Systemic Lupus Erythematosus

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Systemic Lupus Erythematosus. Systemic Lupus Erythematosus. Autoimmune disease Organs and cells undergo damage mediated by tissue-binding autoantibodies and immune complexes 90% are women of child-bearing years In the US: 15-50 per 100,000 highest prevalence in African Americans. - PowerPoint PPT Presentation

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Systemic Lupus Erythematosus

Systemic Lupus Erythematosus1Systemic Lupus ErythematosusAutoimmune diseaseOrgans and cells undergo damage mediated by tissue-binding autoantibodies and immune complexes

90% are women of child-bearing yearsIn the US: 15-50 per 100,000highest prevalence in African AmericansSLE is an autoimmune disease in which organs and cells

While 90% of patients are women of child-bearing years, people of both sexes, all ages and ethnic groups are susceptible.2

Interactions between susceptibility genes and environmental factors result in abnormal immune responses.3Pathogenesis(1) activation of innate immunity (dendritic cells) by CpG DNA, DNA in immune complexes, and RNA in RNA/protein self-antigens;(2) lowered activation thresholds of adaptive immunity cells (antigen-specific T and B lymphocytes);(3) ineffective regulatory and inhibitory CD4+ and CD8+ T cells;(4) reduced clearance of apoptotic cells and of immune complexesThese abnormal immune responses include:4

In summary, SLE is a multigenic disease. All these gene combinations influence immune responses to the external and internal environment; when such responses are too high and/or too prolonged, autoimmune disease results

gene-environment interactions result in abnormal immune responses that generate pathogenic autoantibodies and immune complexes that deposit in tissue, activate complement, cause inflammation, and over time lead to irreversible organ damage5The CaseE.G., 21 FemaleCame in with a chief complaint of SEIZURESThe Case to manageA diagnosed case of systemic lupus erythematosus since December 2008

Initially presented withmalar rash, photosensitivity, arthralgia, hair loss and oral ulcers, edema of both lower and upper extremities, weakness-> was ANA (+)The Case to manageMaintained on

prednisone 10 mg once a day

hydrochloroquine 200 mg once a dayThe Case to manageRegular follow up in the OPD (Fammed and Rheuma)non-hypertensivenon-diabeticnon-asthmaticThe Case to manageCurrently pregnant(PU 14 1/7 weeks AOG, G1P0)

on follow-up at the PGH OB HRC, last seen February 17, 2010The Case to manage2 WEEKS PTA

(+) decreased appetite

(+) gradually increasing generalized body weakness

(-) interventions or consultsThe Case to manage4 DAYS PTA

generalized weakness now more prominent on both right upper and lower extremities(+) behavioral change (+) general decrease in activity (+) episodes of staring blankly into space(+) cough productive of whitish phlegm(+) febrile episodes (qualitative)(-) nasal congestionThe Case to manage2 HOURS PTA

(-) rousability(+) seizure - upward rolling of the eyeballs, grinding of teeth and clenched fists (duration ~5 minutes)

-> Promptly rushed to PGH, hence this admissionGeneral(+) fever, (-) nausea and vomiting, (+) weakness, (+) weight loss Integumentary(+) malar rash, (-) discoid rash,(-) hairlossEyes() blurring of visionEars() loss of hearing, tinnitusNervous() dysphagia, dysphonia, seizures, dizziness, (+) headache priorRespiratory(+) cough, (+) exertional dyspnea, (-) coldsCirculatory() chest pain, (-) bleedingDigestive(-) melena, (-) constipation, (-) diarrhea, (-) abdominal painUrinary/ Reproductive System (-) urinary incontinence, (-) polyuria/nocturia, dysuria (-) dribbling, (-) tea colored urine, (+) vaginal spotting (Feb 14, 2010)Endocrine System() heat intolerance, (-) polyphagia, polydipsia, polyuriaJoints() tremors, (+) joint painReview of SystemsPAST MEDICAL HISTORY (+) SLE, 2008 (-) HPN, DM, goiter, BA, PTB, Ca, (-) heart problems (-) allergy

FAMILY MEDICAL HISTORY(-) SLE, HPN, DM, goiter, BA, Ca, heart problemsPERSONAL/SOCIAL HISTORYPt lives with live-in partner, a college graduate (BA HRM), currently unemployed.Non-smoker, occasional alcoholic beverage drinker.(-) known exposure to chemicals, and radiation

OBSTETRIC HISTORYG1P0 LMP: October 9, 2009 , PU 14 1/7 weeks AOG by LUTZGeneral Survey: Pt is awake, with regard, responds to name calling, inconsistently follows commands, with no verbal output.

