2. Disclaimer
This program is not intended to dispense medical
advice, directly or indirectly. The purpose of this
program is to provide the public with information,
not to prescribe or diagnose. Lupus is a complicated
disorder that requires the regular care of a qualified
physician.
2
3. Lupus is not…
• HIV/AIDS
• Leukemia
• Cancer
• Contagious
• Your fault
• All in your head
3
4. Warning Signs of Lupus
When lupus first sets in, symptoms such as fatigue and pain are often non-specific.
They can be signs of so many other health problems, which can make diagnosis
hard. The most common complaint people have is fatigue that is so severe it stops
them from being able to function normally. This fatigue is often related to
fibromyalgia. Fever, muscle and joint pain are also quite common.
Muscle & Joint Pain
95% of people with lupus experience muscle and joint pain.
Fever Greater Than 100° F
90% of people with lupus get a fever of more than 100 degrees Fahrenheit (38
degrees Celsius).
Prolonged or Extreme Fatigue
81% of people with lupus suffer from prolonged or extreme fatigue.
Anemia
71% of people with lupus simultaneously suffer from anemia.
5. Kidney Problems
50% of people with lupus find that their kidneys are affected. Symptoms include
weight gain, swollen ankles, high blood pressure, and decreased kidney function.
Pain in the Chest
45% of people with lupus experience a pain in their chest upon breathing deeply.
This condition is called pleurisy.
Rashes
80% of people with lupus observe rashes on their skin. A butterfly-shaped rash
across the cheeks and nose is especially common (42%).
Light Sensitivity
30% of people with lupus become sensitive to light, a condition called
photosensitivity.
Hair Loss
27% of people with lupus experience hair loss resulting in patchy or bald spots.
6. Abnormal Blood Clotting
20% of people with lupus report abnormal blood clotting problems.
Eye Disease
20% of people with lupus concurrently suffer from eye complications such as
dry eyes, eye inflammation, and eyelid rashes.
Seizures
15% of people with lupus experience seizures.
Mouth or Nose Ulcers
12% of people with lupus report ulcers on their nose or mouth.
8. Chronic
• Lifelong-no cure
• Ranges from mild to severe
• Most are mild to moderate
• Periods of remission and activity (flares)
• Treatment goals
• Control symptoms
• Prevent permanent damage
8
9. The Immune System
Defending the Body Against Invaders
Retrieved from www.lymphoma.org on 10/23/07
Retrieved from www.humanillness.com on 11/5/07
9
10. Autoimmune & Inflammatory
Genetic predisposition (5-10% risk)
+
Triggers (stress, sunlight, infection)
+
Inadequate suppressor T cells
Autoantibodies & surplus of antibodies
B cells
Aladjem (1988) 10
11. Immune system vs. Blood Cells
Antibodies attach to blood cells
Red blood cells White blood cells Platelets
•Damage to cells •Damage to cells •Damage to cells
•Accelerated spleen clearance •Can’t replace fast enough •Can’t replace fast enough
•Can’t replace fast enough
Anemia Leukopenia Thrombocytopenic anemia
Fatigue Difficulty fighting infection Clotting difficulties
Bleeding into skin & organs
Treatment goal-reduce these antibodies in blood
Aladjem (1988) 11
12. Immune system vs. cell debris
Normal breakdown of cells
Cell debris in blood stream
Antibodies + debris=immune complexes
Defective clearance of immune complexes Immune complex + serum proteins
Mistakenly deposit in organs other than spleen Activation of complement system
Inflammation in organ Complement attracts white cells to site
White cells try to eat complex
White cells release enzymes
Enzymes cause inflammation & tissue damage
Repeated attacks of inflammation, healing and scarring
can result in more severe organ impairment
Aladjem (1988) 12
13. Treatment Goals
• Prevent inflammation
• Manage inflammation when it occurs
• Prevent organ damage
• Maintain health and well-being of patient
• Intervention of acute relapses
• Management of chronic problems
• Medications and risk/benefit ratio
Aladjem (1988) 13
14. Therapy Aimed at Multiple Sites in Cycle
• Prevention of excessive antibody production
• Prevention of complement binding or activation
• Prevention of white blood cell enzyme release
• Blocking of enzyme action
• Prevention of scar formation
No one medication can
accomplish all these tasks.
Aladjem (1988)
14
16. How many people have lupus?
• 1.5-2 million Americans
• 90,000 Floridians
• 16,000 Americans develop lupus annually
• Between 1,500,000 and 2,000,000 people in the
United States have been diagnosed with
lupus, making lupus more prevalent than
AIDS, sickle-cell anemia, cerebral palsy, multiple
sclerosis, and cystic fibrosis combined.
