Lupus

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Lupus is a chronic autoimmune disease that occurs when a person's immune system attacks their own tissues and organs. Learn about the symptoms of lupus, how it is diagnosed and new treatment options to live well with lupus.

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Lupus

  1. 1. Living with Lupus Presented by: Lauren Kennish, MD Rheumatology August 22, 2012
  2. 2. Lupus Outline• What is Lupus?• How Lupus is diagnosed• Symptoms of Lupus• Treatment options• Living with Lupus• New developments
  3. 3. What is Lupus?• Lupus is an autoimmune disease• Autoimmune disease: Immune system incorrectly targets your own tissues and organs Antibodies form and attack cells Inflammation/Swelling Damage/Disease
  4. 4. What is Lupus?• Systemic Lupus Erythematosus• Chronic condition• Can affect almost any organ  Brain •Skin  Heart •Musculoskeletal  Lung  Kidney •Blood• Variable in presentation andcourse Mild Severe
  5. 5. What is Lupus? • 1-10 in 10,000 in US; 300,000-1 million affected • Diagnosed in ages 15-45 • Females (9x) > Males (1x) • More prevalent: African Americans, Hispanics, Asians > CaucasiansHarrisons Internal Medicine, 18th ed. Longo et al. www.CDC.gov Bernatsky et al. Rheumatology, 2007.
  6. 6. What Causes Lupus?• Unknown  Hereditary?  Environment?• Known Triggers:  Smoking  Sunlight  Hormones  Infection  Stress  Sulfa medications
  7. 7. Symptoms of Lupus• Skin  Rash – • Malar (butterfly) • Discoid • Photosensitivity  Mouth/Nose sores  Hair loss• Musculoskeletal System  Arthritis- joint pain and swelling  Muscle weakness American College of Rheumatology www.rheumatology.org
  8. 8. Symptoms of Lupus• Kidney  Lupus nephritis - change in urine, swelling, high blood pressure• Central Nervous System  Seizures, Psychosis  Headaches, neuropathy• Heart/Lungs  Serositis - chest pain, trouble breathing  Lung disease, heart valve disease• Blood www.rheumatology.org  Anemia, leukopenia, thrombocytopenia – low red blood cells, white blood cells, platelets
  9. 9. Symptoms of Lupus• Generalized symptoms:  Unexplained fever  Fatigue  Weakness  Achiness  Headaches  Dizziness  Depression• Less common symptoms:  Ophthalmologic  Gastrointestinal
  10. 10. Related Autoimmune Diseases• Anti-Phospholipid Syndrome• Sjogren’s Syndrome• Raynaud’s Syndrome• Associated disease -  Fibromyalgia
  11. 11. How is Lupus Diagnosed?• Combination of symptoms and tests  No single test• History and physical exam• Laboratory tests  blood, urine• Biopsy  Skin, Kidney• Radiology  X-Ray, CT, MRI• Can take time to diagnose www.rheumatology.org
  12. 12. How is Lupus Diagnosed? • ANA – Anti-Nuclear Antibody – antibody to cells  Virtually all with SLE (>98%)1  ANA does NOT necessarily mean lupus • False positive – up to 30% healthy people2 • More specific antibodies – www.rheumatology.org  Double Stranded DNA  Phospholipid Antibodies  Smith/RNP  Sjogrens • Markers of inflammation and activity - ESR, CRP, complements (C3, C4)1. Harrisons Internal Medicine, 18th ed. Longo et al. 2. Tan et al. Arthritis Rheum, 1997.
