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Understanding Lupus




Lupus Foundation of Florida
   www.lupusflorida.org
                              1
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
Lupus is not…
 •   HIV/AIDS
 •   Leukemia
 •   Cancer
 •   Contagious
 •   Your fault
 •   All in your head


                        3
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.
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.
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.
What is lupus?

• Chronic

• Autoimmune

• Inflammatory


                 7
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
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
Autoimmune & Inflammatory
 Genetic predisposition (5-10% risk)
                    +
 Triggers (stress, sunlight, infection)
                    +
    Inadequate suppressor T cells

Autoantibodies & surplus of antibodies
               B cells

                Aladjem (1988)            10
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
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
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
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
Other Autoimmune Disorders
           23.5 million Americans
  Multiple sclerosis
  Crohn’s Disease-ulcerative colitis
  Insulin dependent diabetes (type I)
  Fibromyalgia-fibromyositis
  Celiac Sprue-Dermatitis Herpetiformus
  Grave’s disease
  Mixed connective tissue disease
  Psoriasis
  Rheumatoid arthritis
  Myasthenia gravis
  Vitiligo
  Alopecia areata
  Raynaud’s
  Scleroderma
                    www.aarda.org         15
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
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
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)
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
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
“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
“Routine” Lab Work
     Blood Chemistry (7 to 25 tests)
     • Blood sugar
     • Kidney function
     • Liver function
     • Electrolytes
     • Lipids
     • Proteins
     • Thyroid function



       Wallace (2005)                  22
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
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
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
Other Diagnostic Tests
•Chest X-ray
•Kidney biopsy
•Neurological exam
•Skin biopsy
•Bone marrow biopsy
Different Forms of Lupus
•   Neonatal
•   Drug induced
•   Cutaneous or discoid
•   Systemic lupus erythematosus (SLE)




                                         27
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
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
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
Cutaneous Features of Lupus

Mouth or nose sores                      Oral thrush



      www.hss.edu



                    Alopecia-hair loss



                          www.hss.edu
                                                       31
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
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
Vascular Rashes

Levido reticularis
                         www.londonlupuscentre.co.uk




Cutaneous vasculitis

                               www.aclaic.org




Purpura & ecchymoses



                            www.medscape.com           34
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
Musculoskeletal System
•   Joints
•   Muscles
•   Bone
•   Supporting structures-
    tendons, ligaments, bur
    sae                       www.msnbc.msn.com




                                                  36
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
Heart and Lungs
Heart
•Endocardium-inside
•Myocardium-heart muscle
•Pericardium-outside lining




                              Lungs
                              •Pleurisy/pleural effusion
                              •Acute lupus pneumonitis
                              •Diffuse interstitial lung disease
                              •Pulmonary embolism              38
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
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
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
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
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
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
Antiphospholipid Syndrome




                            45
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
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
Medications
Only 4 medications approved by the FDA
  specifically for the treatment of lupus
• Aspirin
• Corticosteroids (1955)
• Hydroxychloroquine (1955)
• Belimumab (Benlysta) (2011)
Off Label Medications
•   Prescription anti-inflammatories
•   Neurological medications (Lyrica, Neurontin, etc.)
•   Anti-depressants (Lexapro, Cymbalta, etc. )
•   Chemotherapies (cytoxan, azathioprine, etc.)
•   Anti-rejection medications (Cellcept)
•   DMARDs (Disease Modifying Anti-Rheumatic
    Drugs-monoclonal antibodies-
    Rituximab, belimimumab.)
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
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
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).

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Lupus

  • 1. Understanding Lupus Lupus Foundation of Florida www.lupusflorida.org 1
  • 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.
  • 7. What is lupus? • Chronic • Autoimmune • Inflammatory 7
  • 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
  • 15. Other Autoimmune Disorders 23.5 million Americans Multiple sclerosis Crohn’s Disease-ulcerative colitis Insulin dependent diabetes (type I) Fibromyalgia-fibromyositis Celiac Sprue-Dermatitis Herpetiformus Grave’s disease Mixed connective tissue disease Psoriasis Rheumatoid arthritis Myasthenia gravis Vitiligo Alopecia areata Raynaud’s Scleroderma www.aarda.org 15
  • 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
  • 26. Other Diagnostic Tests •Chest X-ray •Kidney biopsy •Neurological exam •Skin biopsy •Bone marrow biopsy
  • 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
  • 34. Vascular Rashes Levido reticularis www.londonlupuscentre.co.uk Cutaneous vasculitis www.aclaic.org Purpura & ecchymoses www.medscape.com 34
  • 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
  • 38. Heart and Lungs Heart •Endocardium-inside •Myocardium-heart muscle •Pericardium-outside lining Lungs •Pleurisy/pleural effusion •Acute lupus pneumonitis •Diffuse interstitial lung disease •Pulmonary embolism 38
  • 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)
  • 49. Off Label Medications • Prescription anti-inflammatories • Neurological medications (Lyrica, Neurontin, etc.) • Anti-depressants (Lexapro, Cymbalta, etc. ) • Chemotherapies (cytoxan, azathioprine, etc.) • Anti-rejection medications (Cellcept) • DMARDs (Disease Modifying Anti-Rheumatic Drugs-monoclonal antibodies- Rituximab, belimimumab.)
  • 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).