• A word about statistics
16
17. Who gets lupus?
• 90% of lupus patients are women
• 80% are diagnosed between 14 and 45
• 70% have systemic lupus erythematosus (SLE)
• Asians, Hispanics, African Americans and Native
Americans have 2-3 times the incidence of lupus as
Caucasians
• Lupus can develop in any person regardless of age,
gender or ethnicity
17
18. How Is Lupus Diagnosed?
• Symptoms
• Medical history-review of systems
• Elimination of other disorders
• Laboratory tests
• American College of Rheumatology Criteria
Typical adult
•Consults 3 to 5 physicians before diagnosis
•Waits 2-3 years for diagnosis
•Over age 60 up to 4 years for diagnosis
18
Wallace (1988)
19. American College of Rheumatology (ACR)
Revised Criteria for the Classification of Systemic Lupus Erythematosus (1996)
Skin Criteria
• Butterfly rash
• Discoid rash
• Sun sensitivity
• Oral ulcerations
Systemic Criteria
• Arthritis
• Serositis
• Kidney disorder
• Neurologic disorder
Laboratory Criteria
• Blood abnormalities
• Immunologic disorder
• Positive ANA blood test
American College of Rheumatology www.rheumatology.org 19
20. Diagnosis of Systemic Lupus Erythematosus
Patients presenting with disease manifestations
involving two or more organ systems
ANA testing
Titer > 1:40 Titer<1:40
Consider referral to rheumatologist for full Strong argument against SLE-
SLE evaluation including alternative explanation for organ
1. ACR diagnostic criteria system manifestations should be
2. Laboratory tests pursued
Zero to three Four or more Explanation found No explanation found
ACR criteria ACR criteria
Sufficient to Consider referral to
rule out SLE rheumatologist if
No SLE or SLE
question of SLE or
incomplete SLE
incomplete SLE remains
20
American Family Physician retrieved from www.aafp.org/afp/20031201/2179.htmlon 10/23/07
21. “Routine” Lab Work
• Complete blood count (CBC)
• Red blood cells
• Platelets
• White blood cells
• Neutrophils
• Lymphocytes
• Monocytes
• Basophils
• Eosinophils
Retrieved from www.rmlonline.com/patientCen.asp?id=ss160 on 10/23/07 21
22. “Routine” Lab Work
Blood Chemistry (7 to 25 tests)
• Blood sugar
• Kidney function
• Liver function
• Electrolytes
• Lipids
• Proteins
• Thyroid function
Wallace (2005) 22
23. Other Tests Relevant to SLE
• Creatine phosphokinase (CPK)-muscle
inflammation
• Westergren sedimentation rate or C-reactive
protein (CRP)-quantitates
levels of inflammation
• Prothrombin time (PT) and partial
thromboplastin time (PTT)-clotting tests
Wallace (2005) 23
24. Antibody Panels and Screens
• Antinuclear antibody (ANA)
• Anti-double-stranded DNA
• Serum complement
• Rheumatoid factor
• 80% positive in RA
• 20-30% positive in SLE
• Antiphospholipid antibodies
• Anticardiolipin www.bindingsite.uk.co
• Lupus anticoagulant
Wallace (2005)
24
25. Antinuclear Antibody Test
• Positive in 95-98% of SLE patients
• 68% positive with Sjogren’s
• 40-75% positive with scleroderma (especially
with speckled ANA)
• 16% positive juvenile rheumatoid arthritis
• 25-50% positive in rheumatoid arthritis
(especially with diffuse pattern)
Aladjem (1988) 25
27. Different Forms of Lupus
• Neonatal
• Drug induced
• Cutaneous or discoid
• Systemic lupus erythematosus (SLE)
27
28. Neonatal Lupus
• Very rare
• Affects fetus or newborn
• Mother passes auto-antibodies to
baby
• Affects skin, heart, and blood of baby
• Rash appears in first weeks-disappears
by six months
• Can cause congenital heart block
• Neonatal lupus is not systemic lupus
erythematosus
28
29. Drug Induced Lupus
• Long term use of certain medications
• Symptoms of SLE
• Stopping medications reduces symptoms
within weeks
• Some implicated medications
•Chloropromazine
•Methyldopa
•Hydralazine
•Precainamide
•Isoniazid
•Dilantin
•Penicillamine
•Quinidine
•Certain antibiotics
•Allupurinol
29
30. Lupus and the Skin
• 60-70% of lupus patients report some skin complaint
• Ultraviolet light
• UVA & UVB damage DNA deposits near skin surface
• Altered DNA leads to formation of anti-DNA
• UV light induces production of anti-Ro (SSA), anti-La (SSB)
and anti-RNP
• Patients who are positive for anti-Ro are very sun-sensitive
• Immune complexes may be deposited in organs
• Some medications increase photosensitivity
30
31. Cutaneous Features of Lupus
Mouth or nose sores Oral thrush
www.hss.edu
Alopecia-hair loss
www.hss.edu
31
32. Cutaneous Features of Lupus
Butterfly (malar) rash Changes in pigmentation
www.medstudents.com.br
www.humanillnesses.com
Hives or welts (urticaria)
www.users.globalnet.co.uk www.answers.com
32
33. Vascular Rashes
