEWomen with serious SLE should be counseled against pregnancy

Nursing ManagementNursing ImplementationPsychosocial issuesCounsel patient and family that SLE has good prognosisPhysical effects can lead to isolation, self-esteem, and body image disturbancesAssist patient in developing goalsNursing ManagementEvaluationExpected outcomesCompletion of priority activitiesVerbalization of having more energyExpression of satisfaction with pain relief measures

Nursing ManagementEvaluationExpected outcomes (contd)Performance of activities of daily living without painLimitation of direct exposure to sun and use of sunscreen No open skin lesions

Nursing ManagementEvaluationExpected outcomes (contd)Expression of satisfaction with activity levelPacing of activities to match level of toleranceExpression of confidence in ability to manage SLE over time and in home environment