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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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