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SLE             BYDR. EHAB ELTORABY     MD GENERAL MEDICINERHEUMATOLOGY &IMMUNOLOGY UNIT
Agenda  Definition of SLE  Causes of SLE  Clinical Picture of SLE  Investigations of SLE  Treatment of SLE
Definition   Systemic lupus erythematosus (SLE) is    a multi-system auto-immune disease    that is caused by tissue dama...
Definition (Cont.)   The immune system loses the ability to    differentiate between foreign cells and    it’s own cells ...
INCIDENCE OF LUPUS   Between 500,000 to 1.5 million    Americans have lupus.   80-90% of lupus patients are female.   8...
WHAT CAUSES LUPUS? Genetics   Hormones     Environment
Etiology   The specific cause is unknown   Genetic factors may play a role   Environmental agents   Drugs or other che...
LUPUS IS…   Different for each person.   A disease that ranges from mild to life    threatening.   Characterized by fla...
TYPES OF LUPUS   Discoid or Cutaneous Lupus (DLE)   Drug Induced Lupus (DIL)   Neonatal Lupus   Systemic Lupus Erythem...
DISCOID LUPUS   Affects the skin, hair or mucous    membranes.   Identified by a rash or lesions.   Diagnosed by biopsy...
Discoid Rash
DRUG INDUCED LUPUS   Develops after long-term use of certain    medications.   Most common in men over 50 years old.   ...
NEONATAL LUPUS   Occurs when the mother’s antibodies cross over    the placenta to the baby.   Can affect the skin, hear...
SYSTEMIC LUPUS       ERYTHEMATOSUS   Can affect any organ in the body    including the joints, skin, lungs, heart,    blo...
COMMONSYMPTOMS OF SLE    Achy joints    Fever    Fatigue    Skin Rashes
OTHER            LUPUS SYMPTOMS    Chest pain                Headache    Hair loss                 Dizziness    Mouth...
ORGAN INVOLVEMENT       WITH LUPUS   Kidneys              Blood vessels   Lungs                Blood   Central nervou...
DIAGNOSING LUPUS   Medical history (including family history)   Complete physical examination   Laboratory tests   Ski...
ACR DIAGNOSTIC CRITERIASkin criteria             Systemic criteria1.   Butterfly rash       5.   Arthritis2.   Discoid ras...
Criterion                                      Definition    Malar Rash     Rash over the cheeks  Discoid Rash     Red rai...
Criterion                                                    Definition   Neurologic      Seizures (convulsions) and/or ps...
Malar Rash
Oral Ulcers
Systemic Lupus Erythematosus   Head and Neck Manifestations     Malar rash first sign in 50%     Erythematous maculopap...
Musculoskeletal System    All joints can be affected,     however the wrists, knees,     ankles, elbows, and shoulders ar...
Musculoskeletal System(Cont.)    Hand deformities can include ulnar     deviation and subluxation, swan neck     deformit...
Cardiovascular System Pericarditis is the most  common cardiac  manifestation myocarditis Endocarditis with  characteri...
Pulmonary System   Pleurisy is the most common    manifestation of pulmonary    involvement   Interstitial lung disease...
Gastrointestinal Sytem   Difficulty swallowing   Gastric reflux disease   Anorexia, nausea, or vomiting may be present...
Raynaud’s Phenomena     Http://www.dermis.net/doi      a
Renal manifestations   Tend to appear within the 1st 2yrs of SLE   Almost ½ have asymptomatic urine    abnormalities    ...
Nervous System   Neuropsychiatric manifestations of    lupus occur frequently       May be mild to severe       Any loc...
Nervous System (Cont.)   Central Nerve System      Acute confusional       state      Psychosis      Anxiety disorder ...
Nervous System (Cont.)   Peripheral Nerve System      Cranial neuropathy      Mononeuropathy      Polyneuropathy
COMMON LABORATORY      TESTS   Antinuclear Antibody (ANA)   Anti DNA   Anti-Sm   Anti-RNP   Anti-Ro   Anti-La
OTHER LABORATORY                TESTS   CBC (RBC, WBC, platelets)      Rheumatoid Factor   Urinalysis                  ...
EFFECT OF LABORATORY TESTS      WITH INCREASED LUPUS ACTIVITYC reactive protein (CRP) Sedimentation rate (ESR)      C...
LUPUS TREATMENT    Team effort    Tailored    Tentative
LUPUS PHYSICIANS    Family Practitioner    Internist    Rheumatologist    Clinical Immunologist    Dermatologist    ...
