1 / 27

Systemic Lupus Erythematosis

Systemic Lupus Erythematosis. Patient. 14 year old girl Presented 2 mths previously with dyspnoea and chest pain at a regional hospital Initially treated as pneumonia Progressed to ICU admission 5 seizures in ward (started on phenytoin) TB as a child

nitara
Download Presentation

Systemic Lupus Erythematosis

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Systemic Lupus Erythematosis

  2. Patient • 14 year old girl • Presented 2 mths previously with dyspnoea and chest pain at a regional hospital • Initially treated as pneumonia • Progressed to ICU admission • 5 seizures in ward (started on phenytoin) • TB as a child • No history of medicines, allergies, family risk • Development: Grade 6 – doing well

  3. History continued • Transferred to tertiary hospital • Blood tests done, started on prednisone • Referred to Renal Clinic for renal biopsy

  4. Results from clinic • ANA, ANF, Anti-Sm, Anti dsDNA, Ro, La Positive • Low C3, C4

  5. On Examination • Weight: 44,7 kg (25th) • Height: 170 cm (75th) • Apyrexial, RR 18, HR 119, BP 96/70 • Marfanoid habitus, rash on face and body • Not anaemic, no oedema, shotty cervical glands • CVS: tachycardic • Resp, Abdo, neuro, ENT: normal • Musculo-skeletal: Marfanoid. No arthritis • Tanner stage 4

  6. Urine • 4+ haemoglobin, no protein • Spun: scanty red cells and casts • Lab: 18 000 leukocytes < 1000 RBC’s Epithelial cells Gram negative bacilli

  7. Laboratory investigations • Hb 11 (MCV 90.6), Plt 369 000 • WCC 5.6 (N61%, M4%, L35%) • U+E, CMP: normal • ESR 36, CRP 2.7 • INR, PTT, CK, Cholesterol, WR: normal

  8. Special investigations • CXR: - residual opacification (RUL + RML) with fluid in fissure - ?some scoliosis - Heart size + aortic knuckle normal • ECG: normal • Echo: pending • Ophthalmology, dermatology consultations: pending

  9. Introduction to SLE • Auto-immune disorder • Multisystem microvascular inflammation • Formation of autoantibodies • Chronic with relapsing and remitting course

  10. Pathophysiology • Proposed mechanism for autoantibodies: • Defect in apoptosis • ↑cell death → disturbance in immune tolerance • Plasma + nuclear antigens displayed on cell surface • Dysregulated lymphocytes target Ag (normally intracellular) • Immune complexes form in microvasculature → complement activation + inflammation • Ag-Ab complexes deposit in basement membranes of skin and kidneys

  11. Etiology • Unknown • At least 10 gene loci known to ↑ risk • Genetic predisposition (10x more in monozygotic twins) • Human leukocyte Ag: ↑ HLA-DR2, HLA-DR3 + HLA-B8 • Null complement alleles + congenital ↓complement (esp C4, C2 etc)

  12. Epidemiology • Prevalence: 4 – 250/100 000 • Incidence US: 1/10 000 • Onset: before 8 yrs unusual • Female predominance (prepubertal 4:1, postpubertal 8:1) • Rare in black African population

  13. History • Constitutional – fatigue, fever, weight loss • Skin – malar rash, photosensitive, discoid lupus, alopecia, Raynaud phenomenon, livido reticularis • Musculoskeletal – arthralgia, myalgia, arthritis • Renal • Neuropsychiatric – headache, mood disorders, cognitive disorders, psychosis, seizures, TIA/ stroke, movement disorders, mononeuritis • Pulmonary – chest pain, dyspnoea • Gastrointestinal – Abdominal pain, jaundice • Cardiac – heart failure/chest pain • Haematological – multiple ‘cytopenias’ • Other – miscarriages, family history of autoimmune disease

  14. On examination • Constitutional – lymphadenopathy, hepatosplenomegaly • Musculoskeletal – Jaccoud arthropathy • Dermatologic - capillaroscopy • Renal • Neuropsychiatric • Cardiopulmonary – friction rubs, pulmonary embolism, Libman-Sacks endocarditis • GIT – peritonitis, pancreatitis, mesenteric vasculitis

  15. Diagnostic criteria American College of Rheumatology 4/11 criteria (sens 85%, specif 95%) “SOAP BRAIN MD” • Serositis – heart, lung, peritoneum • Oral ulcers – painless esp palate • Arthritis – non-erosive • Photosensitivity

  16. Diagnostic criteria continued • Blood disorders - ↓RBC (Coombs +), PLT, WCC, Lymphocytes • Renal involvement – proteinuria /± casts • ANA – titer > 1:160 • Immunologic phenomena – LE cells, anti-dsDNA Ab, anti-Sm Ab, antiphospholipid Ab, false WR + • Neurological disorders – seizures/ psychosis • Malar rash – cheeks + nasal bridge • Discoid rash – rimmed with scaling, follicular plugging

  17. Laboratory studies High clinical suspicion/ high ANA titres SLE Screen: • FBC and diff • S-creatinine • Urinalysis with microscopy • Basic inflammatory markers • Antibodies to dsDNA • Complement • ANA subtypes (anti-Sm, Ro, La, RNP Ab’s)

  18. Autoantibody tests used in SLE • ANA – 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 RBC’s) • Anti-histone (drug-induced lupus)

  19. Radiological studies • Joint x-rays: no erosions, periarticular osteopenia + soft tissue swelling • CXR/CT chest: interstitial lung disease, pneumonitis, pulmonary emboli, alveolar hemorrhage • CTBrain or Brain MRI ± angiography: lupus white matter changes, vasculitis or stroke • Echo: pericardial effusion, pulmonary hypertension or Libman-Sacks endocarditis

  20. Invasive procedures • LP – nonspecific ↑cells + protein, ↓ glucose • Renal biopsy – prognosis and Rx • Skin biopsy

  21. Lupus nephritis

  22. Differential diagnosis • Drug induced lupus erythematosis • Vasculitis • Leukemia • HIV • Multiple sclerosis • Parvovirus or other viral infections

  23. Treatment principles • Depends on disease severity • Fever, skin, musculoskeletal and serositis = milder disease • CNS and renal involvement – aggressive Rx • Emergencies: - severe CNS involvement - systemic vasculitis - profound thrombocytopenia (TTP-like syndrome) - rapidly progressive nephritis - diffuse alveolar hemorrhage

  24. Medications used • NSAIDS • Chloroquine • Steroids • Cyclophosphamide • Azathioprine • Mycophenolate • (Rituximab) • Plasma exchange/ IVIG

  25. Preventive care • Medication-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 exposure • Avoid estrogen therapies • Avoid sulfa-containing medications • Pregnancy planning

  26. Prognosis • Benign to rapidly progressive • Better for isolated skin + musculoskeletal disease vs renal and CNS • Death rate 3X age-comparable general populationMortality • 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)

  27. Summary • Autoimmune disorder • Multiple manifestations • Aggressive investigation and treatment • Continued surveillance

More Related