SLE Year 4 Internal Medicine Notes OmpathStudy

SLE Year 4 · Internal Medicine Collection: 4TH YEAR ALL NOTES / IMED / IMED 4.3 / NGUGI -- Slide 1 Systemic Lupus Erythematosus and Neuropsychiatric Disease DR. ROSSLYN NGUGI Slide 2 Case 1 40 yo African-American F diagnosed with SLE x 6 mos - 2006 SLE features leading to dx: polyarthritis, +ANA, +anti-DNA, rash, fatigue, cytopenia 2007 presented with fever, muscle pain, malaise, overwhelming depressive sx for 2 wks WBC 3000, Hb 11.0; ESR 90 urinalysis 2+protein, 1+ RBC seems very withdrawn dialysis required; depressive sx progress Slide 3 Case 2 15 yo Hispanic F with no past hx presents to ED in 2006 for 2nd time in the past month 6 weeks of recurrent fevers, HA, fatigue on exam, temp 39.0; BP 145/85; HR 100 obviously in pain with photophobia from HA no rash tender joints on exam no edema Labs:\nWBC 2500; Hemoglobin 7.5 platelets 140,000 ESR 80 mm/h vision deteriorates; HA continues Slide 4 Case 3 36 yo white F with SLE x 20 yrs historical SLE features:\nchorea, arthritis, WBC 2.0-3.0, low platelets; adrenal insufficiency, + ACL Ab age 31, after 2 miscarriages, delivered 30 wk baby peri-partum: SLE flare with rash, arthritis, low platelets 6-9 months post-partum: notes gradual memory difficulties short term memory, work with #s, sequencing tasks Slide 5 Case 4 30 yo Asian F with SLE x 15 yrs historical SLE features:\nrash, arthritis, cytopenias: WBC 2.5-4.5, Hb 10-11, platelets 150,000\nAVN both hips, + DNA Ab, ESR 30-50 presents in 2001 with fever, severe malaise, muscle and joint pain, HA, tingling feet WBC 2,000, Hb 10.0, ESR 80,creatinine 0.7 rapidly progressive weakness of legs over 2 days in ICU Slide 6 Objectives review the epidemiology of SLE discuss the criteria of SLE discuss organ system involvement and tailored treatment of SLE\nfocus on the various neurologic features of SLE discuss morbidity/mortality of SLE Slide 7 Incidence 5% of pts in US pediatric rheum practices 10% of US adult rheum practices Incidence:\n2-8/100,000/yr in adults\n0.3-0.9/100,000/yr in children 5,000-10,000 prevalence in US children ~ 100,000-200,000 adults varies with ethnic groups up to 3 fold in other ethnic groups: African American, Hispanic, Asian Slide 8 Demographics Adult onset 80% cases Childhood onset SLE 20% of all cases < 5% with onset prior to age 10 F:M ratio = 9:1 in teens/adults 5:1 in children Twin studies:\n24% concordance in monozygotic twins 2% in dizygotic twins\nHLA-DR2 and DR3\nincreases RR 2-3 x in Caucasians Slide 9 Etiology/ Pathogenesis Etiologic factors include: Immune dysregulation: hypergammaglobulinemia complement deficiency (C2, C4, C5) autoantibody production cytokine activitation inability to clear immune complexes organ and tissue deposition T cell lymphocytopenia defect in switch from T helper 0 to T helper 2 cells thus promoting B cell activation. Slide 10 Etiology/ Pathogenesis Hormones relative  androgens  estrogens Exacerbated by pregnancy in some Environmental factors:\nUV light Viral infection (?EBV,?CMV) Drugs\nHydralazine, isoniazid, minocycline, anticonvulsants Slide 11 1997 ACR Criteria Malar rash Discoid rash Photosensitivity Oral/nasal ulcers Serositis Nephritis Non-erosive arthritis Cytopenias Neuropsychiatric + ANA + Immunoserology dsDNA Antibodies Anti-Smith Antibodies Antiphospholipid Ab’s Aim: more homogeneous population in research 4/11 criteria: 88% Sensitivity/Specificity Slide 12 Cutaneous Malar “butterfly” rash ½ to 1/3 at onset symmetric SPARES nasolabial fold no scarring Discoid face, helix, scalp Often asymmetric demarcated papular lesions Scarring, alopecia More severe in dark skinned people Photosensitivity Slide 13 Treatment for Skin Sun block 70% are photosensitive at least 45 SPF antimalarial agents NON immune suppressing hydroxychloroquine chloroquine quinacrine ~70% response rate monitor annual eye exams occasionally:\ndapsone, steroids, azathioprine, rituximab Slide 14 Oral and Nasal Ulcerations Classically, PAINLESS ulcerations occur on the HARD palate buccal = non-specific In the nose they occur on the septum asx septal perforation can occur Slide 15 Serositis/ Cardio-pulmonary Pericarditis is most common cardiac complication (30%) May be subclinical Sx: precordial chest pain,  by laying down,  by sitting up and forward Constriction, tamponade rare Pleural effusions (~30%) in children 2º pleural inflammation or pneumonitis\nor to nephrotic syndrome Usually bilateral and small w/o respiratory compromise Pulmonary hemorrhage - rare life threatening complication Slide 16 Serositis Treatment Rule out infection serosal fluid analysis for infection, ANA + ANA MAY suggest lupus etiology Rule out other causes of chest pain or effusions: MI, PE, coaguloapthy, GI cause NSAID can help pain steroids low to moderate dose 1-2 mg/k/d max ~ 40-60mg a day usually do not need very hi doses Slide 17 Nephritis Clinically active in 50-75% of pts Likely to some degree in ALL with SLE Bx, EM shows some change on all\nPresentation (in order of frequency): Microscopic hema