COMMONLUPUS MEDICATIONS   NSAIDs   Antimalarials   Corticosteroids   Immunosuppressants   Investigational (research)
NSAIDs   Aspirin   Ibuprofen   Indomethacin
CORTICOSTEROIDS    Prednisone    Prednisolone    Methylprednisolone    Hydrocortisone
ANTIMALARIALS   Chloroquine
IMMUNE SUPPRESSANTS   Cyclophosphamide   Azathioprine
PREVENTIVE MEASURES      Sun precautions      Rest      Nutrition/diet      Exercise      Moist heat      Prevent in...
Th a n k Y o u
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  • It is estimated that between 500,000 to 1.5 million Americans have lupus. More than 16,000 Americans develop lupus each year. Both men and women can get lupus, however, lupus is 10-15 times more common in females than males. 80-90% of lupus patients are female. Although children, teenagers and the elderly can get lupus, it most commonly affects women of childbearing years between the ages of 22 and 40. 80% of lupus patients are between 15 and 45 years of age. Lupus disproportionately affects African Americans, Asian Americans, Hispanics, and Native Americans. African American women are 3 times more likely than Caucasian women to be affected by lupus.
  • Lupus is an extremely complex disease, and although scientists are making progress in understanding the causes of lupus, there is still no single known cause. It is thought that a combination of genetics, environment, and possibly hormones act together to trigger the disease. Genetics: There is considerable evidence indicating that genes play a major role in the disease process. Researchers believe that there may be as many as 100 genes, which contribute to the genetic predisposition and development of SLE, and they have recently discovered a single gene that causes a lupus-like illness in mice. Hormones: The effect of hormones in humans with lupus is not clear. However, because the majority of lupus patients are women in their childbearing years, it seemed a logical aspect to study. Female hormones tend to stimulate the immune system or promote an immune response, (remember that having lupus means having an overactive immune system), whereas male hormones have the opposite effect and are more immunosuppressive. There does not seem to be any evidence that men or women with lupus produce abnormal levels of hormones, however, there may be differences in the way people with lupus process these hormones. Environment: Although it has not yet been fully proven, there may be certain environmental factors that play a role in initiating or triggering lupus in a genetically predisposed person.
  • Since lupus can attack any organ or organ system in the body, it affects everyone differently and there are no two cases that are alike. It can range from mild to life threatening and anywhere in between. People with lupus generally go through periods of flares , which are sudden increases in disease activity or development of new symptoms, and remissions, which are periods of disease-free activity.
  • The term "lupus" is commonly used when talking about any form of the disease. When someone says, "I have lupus," he or she could be affected in many different ways depending on the type of lupus present. Even within the same type of lupus, each case is different. The different types of lupus include: ·         Discoid or Cutaneous Lupus (DLE) ·         Drug Induced Lupus (DIL) ·         Neonatal Lupus ·         Systemic Lupus Erythematous (SLE)
  • Discoid or Cutaneous lupus (skin) lupus (DLE) : affects primarily the skin, but may also involve the hair and mucous membranes . There is more than one type of cutaneous lupus, which can cause different looking rashes and symptoms. These include: Acute cutaneous lupus erythematosus (ACLE) Subacute cutaneous lupus erythematosus (SCLE) Chronic cutaneous lupus erythematosus (CCLE) or Discoid lupus erythematosus (DLE) Cutaneous lupus is usually identified by a rash or lesions that appear on the face, neck, and/or scalp (or other sun exposed areas). The lesions can appear patchy, crusty and cause scarring. Cutaneous lupus does not generally involve the body's internal organs. Therefore, the ANA test , which is a blood test used to help diagnose systemic lupus, may be negative in these patients. However, in some patients with discoid lupus, the ANA test is positive, but at a low level or "titer ." Cutaneous lupus is diagnosed by examining a biopsy of the skin rash . In approximately 10 percent of patients, cutaneous lupus will evolve into the systemic form of the disease . This cannot be predicted or prevented. Treatment of cutaneous lupus will not prevent its progression to the systemic form, and individuals who progress to the systemic form probably had systemic lupus with the rash as their initial symptom. Topical and interlesional corticosteroids are usually effective for localized lesions , antimalarials or thalidomide may be needed for more generalized lesions.