Raynaud’s Phenomenon
• 1/3 of lupus patients
www.pdrhealth.com
• Autonomic system
malfunction
• Dilate or constrict small
blood vessels
www.immunologyclinic.com
33
35. Other Skin Disorders in Lupus
Lupus panniculitis (profundus)
• Inflamed fat pads in skin
• Rare
• Responds to treatment
www.merckmedicus.com
www.accessmedicine.com
• 1 in 200
Blisters (bullous lupus)
•Fluid filled blisters or blebs
•Biopsy required to determine treatment
•1 in 500 lupus patients www.merckmedicus.com
Source: Wallace (2005) 35
36. Musculoskeletal System
• Joints
• Muscles
• Bone
• Supporting structures-
tendons, ligaments, bur
sae www.msnbc.msn.com
36
37. Joints and Soft Tissues
• Arthralgia-joint pain
• Arthritis-visible joint
inflammation
• Synovium-thin membrane lining
certain joints
• Inflammation of synovium causes
pain and damage over time
37
39. Atherosclerosis
Hardening of the Arteries
“Atherosclerotic heart disease…is the third
most common cause of death in lupus
patients, following complications of kidney
disease and infection.” Wallace (2005)
Long term moderate to high dose steroid use
•High blood pressure
•Diabetes
•High cholesterol
•Premature atherosclerosis
39
40. Nervous System
• CNS vasculitis-inflammation of
blood vessels in brain (10%)
• Seizures
• Strokes & transient ischemic
attacks (TIA’s) clots caused by
antiphospholipid antibodies
• Neuropathy-
numbness, tingling, local palsies
• Lupus headache
www.nlm.nih.gov
Wallace (2005)
40
41. Lupus in the Kidney and Urinary Tract
Lupus primarily affects the glomerulus
•Usually no specific complaints traceable to kidney
•Other causes of “kidney” pain-pleurisy, kidney stone, kidney
infection, muscle spasm in lumbar spine
41
42. Lupus in the Kidney
Patient awareness of kidney problem
• Nephrotic or uremic-kidney spills large
amounts of protein
• Swelling in ankles and abdomen
• General sense of bloating and
discomfort
www.clevelandclinic.org
• Pleural and pericardial effusion
42
43. Lupus in the Kidney
Blood and urine tests
• Blood urea nitrogen (BUN)
• Creatinine clearance
• Casts (cellular debris)
• Protein
Biopsy
1. No disease
2. Mesangial
3. Focal proliferative
4. Diffuse proliferative
5. Membranous
6. Glomeruloscleroisis
43
44. Lupus and Pregnancy
• 10-15 % of all pregnancies end in miscarriage
• Slightly higher miscarriage rate in lupus
• Exacerbation may occur during or shortly after delivery
• Fertility affected by disease activity, dialysis or
medications
• Chances baby will have lupus-fewer than 10% of patients
who carry a lupus gene will ever develop the disease
• Patients with anti-Ro (SSA) and anti-La (SSB)
• Neonatal lupus
• Congenital heart dysfunction or block
Wallace (2005) 44
46. Fatigue
• What is the cause? Other factors?
• What are the implications for treatment?
• What is normal vs. abnormal fatigue?
• The role of inflammatory cytokines.
“Abnormality is when the number of bad
days exceed the number of good ones or
when there are more bad days than there
used to be.” Peter H. Schur, M.D. (Aladjem, 1988)
46
47. Other Causes of Fatigue
• Increased work load (home, work, family)
• Lack of sleep
• Depression
• Unhealthful habits (smoking drinking, drugs, fad
diets, sedentary lifestyle)
• Internal conflicts
• Deconditioning-being out of shape
• Anemia
• Mononucleosis
• Certain medications
• Inflammatory disease
• Systemic infections
47
48. Medications
Only 4 medications approved by the FDA
specifically for the treatment of lupus
• Aspirin
• Corticosteroids (1955)
• Hydroxychloroquine (1955)
• Belimumab (Benlysta) (2011)
50. Lupus
www.sdmines.sdsmt.edu www.juliaswartz.com
Lupus can strike any
Lupus can develop
person regardless
at any age
of gender or ethnicity
Lupus can affect every part of the body.
Lupus affects each patient differently.
50
51. The Lupus Foundation of Florida
• Educational Seminars
• Support groups with trained facilitators
• Funding for research
• Advocacy at National and State levels
• Patient referrals
www.lupusflorida.org
51
52. Sources
American College of Rheumatology http://www.rheumatology.org/
Medscape http://www.medscape.com/
Could I Have Lupus http://www.couldihavelupus.gov/
National Institute of Arthritis Musculoskeletal and Skin Diseases
http://www.niams.nih.gov/
Lupus Initiative www.thelupusinitiative.org/
Lupus Research Institute http://www.lupusresearch.org/
Lahita, Robert, ed. Systemic Lupus Erythematosus: Fourth Edition. San
Diego, CA: Academic Press 2004.
Wallace, Daniel. The Lupus Book: A Guide for Patients and Their Families,
4th ed. Oxford: Oxford University Press (2009).