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Systemic Lupus Erythematosus Internist Update

Systemic Lupus Erythematosus Internist Update. Khaled Al Jarallah ,MD FRCPC,FACP,FACR Internist, Rheumatologist Medical Department Faculty of Medicine Kuwait University. No disclosures related to the presentation. Learning objectives. To highlight the natural history of SLE

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Systemic Lupus Erythematosus Internist Update

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  1. Systemic Lupus ErythematosusInternist Update Khaled Al Jarallah ,MD FRCPC,FACP,FACR Internist, Rheumatologist Medical Department Faculty of Medicine Kuwait University

  2. No disclosures related to the presentation

  3. Learning objectives • To highlight the natural history of SLE • To develop a clinical approach to patient suspected to have SLE • To define your therapeutic strategies to each SLE patient based upon activity, severity, organ damage, and comorbidities

  4. Multi-Systemic Autoimmune Disease

  5. Heterogeneous disease

  6. Lupus patient mind map !

  7. Aetiology ? Genetics Hormonal Environmental

  8. Genetics Moser K et al, Genes and Immunity 2009

  9. Genetics Criswell LA et al. The Rheumatologist 2011.

  10. Environmental • Ultraviolet light • Drugs • Infection • Smoking • Silica dust

  11. Pathogenesis Innate Adaptive Ann Rheum Dis 2010;69:1603–11

  12. Pathogenesis www.dressage-de-chien.net

  13. Natural history of SLE Ann Rheum Dis 2010;69:1603–11

  14. The 4 D’s clinical approach • Diagnostic workup • Disease activity assessment • Damage assessment • Design treatment goals

  15. Diagnostic workup • No gold standard test • Clinical reasoning • Classification criteria

  16. Diagnostic workup • Identify disease manifestations • Perform Lab. Tests • Exclude other diseases • Distinguish activity from chronicity • Prioritize active disease manifestations

  17. SLE target

  18. Which one is lupus rash ? A B C

  19. Lupus mimickers • Dermatological conditions: Rosocea, dermatitis • Chronic autoimmune disorders: sjogrens, MCTD , JIA • Vasculitis either primary or secondary to infection (Hep C, parvovirus B19 ,HIV, EB virus) or malignancy • Kikuchi-Fujimoto disease • Multiple Sclerosis

  20. Case presentation • A 25-year-old woman. Her disease manifestations consists of : • Fatigue • Hemolytic anemia • ANA + • Anti-Sm + • C3,C4,CH50 Low

  21. Classification criteria for SLE

  22. 1971 ACR criteria for classification of SLE Any of four or more criteria should be present , serially or simultaneously

  23. 1982 revised classification criteria Any of four or more criteria should be present , serially or simultaneously

  24. 1997 Revised classification criteria Any of four or more criteria should be present , serially or simultaneously

  25. 2012-SLICC* classification criteria At least 1 clinical +at least 1 immunologic Criteria ( for a total of4) OR Lupus Nephritis by biopsy with ANA or anti-dsDNA antibodies *Systemic Lupus International Collaborating Clinics Petri M et al. Arthritis Rheum 2012;64(8):2677-86

  26. 2012-SLICC classification criteria Lupus specific

  27. Histopathologic findings of interface dermatitis

  28. Acute Cutaneous / Subacute Cutaneous Lupus • Malar rash • Bullous lupus • Toxic epidermal necrolysis • Maculopapular lupus rash • Photosensitive lupus rash • Nonindurated psoriasiform • Annular polycyclic rash

  29. Chronic Cutaneous Lupus • Discoid rash, localized & generalized • Hypertrophic ( verrucous ) lupus • Lupus panniculitis ( profundus ) • Lupus erythematosus tumidis • Chilblains lupus • Mucosal lupus • Lichen planus overlap

  30. SLE- Cutaneous manifestations

  31. 2012-SLICC classification criteria

  32. 2012-SLICC classification criteria

  33. Performance of the classifications as compared to the current ACR criteria on the sample based on 702 cases* *Petri M et al. Arthritis Rheum. 2012;64(8): 2677

  34. Case presentation • A 25-year-old woman. Her disease manifestations consists of : • Fatigue • Hemolytic anemia • ANA + • Anti-Sm + • C3,C4,CH50 Low

  35. Cumulative SLE features in different ethnic groups *Lupus 1997; **1998; @2009; $Mod Rheumatol 2008; #Medicine (Baltimore) 1993

  36. Cumulative frequencies of SLE features Vitali C et al.Clin Exp Rheumatol 1992

  37. The 4 D’s clinical approach • Diagnostic workup • Disease activity assessment • Damage assessment • Design treatment goals

  38. Disease activity assessment • Which instrument to choose? • SLEDAI ,BILAG ,SLAM, ECLAM, SRI • Activity, severity, reversibility SELENA-SLEDAI

  39. SLEDAI • Evaluates 24 lupus manifestations • Parameters are scored √ if present • Manifestation items are weighted with scores ranging from 1 to 8 • Scores are totaled • Mild: 0-5 • Moderate: 6-12 • Severe: 13-20 Bombardier C et al. Arthritis Rheum. 1992; 35:630-640

  40. SLEDAI

  41. SLEDAI

  42. The 4 D’s clinical approach • Diagnostic workup • Disease activity assessment • Damage assessment • Design treatment goals

  43. Damage assessment • Which instrument to choose? • SLICC/ACR damage index • Chronicity, damage, irreversibility

  44. SLICC/ACR damage Index • Validated & used in clinical trails • Records damage in 12 organs or systems • The damage must present in the last 6 month • High damage index correlate with poor prognosis

  45. SLICC/ACR damage Index Damage (nonreversible change, not related to active inflammation) occurring since onset of lupus, ascertained by clinical assessment and present for at least 6 months unless otherwise stated. Repeat episodes must occur at least 6 months apart to score 2. The same lesion cannot be scored twice.

  46. SLE Disease assessment Disease Activity SLEDAI Disease Damage SLICC/ACR Damage Index Health Status Assessment SF-36

  47. The 4 D’s clinical approach • Diagnostic workup • Disease activity assessment • Damage assessment • Design treatment goals

  48. Design treatment goals • Do good Control disease activity Prevent organ damage Prevent flares • Do no harm Safety profile Drugs Drugs related damage

  49. Mortality and Treatment 1970-1990s Methotrexate Organ transplantation Plasmapheresis Cyclosporine 10 year survival >80% 1950-1954 Corticosteroids 5 year survival , 50% 2011 Belimumab 1960-1970s Cyclophosphamide Azathioprine ‘dialysis’ 10 year survival >60% 2000-2010s Mycophenolate mofetil Biologics , Retuximab 1940-1950 Antimalarials Improvement in antibiotic antihypertensive, and antithrombotic therapies Adapted from Manzi S ,ACR 2012

  50. Hydroxychloroquine • Reduction in flares N Engl J Med 1991;324:150 • Reduction in lipids Am J Med 1990;89:322 • Reduction in thrombosis Scand J Rheumatol 1996;25:191 • Reduction in organ damage Arthritis Rheum 2005;52:1473 • Improved survival Lupus 2005;14:220 • Triples mycophenolate response Lupus 2006;15:366 • Prevents seizure Ann Rheum Dis 2012;71:1502 • Reduction in CHB in neonatal lupus Circulation 2012

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