  13. 13. How is Lupus Diagnosed?• American College of Rheumatology Criteria  ≥4 out of 11 criteria • Lupus likely  <4 criteria • Undifferentiated syndrome? www.uptodateonline.com
  14. 14. What is the Course of Lupus?• Variable among patients• Most people with Lupus can lead full life• 5 year survival rates are now over 95%• 50% have milder symptoms - fatigue, joint pain, rash flare relapse remission
  15. 15. What is the Course of Lupus?• More severe disease associated with:  Heart, Lung, Brain, Kidney involvement  Kidney Failure/Dialysis  Infections• Increased risk of heart disease• Increased risk of cancer
  16. 16. How is Lupus Treated?• There is no cure yet• BUT…• Many treatment options• Can lead active, healthy lives
  17. 17. How is Lupus Treated?• Goal:  Reduce symptoms  Prevent flares  Prevent complications• Treatment is tailored to individual  Based on symptoms, concurrent conditions, lifestyle  Treatment can be continuous or intermittent
  18. 18. How is Lupus Treated?NSAIDs: Non-Steroidal Anti-Inflammatory Drugs • Over-the-counter or prescription • Treats:  Joint pain  Swelling  Fever • Side effects:  Stomach ulcers, kidney/liver problems, high blood pressure, heart disease
  19. 19. How is Lupus Treated? Immunosuppressive Therapy• Suppresses immune system cells from forming and making antibodies to tissues• Slow acting• Oral, Injection• Treats:  Kidney, Heart, Lung, Brain  Musculoskeletal  Skin  Blood• Side effects:  Infections, cancer risk, hair loss, nausea
  20. 20. How is Lupus Treated? Steroids / Prednisone• Anti-inflammatory / Immune suppressing• Works rapidly• Topical, Oral, or Injection• Treats:  Kidney, Heart, Lung, Brain  Musculoskeletal  Skin  Blood• Side effects:  Weight gain, mood change, infections, diabetes, high blood pressure, osteoporosis, cataracts
  21. 21. How is Lupus Treated? Plaquenil / Hydroxychloroquine• Widely used medication• Treats:  Musculoskeletal  Skin  Fatigue  Prevents flares• Side effects:  Stomach upset, retinal toxicity (very rare) • Screening eye exams
  22. 22. How is Lupus Treated? Other Immunosuppressives• Methotrexate• Imuran (Azathioprine)• Arava (Leflunomide)• Cellcept (Mycophenolate Mofetil)• Cytoxan (Cyclophosphamide)• Tacrolimus• Cyclosporine• Rituxan (Rituximab)• Benlysta (Belimumab)
  23. 23. How to Live Well with Lupus• How to:  Maintain control of disease  Prevent flares  Avoid complications  Minimize risk of associated diseases  Feel healthy• To lead an active, high quality life
  24. 24. How to Live Well with Lupus?Follow-up with Rheumatologist Regularly:• 2-6 months for monitoring• Even if feeling well• Recognize symptoms of flares• Get treated early• Take medications consistently
  25. 25. How to Live Well with Lupus?Follow-up with Your Doctors:• Follow regularly for preventative care  Internist/Primary care  Gynecology• Follow with Specialists• Planned surgery  Best when low activity  Possible pre-medications
  26. 26. How to Live Well with Lupus?Women’s Health:• Pregnancy  Can be associated with complications and flares  Planned is ideal  Monitored more closely  May need to change medications• Hormones – birth control pills, estrogen  Safe if Lupus stable and no anti-phospholipid antibodies  Possible increase in flares  Non-estrogen pills, IUD Buyon et al.Annals Int Med, 2005.
  27. 27. How to Live Well with Lupus?Reduce Risk of Infections:• Lupus increases risk for infection• Up to date with vaccines  Influenza, Pneumovax, Shingles• Wash hands frequently• Stay away from people who are sick• Get evaluated sooner rather than later
  28. 28. How to Live Well with Lupus?Manage Fatigue:• Get enough sleep• Rest during the day• Plan your time effectively• Exercise• Medications
  29. 29. How to Live Well with Lupus?Healthy Lifestyle:• Reduce sun exposure  UV-B – sunlight or fluorescent light  UV-A – sunlight, photocopier  Use sunscreen >30 SPF, broad spectrum  Avoid peak sun times  Wear hats, protective clothing
  30. 30. How to Live Well with Lupus?Healthy Lifestyle:• Exercise  Low-moderate activities  Stretching, strengthening• Don’t smoke• Reduce stress• Social support Costenbader et al. Arthritis Rheum, 2004.
