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Lupus

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    Lupus Lupus Presentation Transcript

    • Understanding LupusLupus Foundation of Florida www.lupusflorida.org 1
    • DisclaimerThis program is not intended to dispense medicaladvice, directly or indirectly. The purpose of thisprogram is to provide the public with information,not to prescribe or diagnose. Lupus is a complicateddisorder that requires the regular care of a qualifiedphysician. 2
    • Lupus is not… • HIV/AIDS • Leukemia • Cancer • Contagious • Your fault • All in your head 3
    • Warning Signs of LupusWhen 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 diagnosishard. The most common complaint people have is fatigue that is so severe it stopsthem from being able to function normally. This fatigue is often related tofibromyalgia. Fever, muscle and joint pain are also quite common.Muscle & Joint Pain95% of people with lupus experience muscle and joint pain.Fever Greater Than 100° F90% of people with lupus get a fever of more than 100 degrees Fahrenheit (38degrees Celsius).Prolonged or Extreme Fatigue81% of people with lupus suffer from prolonged or extreme fatigue.Anemia71% of people with lupus simultaneously suffer from anemia.
    • Kidney Problems50% of people with lupus find that their kidneys are affected. Symptoms includeweight gain, swollen ankles, high blood pressure, and decreased kidney function.Pain in the Chest45% of people with lupus experience a pain in their chest upon breathing deeply.This condition is called pleurisy.Rashes80% of people with lupus observe rashes on their skin. A butterfly-shaped rashacross the cheeks and nose is especially common (42%).Light Sensitivity30% of people with lupus become sensitive to light, a condition calledphotosensitivity.Hair Loss27% of people with lupus experience hair loss resulting in patchy or bald spots.
    • Abnormal Blood Clotting20% of people with lupus report abnormal blood clotting problems.Eye Disease20% of people with lupus concurrently suffer from eye complications such asdry eyes, eye inflammation, and eyelid rashes.Seizures15% of people with lupus experience seizures.Mouth or Nose Ulcers12% 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 SystemDefending the Body Against Invaders Retrieved from www.lymphoma.org on 10/23/07Retrieved from www.humanillness.com on 11/5/07 9
    • Autoimmune & Inflammatory Genetic predisposition (5-10% risk) + Triggers (stress, sunlight, infection) + Inadequate suppressor T cellsAutoantibodies & surplus of antibodies B cells Aladjem (1988) 10
    • Immune system vs. Blood Cells Antibodies attach to blood cellsRed 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 complexesDefective clearance of immune complexes Immune complex + serum proteinsMistakenly deposit in organs other than spleen Activation of complement systemInflammation 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<1:40Consider referral to rheumatologist for full Strong argument against SLE- SLE evaluation including alternative explanation for organ1. ACR diagnostic criteria system manifestations should be2. Laboratory tests pursuedZero to three Four or more Explanation found No explanation foundACR criteria ACR criteria Sufficient to Consider referral to rule out SLE rheumatologist ifNo SLE or SLE question of SLE orincomplete 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 LupusMouth or nose sores Oral thrush www.hss.edu Alopecia-hair loss www.hss.edu 31
    • Cutaneous Features of LupusButterfly (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 vesselswww.immunologyclinic.com 33
    • Vascular RashesLevido reticularis www.londonlupuscentre.co.ukCutaneous vasculitis www.aclaic.orgPurpura & ecchymoses www.medscape.com 34
    • Other Skin Disorders in LupusLupus panniculitis (profundus)• Inflamed fat pads in skin• Rare• Responds to treatment www.merckmedicus.com www.accessmedicine.com• 1 in 200Blisters (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 LungsHeart•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 TractLupus primarily affects the glomerulus•Usually no specific complaints traceable to kidney•Other causes of “kidney” pain-pleurisy, kidney stone, kidneyinfection, muscle spasm in lumbar spine 41
    • Lupus in the KidneyPatient 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 KidneyBlood and urine tests• Blood urea nitrogen (BUN)• Creatinine clearance• Casts (cellular debris)• ProteinBiopsy1. No disease2. Mesangial3. Focal proliferative4. Diffuse proliferative5. Membranous6. 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 baddays exceed the number of good ones orwhen there are more bad days than thereused 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
    • MedicationsOnly 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 ageof 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
    • SourcesAmerican 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 Diseaseshttp://www.niams.nih.gov/Lupus Initiative www.thelupusinitiative.org/Lupus Research Institute http://www.lupusresearch.org/Lahita, Robert, ed. Systemic Lupus Erythematosus: Fourth Edition. SanDiego, CA: Academic Press 2004.Wallace, Daniel. The Lupus Book: A Guide for Patients and Their Families,4th ed. Oxford: Oxford University Press (2009).