  • Although the exact cause is unknown, Drug Induced Lupus can develop after long-term use of certain medications . It is most common in men over 50 years of age (because they are given the offending drugs more often). The symptoms of drug-induced lupus are similar to those of systemic lupus and are mild in most people, but can become debilitating if the person continues to take the offending medication. It usually takes several months to years of continuous therapy with the medication before symptoms appear. Once the suspected medication is stopped, symptoms should go away within days. Usually symptoms disappear within one or two weeks and the symptoms usually disappear completely within six months . The antinuclear antibody test (ANA) may stay positive for years .   At least 38 drugs currently in use can cause DIL . However, most cases have been associated with the following: Hydralazine (Apresoline) Procainamide (Procan, Pronestyl) Methyldopa (Aldomet) Quinidine (Quinaglute) Isoniazid (INH) Some anti-seizure medications such as phenytoin (Dilantin) or carbamazepine (Tegretol)   The most important aspect of treating drug-induced lupus is to recognize the medication that is causing the problem and discontinue its use . This step is often sufficient to improve the symptoms within a few days. Individuals sometimes improve more quickly if non-steroidal anti-inflammatory drugs (NSAIDs) are then used and corticosteroids may be used for patients with severe symptoms of DIL.
  • Neonatal lupus is not SLE or Cutaneous lupus . Neonatal lupus occurs when the mother’s antibodies cross over the placenta to the baby . This can affect the skin, heart, liver and/or blood of the fetus and newborn . It is associated with a rash that appears within the first several weeks of life and may persist for about six months before disappearing. Some babies with neonatal lupus may have a serious heart defect called congenital heart block . This disorder can usually be controlled by placing a pacemaker in the baby . Congenital heart block is much less common than the skin rash. Early detection and treatment is vital in achieving a positive outcome for the baby and because doctors can now identify most at risk patients, neonatal lupus is very rare and most infants of mothers with SLE are entirely healthy.
  • Systemic lupus erythematosus (SLE) can affect any system or organ in the body including the joints, skin, lungs, heart, blood, kidney, or nervous system . Symptoms of SLE can range from being minor to very serious or life threatening . A person may experience very little to no pain or they may experience extreme pain, especially in the joints. There may be no skin involvement or rashes that are disfiguring. They may have no organ involvement or extreme organ damage. Generally, no two people with systemic lupus will have identical symptoms . Most often when people mention "lupus," they are referring to the systemic form of the disease.
  • Although SLE can affect any part of the body, most people experience symptoms in only a few organs. Common symptoms include painful or swollen joints, unexplained fever, skin rashes , and extreme fatigue . A characteristic skin rash--the butterfly rash may appear across the nose and cheeks. Other rashes occur elsewhere on the face and ears, upper arms, shoulders, chest, and hands.
  • Other symptoms of lupus include chest pain , hair loss, mouth sores, sensitivity to the sun , anemia (a decrease in red blood cells), and pale or purple fingers and toes from cold and/or stress (Raynaud’s phenomenon). SLE patients can also experience repeated miscarriages, headaches, dizziness, depression, seizures, cognitive dysfunction and/or memory disturbances. New symptoms may continue to appear years after the initial diagnosis, and different symptoms can occur at any time during a person’s lifetime.
  • In many people with lupus, only one system of the body such as the skin or joints is affected. Other people experience symptoms in many parts of their body. Just how seriously a body system is affected can also vary from person to person. The following systems in the body can be affected by lupus: Kidneys Lungs Central nervous system Blood vessels Blood Heart
  • Because lupus symptoms can come and go, are usually vague and often mimic other illnesses, it can be a very difficult disease to diagnose. It may take months or even years for doctors to piece together the symptoms to diagnose this complex disease. The onset of lupus may be acute, resembling an infectious process consisting of fever, pleurisy and muscle aches or it may be a progression of vague symptoms over a long period of time. Because there is no one single test that can tell if a person has lupus, diagnosis is usually made by a careful review of a person’s entire medical history (including family history), complete physical examination, and analysis of laboratory tests. Sometimes a biopsy of the skin or kidney may be used to help with the diagnosis. The symptoms described by the patient may appear to be totally unrelated; therefore an open mind and good communication is necessary when assessing the patient.
  • To assist the physician in the diagnosis of lupus, the American College of Rheumatology (ACR) has developed a set of classification criteria used to help distinguish lupus from other diseases. A person should have four or more of these symptoms to suspect lupus. It is important to remember that the symptoms do not have to occur at the same time, and the criteria do not include all possible symptoms of lupus like fever, fatigue, hair loss, or Raynaud's phenomenon. The criteria are meant only to help with diagnosis. Butterfly or Malar Rash Rash over the cheeks and/or nose. Discoid Rash Red raised patches anywhere on the skin, but usually in the sun exposed areas of the face, arms, neck, hands. Photosensitivity Reaction to sunlight, resulting in the development or increase in skin rash or general feeling of illness. Oral Ulcers Ulcers in the nose or mouth, usually painless. Arthritis Non-erosive arthritis involving two or more peripheral joints (arthritis in which the bones around the joints do not become destroyed). Serositis Pleuritis or pericarditis – pain in the chest with deep breathing. Renal Disorder Excessive protein and/or cellular casts (abnormal elements the urine, derived from red and/or white cells and/or kidney tubule cells) in the urine. Neurologic Disorder Seizures (convulsions) and/or psychosis. Hematologic Disorder Hemolytic anemia (low red blood cell count), leukopenia (low white blood cell count) or thrombocytopenia (low platelet count). Immunologic Disorder Positive LE prep test, positive anti-DNA test, positive anti-Sm test or false positive syphilis test (VDRL). Antinuclear Antibody Positive test for antinuclear antibodies.