  31. 31. How to Live Well with Lupus?Healthy Lifestyle:• Eat well balanced diet Fresh fruits/vegetables, fiber and grains, lean meats Vitamin D, Calcium ? - Omega-3 fatty acids, Anti-oxidants ? - Avoid certain foods • Alfalfa sprouts, Nightshade vegetables • Moderate alcohol• Alternative treatments – not well studied
  32. 32. What’s New with Lupus?• Benlysta / Belimumab  Injection  Treats: • Moderately active Lupus  Side effects: infection, allergy, cancer• In development…
  33. 33. What’s New with Lupus?Treatment name Type Focus of Studies Company or OrganizationAnti-interferon alpha therapies Anti-malarial / Assistance Publique - Hôpitaux de Paris &Hydroxychloroquine Recent studies to measure the reduction of lupus flares TLR antagonist Sanofi-Synthelab Anti-Interferon-alpha / Safety and efficacy studies to evaluate anti-inflammatory benefitsRontalizumab (rhuMAb IFNalpha)* Genentech Monoclonal antibody in lupus patients Anti-Interferon-alpha /Sifalimumab (Medi-545) Studies to measure safety and tolerability in lupus patients Medimmune, Inc. Monoclonal antibody Anti-Interferon-alpha /AGS-009* Single dose escalation trial in SLE Argos Therapeutics Monoclonal antibodyIFNα Kinoid Interferon-alpha vaccine Dose escalation study in SLE Neovacs Early studies evaluating anti-inflammatory effects (drug intended toIMO-3100* TLR7 and TLR9 blockers Idera Pharmaceuticals block production of multiple pro-inflammatory cytokines) Dynavax TechnologiesDV1179* TLR7 and TLR9 inhibitor Recent studies to measure the reduction of lupus flares GlaxoSmithKline National Institutes of HealthAnti-Cytokine therapies Anti-interleukin-6 Studies to measure the safety and efficacy in patients with lupusCNTO-136 Centocor, Inc. antibody nephritis (inflammation of the kidneys) Anti-interferon-gamma / Study to evaluate the safety, tolerability, and clinical efficacy inAMG811 Amgen monoclonal antibody discoid lupusImmunosuppressive therapies Immunosuppressant / Organ transplant rejection drug being studied for lupus & lupusMycophenolate mofetil (cellcept) transplant anti-rejection Genentech / Roche, multiple institutions nephritis drug Immunosuppressant / Organ transplant rejection drug being studied for lupus & lupusTacrolimus (sustained release capsules) transplant anti-rejection Multiple sites in Asia nephritis drug State University of New York-UpstateSirolimus Immunosuppressant Study in SLE Medical Center www.lupusresearch.org
  34. 34. What’s New with Lupus?B-Cell therapies B-cell inhibitor / recombinantAtacicept (TACI-Ig)* Studies ongoing to pinpoint most effective dosing EMD Serono fusion protein B-cell inhibitor / MonoclonalEpratuzumab (anti-CD22)* Study of the CD22 protein UCB and Immunomedics antibody B cell-inhibitor / Small ModularSBI-087 (anti-CD20) Ascending single dose study Pfizer ImmunoPharmaceuticalBenlysta® (FDA approved drug) B-cell inhibitor / Long-term safety and efficacy studies continue GlaxoSmithKline Human Genome Sciences(Belimumab/anti-BLys)* monoclonal antibodyLY2127399 B-cell inhibitor / monoclonal Studies to measure the safety and tolerability in Eli Lilly(anti-BAFF monoclonal antibody) antibody lupus patientsA623 (anti-BAFF) Anti-BAFF peptibody Efficacy, safety and tolerability in SLE AntheraBortezomib (Velcade) Proteosome inhibitor Proliferative lupus nephritis Weill Cornell Medical CollegeImmunomodulatory therapy Ongoing studies to measure efficacy; Lupuzor is thought to modulate, through a unique mechanism,Lupuzor™ (CEP-33457) CD4 T-Cell Modulator Cephalon a specific subset of CD4 T cells which play a critical role in the physiopathology of lupus Inhibits B7-CD28 interaction, National Institute of Allergy and InfectiousAbatacept (CTLA4-lg)*, Safety and efficacy studies to evaluate anti- rheumatoid Diseases (NIAID) Immune Tolerance NetworkOrencia® inflammatory benefits in lupus patients arthritis drug Bristol Myers SquibAMG557 Anti-B7RP-1 Active lupus nephritis AmgenLaquinimod Immunomodulator Mild active SLE TevaPaquinimod Immunomodulator Maintenance therapy for lupus nephritis Active Biotech ResearchLeflunomide Immunomodulator Single ascending dose study RenJi Hospital www.lupusresearch.org
  35. 35. For more Information• Lupus Foundation of America  www.lupus.org• National Institute of Arthritis and Musculoskeletal and Skin Diseases  www.niams.nih.gov• American College of Rheumatology  www.rheumatology.org
  36. 36. Questions? Thank you! Lauren Kennish, MD Summit Medical Group Rheumatology 908-273-4300www.summitmedicalgroup.com Facebook/SummitMedicalNJ Twitter: @SummitMedicalNJ

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