Vital Signs: BP 100-110/60-70 HR 142RR 40Temp 38C HEENT:(+) slight exophthalmos, anicteric sclerae, pink palpebral conjunctivae, (-) TPC, ANM, (-) NVE, (-) CLADChest/Lungs:ECE, (+) bibasal and mid-field rhonchi, both lung fieldsCVS: AP, DHS, tachycardic, regular rhythm, (-) murmursAbdomen: flat, normoactive bowel sounds, soft, (-) masses, (-) tenderness, liver edge not palpableGU/IE: DeferredSkin/Extremities: FEP, PNB, (-) edema/cyanosis, (+) blanching erythematous rash over extremitiesPhysical ExaminationNeuro Pt is awake, with regard, responds to name calling, inconsistently follows commands, with no verbal output, uncooperative. CN: I not assessedII pupils 2/2 EBRTL, primary gaze midline, (-) preferential gazeIII, IV, VI (+) slight LR palsy, R; (+) visual threat, BV brisk cornealsVII (-) facial assymetryVIII gross hearing intactIX, X good gag and swallow XII refuses to protrude tongue With spontaneous, purposeful movement of the extremities.Withdraws to pain, B ext. (-) Babinski (-) clonuspatient resists neck flexion.(-)dysmetria/dysdiadochokinesia(-) nystagmusMOTOR Strength: 3/5 on all extremities Sensory: WTP on all extremities Physical ExaminationDate 02/28 InterpGlucose 5.83 NBUN --Creatinine 72 NSodium 134 Sl lowPotassium 3.5 HypokalemiaChloride 100 NormalDate 02/28 Protime Ctrl 12.4 Patient 9 Activity >1 INR 1 Date 02/28 Control 36.1 Patient 43.1 Px:Ctrl Ratio 1.1919Date Normal 2/28 WBC 4-11x109/L 14.1 LeucocytosisRBC 4-6x109/L 3.55 Low Hgb 120-180g/L 103 LowHct 0.370-0.540% 0.291 LowMCV 80-100fL 81.8 normalMCH 27-31pg 29.1 NormalMCHC 320-360g/L 356 NormalRDW-CV 11-16 L 15.2 LowPlatelets 2-4x1011/L CLUMPING then 94 Neut% 0.5-0.7 0.764 elevatedLymph% 0.2-0.5 0.197 nMono% 0.02-0.09 0.036 nEo% 0.0-0.06 0.002 nBaso% 0.0-0.02 0.001 NNormocytic normochromic anemia prob from chronic disease rule out IDABicytopenia: erythrocyopenia thrombocytopeniaLeukocytosis with neutrophilic predominance : evidence of acute infection20Date Normal 02/28 FiO2 60 Temp Hb pH 7.35-7.45 7.429 PCO2 35-45mmHg 26.4 PO2 90-100mmHg 63.5 HCO3 22-28mEq/L 17.9 TCO2 Beb O2 satn 92.9 Uncompensated respiratory alkalosis21Date Normal 02/28 Color Yellow dark yellow Transparency Clear/hazy sl turbid SG 1.016-1.022 1.02 PH 4.6-6.5 6 Sugar (-) (-) Albumin (-) +++ RBC 0/0-2/hpf 1-2 WBC 0-2/0-5/hpf 15-25 Casts hyaline, coarse, fine, granular, RBC, WBC, waxy 8-25 hyaline casts 0-2 waxy casts 2-4 coarse granular casts Crystals Small amounts Epith cells Small amounts occasional Bacteria (-) 2+ Mucus thr Small amounts rare RBC morph (+) proteinuriaWBC increased, evidence of infectionPresence of bacteriacasts22ECG2/28: sinus tachycardia, normal axis, low voltage complexes3/11: sinus tachycardia, normal axis, low voltage complexes3/12: sinus tachycardia, normal axis, low voltage complexes

Plasma K7.6

2D ECHO: concentric LVH with good wall motion and contractility, EF=73% minimal pericardial effusion, mild pulmo HPN, incidental finding of pleural effusion, mild TR, PR.

CRANIAL CT enhanced: unremarkableSystemic lupus erythematosus in activityt/c SLE cerebritis, myocarditist/c sec APASCommunity Acquired Pneumoniar/o SOL vs electrolyte imbalance vs CNS infection as cause of seizureAnemia of chronic diseaseComplicated UTIPresent Working ImpressionDiagnosis

?Diagnostic Criteria for Systemic Lupus Erythematosus

If 4 of these criteria, well documented, are present at any time in a patient's history, the diagnosis is likely to be SLE. Specificity is 95%; sensitivity is 75%.

Histologic abnormalities in blood vessels may also determine therapy. Patterns of vasculitis are not specific for SLE but may indicate active disease: leukocytoclastic vasculitis is most common

Lymph node biopsies are usually performed to rule out infection or malignancies. In SLE, they show nonspecific diffuse chronic inflammation.

Antinuclear antibodies (ANA) are positive in >98% of patients during the course of disease; repeated negative tests suggest that the diagnosis is not SLE, unless other autoantibodies are present.

High-titer IgG antibodies to double-stranded DNA and antibodies to the Sm antigen are both specific for SLE and, therefore, favor the diagnosis in the presence of compatible clinical manifestations.

The presence in an individual of multiple autoantibodies without clinical symptoms should not be considered diagnostic for SLE, although such persons are at increased risk since clinical SLE begins in most patients years after autoantibodies appear

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Algorithm for diagnosis and initial therapy of SLE26Systemic ManifestationsSeverity varies from mild and intermittent to severe and fulminantExacerbations interspersed with periods of relative quiescencePermanent complete remissions (absence of symptoms with no treatment) rareSystemic symptoms, particularly fatigue and myalgias/arthralgias present most of the timeSevere systemic illness requiring glucocorticoid therapy can occur with fever, prostration, weight loss, and anemia with or without other organ-targeted manifestations.Musculoskeletal

Clinical Manifestations of SLE and Prevalence over the Entire Course of Disease

intermittent polyarthritis, varying from mild to disabling, characterized by soft tissue swelling and tenderness in joints, most commonly in hands, wrists, and knees. Joint deformities (hands and feet) develop in only 10%. Erosions on joint x-rays are rare; their presence suggests a non-lupus inflammatory arthropathy such as rheumatoid arthritisIf pain persists in a single joint, such as knee, shoulder, or hip, a diagnosis of ischemic necrosis of bone should be considered, particularly if there are no other manifestat