  • No single test can determine whether a person has lupus, but several laboratory tests may help the doctor to make a diagnosis. The most useful tests identify certain autoantibodies often present in the blood of people with lupus. Initial screening usually includes the antinuclear antibody test (ANA). This test is positive in 95-98% of people with lupus. However, just because a person has been told they have a positive antinuclear antibody test (ANA), does not mean they have lupus. The ANA is NOT a lupus test. It is only a test that points the doctor in several different directions. People with other rheumatic diseases and healthy relatives of people with autoimmune diseases can also have a positive ANA. It can also become positive with many other things like aging, pregnancy, viral infections and also as the result of taking certain medications. About 10 million Americans have a positive ANA and only about 1 million of them have SLE. If three or more clinical features, such as skin, joint, kidney, pleural, pericardial, hematological, or central nervous system findings are present, a positive test can confirm the disease. Anti-DNA- Are present in about 60-80% of patients with active SLE. The test is highly specific for SLE and not found in patients with other rheumatic diseases. It is also associated with a greater risk of lupus nephritis. Anti-Sm- Are present in about 30% of lupus patients. This test is highly specific for patients with SLE and rarely found with other rheumatic diseases. It is often used to confirm a lupus diagnosis. Anti-RNP is seen in SLE and mixed connective tissue disease. Anti-Ro(SSA) and Anti-La(SSB)- Anti-Ro is found in about 30% of SLE patients. It is also highly associated with photosensitivity. Anti-La is found in about 15% of lupus patients. Both of these are almost always found in babies who are born with neonatal lupus. They are also present in people with subacute cutaneous lupus, and Sjögren’s Syndrome.
  • Other laboratory tests that may be helpful include: CBC/Complete blood count- RBC/Red blood count - About 40% of patients with SLE will have problems with their RBC’s (anemia), which may be caused by iron deficiency, GI bleeding, medications or autoantibody formation to RBC’s. WBC/White blood count - About 15-20% of lupus patients have a decrease in WBC’s (leukopenia). Platelets - About 25-35% of patients with lupus have low platelets (thrombocytopenia). Urinalysis: There is no one test to tell if there is lupus disease in the kidneys. A doctor can test for kidney problems by using urine tests, kidney function tests, blood tests and/or x-rays. If red blood cells, protein and/or cell casts (fragments of dead cells from blood cells or the kidney tubes themselves) are found in a urinalysis, it may mean that the kidney is not working correctly or that there is major damage to the kidneys. In these cases, further testing may need to be done. Blood Chemistry : tests are important, especially the creatinine and urea which are raised if there has been evidence of kidney disease. ESR (erythrocyte sedimentation rate) and CRP (C-reactive protein) - These tests frequently rise with generalized inflammation. The levels are usually increased with patients who have active lupus and decrease with corticosteroid or NSAID use. Antiphospholipid Antibodies - These tests are associated with the problem of 'sticky blood'. Patients with high levels of antiphospholipid antibodies have an increased tendency to clotting or thrombosis, both in the veins and arteries, and in pregnant women with this condition, there is a risk of thrombosis leading to miscarriage. The most widely measured are the lupus anticoagulant and the anticardiolipin antibody. Rheumatoid factor is positive in 20-30% of SLE patients and 80% of Rheumatoid Arthritis patients. High levels of rheumatoid factor with low levels of ANA are more indicative of RA and not SLE. It is also seen in people with Sjögren’s Syndrome. Complement (C-3, C-4 and CH50)- If the total blood complement level is low, or the C3 or C4 values are low and the person also has a positive ANA, some weight can be added to the diagnosis of lupus. Low C3 and C4 levels in patients with a positive ANA may show the presence of active disease, especially kidney involvement. Syphilis test (VDRL or RPR)- This test may be falsely positive in lupus patients. Biopsy or tissue sample- Can be helpful in making a diagnosis and also helps to evaluate organs. The most common sites are the skin and kidney. The doctor removes a small piece of tissue and looks at it under a microscope. All of these tests serve as tools to give the doctor clues and information in making a diagnosis. The doctor will look at the entire picture--medical history, symptoms, and test results--to determine if a person has lupus.
