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.

1. Living with Lupus
Presented by:
Lauren Kennish, MD
Rheumatology
August 22, 2012

2. Lupus Outline
• What is Lupus?
• How Lupus is diagnosed
• Symptoms of Lupus
• Treatment options
• Living with Lupus
• New developments

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. What is Lupus?
• Systemic Lupus Erythematosus
• Chronic condition
• Can affect almost any organ
 Brain •Skin
 Heart
•Musculoskeletal
 Lung
 Kidney •Blood
• Variable in presentation and
course Mild Severe

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 > Caucasians
Harrisons Internal Medicine, 18th ed. Longo et al. www.CDC.gov Bernatsky et al. Rheumatology, 2007.

6. What Causes Lupus?
• Unknown
 Hereditary?
 Environment?
• Known Triggers:
 Smoking
 Sunlight
 Hormones
 Infection
 Stress
 Sulfa medications

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. 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. Symptoms of Lupus
• Generalized symptoms:
 Unexplained fever
 Fatigue
 Weakness
 Achiness
 Headaches
 Dizziness
 Depression
• Less common symptoms:
 Ophthalmologic
 Gastrointestinal

10. Related Autoimmune Diseases
• Anti-Phospholipid Syndrome
• Sjogren’s Syndrome
• Raynaud’s Syndrome
• Associated disease -
 Fibromyalgia

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. 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. How is Lupus Diagnosed?
• American College
of Rheumatology
Criteria
 ≥4 out of 11 criteria
• Lupus likely
 <4 criteria
• Undifferentiated
syndrome?
www.uptodateonline.com

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. 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. How is Lupus Treated?
• There is no cure yet
• BUT…
• Many treatment options
• Can lead active, healthy lives

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. 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. 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. 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. 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. How is Lupus Treated?
Other Immunosuppressives
• Methotrexate
• Imuran (Azathioprine)
• Arava (Leflunomide)
• Cellcept (Mycophenolate Mofetil)
• Cytoxan (Cyclophosphamide)
• Tacrolimus
• Cyclosporine
• Rituxan (Rituximab)
• Benlysta (Belimumab)

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. 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. 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. 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. 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. How to Live Well with Lupus?
Manage Fatigue:
• Get enough sleep
• Rest during the day
• Plan your time effectively
• Exercise
• Medications

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. 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. 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. What’s New with Lupus?
• Benlysta / Belimumab
 Injection
 Treats:
• Moderately active Lupus
 Side effects: infection, allergy, cancer
• In development…

33. What’s New with Lupus?
Treatment name Type Focus of Studies Company or Organization
Anti-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 benefits
Rontalizumab (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 antibody
IFNα Kinoid Interferon-alpha vaccine Dose escalation study in SLE Neovacs
Early studies evaluating anti-inflammatory effects (drug intended to
IMO-3100* TLR7 and TLR9 blockers Idera Pharmaceuticals
block production of multiple pro-inflammatory cytokines)
Dynavax Technologies
DV1179* TLR7 and TLR9 inhibitor Recent studies to measure the reduction of lupus flares GlaxoSmithKline
National Institutes of Health
Anti-Cytokine therapies
Anti-interleukin-6 Studies to measure the safety and efficacy in patients with lupus
CNTO-136 Centocor, Inc.
antibody nephritis (inflammation of the kidneys)
Anti-interferon-gamma / Study to evaluate the safety, tolerability, and clinical efficacy in
AMG811 Amgen
monoclonal antibody discoid lupus
Immunosuppressive therapies
Immunosuppressant /
Organ transplant rejection drug being studied for lupus & lupus
Mycophenolate mofetil (cellcept) transplant anti-rejection Genentech / Roche, multiple institutions
nephritis
drug
Immunosuppressant /
Organ transplant rejection drug being studied for lupus & lupus
Tacrolimus (sustained release capsules) transplant anti-rejection Multiple sites in Asia
nephritis
drug
State University of New York-Upstate
Sirolimus Immunosuppressant Study in SLE
Medical Center
www.lupusresearch.org

34. What’s New with Lupus?
B-Cell therapies
B-cell inhibitor / recombinant
Atacicept (TACI-Ig)* Studies ongoing to pinpoint most effective dosing EMD Serono
fusion protein
B-cell inhibitor / Monoclonal
Epratuzumab (anti-CD22)* Study of the CD22 protein UCB and Immunomedics
antibody
B cell-inhibitor / Small Modular
SBI-087 (anti-CD20) Ascending single dose study Pfizer
ImmunoPharmaceutical
Benlysta® (FDA approved drug) B-cell inhibitor /
Long-term safety and efficacy studies continue GlaxoSmithKline Human Genome Sciences
(Belimumab/anti-BLys)* monoclonal antibody
LY2127399 B-cell inhibitor / monoclonal Studies to measure the safety and tolerability in
Eli Lilly
(anti-BAFF monoclonal antibody) antibody lupus patients
A623 (anti-BAFF) Anti-BAFF peptibody Efficacy, safety and tolerability in SLE Anthera
Bortezomib (Velcade) Proteosome inhibitor Proliferative lupus nephritis Weill Cornell Medical College
Immunomodulatory 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 Infectious
Abatacept (CTLA4-lg)*, Safety and efficacy studies to evaluate anti-
rheumatoid Diseases (NIAID) Immune Tolerance Network
Orencia® inflammatory benefits in lupus patients
arthritis drug Bristol Myers Squib
AMG557 Anti-B7RP-1 Active lupus nephritis Amgen
Laquinimod Immunomodulator Mild active SLE Teva
Paquinimod Immunomodulator Maintenance therapy for lupus nephritis Active Biotech Research
Leflunomide Immunomodulator Single ascending dose study RenJi Hospital
www.lupusresearch.org

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. Questions?
Thank you!
Lauren Kennish, MD
Summit Medical Group
Rheumatology
908-273-4300
www.summitmedicalgroup.com
Facebook/SummitMedicalNJ
Twitter: @SummitMedicalNJ