  • An increase in lupus activity usually causes the following test results to rise CRP, sed rate, anti DNA, liver and kidney function tests (AST, ALT, BUN, Creatinine- with kidney or liver involvement), CPK (if muscle involvement is present), urine protein or cellular casts. An increase in lupus activity usually causes the following test results to fall: CBC (WBC, RBC, platelets), complement, serum albumin.
  • At present, there is no cure for lupus; however, the symptoms can be minimized with proper treatment. In developing a treatment plan, the doctor has several goals: to prevent flares, to treat flares when they do occur, and to minimize organ damage and other complications. The variations and effectiveness of treatments for lupus have increased tremendously and doctors have many more choices when treating the disease. Lupus treatment is a team effort between the patient and their physician(s). The patient should work closely with the doctor and take an active role in treatment. Good communication between everyone is extremely important. Because there are no two cases of lupus that are alike, the treatment must be tailored to the specific needs and symptoms of each person. Once lupus has been diagnosed, the doctor will develop a treatment plan based on the patient’s age, sex, health, symptoms, and lifestyle. The treatment will also be tentative and probably change over time. Ongoing medical supervision is essential to ensure that proper care is given as the course of the disease changes. The doctor and patient should reevaluate the plan regularly to ensure that it remains as effective as possible.
  • Diagnosing and treating lupus is often a team effort between the patient and several types of health care professionals. Most people usually seek the help of their Family Practitioner or Internist first, which is often sufficient. However, when unresolved questions or more complicated symptoms arise, an opinion from a specialist may be necessary. The choice of specialist(s) depends on the organ or organs involved. For example, a Dermatologist is usually seen for skin problems, and a Nephrologist for kidney problems. Most often, a Rheumatologist or Clinical Immunologist specializing in lupus or immune system disorders is recommended.
  • Medications play an important role in the care of people with SLE. For most people with lupus, effective treatment can minimize symptoms, reduce inflammation, and maintain normal body functions. Treatment approaches are based on the specific needs and symptoms of each person. The medication prescribed usually depends on which organ(s) are involved and the severity of the involvement. The choice of drugs is highly individualized and typically changes often during the course of the disease. The medications used in the management of lupus include: ·         NSAIDs ·         Antimalarials ·         Corticosteroids ·         Immunosuppressants ·        Investigational (research)
  • NSAIDs are prescribed either alone or in combination with other types of drugs to reduce the inflammation responsible for the pain and discomfort associated with lupus. Common side effects of NSAIDs can include stomach upset, heartburn, diarrhea, and fluid retention. Some patients with lupus also develop liver and kidney inflammation while taking NSAIDs, making it especially important to stay in close contact with the doctor while taking these medications. Examples include: ·         Aspirin ·         Ibuprofen (Motrin, Advil) ·         Naproxen (Naprosyn) ·         Indomethacin (Indocin) ·         Nabumetone (Relafen) A new class of anti-inflammatory drugs called COX-2 inhibitors (celecoxib [Celebrex]; rofecoxib [Vioxx]; work like NSAIDs on pain and inflammation but have a much lower risk of gastrointestinal side effects.
  • Corticosteroids (steroids) are hormones that have very potent anti-inflammatory properties. They are normally produced in small quantities by the body. Prednisone, prednisolone, methylprednisolone, and hydrocortisone are man- made or synthetic steroids. They are an important part of lupus therapy because they work well at suppressing the immune system and reducing inflammation. Unfortunately, long term use of steroids can cause serious side effects such as avascular necrosis, osteoporosis, coronary artery disease, muscle weakness, thinning of the skin, elevated blood sugar, increased appetite, cataracts, glaucoma, depression, mood swings, hypertension and increased susceptibility to infections. It can be dangerous to stop taking corticosteroids suddenly, so it is very important that the doctor and patient work together anytime the dose is changed. Sometimes doctors give very large doses of IV corticosteroids over a brief period of time. This is called “bolus" or "pulse" therapy. With this treatment, the typical side effects are less likely and slow withdrawal is not necessary.  
  • Antimalarial drugs such as Chloroquine and Plaquenil are commonly used in the management of lupus symptoms. These drugs were originally used to treat malaria, but doctors found that they are also beneficial for lupus patients. It is not exactly clear how antimalarials work, but researchers believe that they may suppress parts of the immune response, reducing inflammation. They are prescribed for fatigue, lupus arthritis, mouth ulcers and skin manifestations. It may take months before these drugs demonstrate beneficial effects. Antimalarials are usually tolerated well. Side effects are rare, and consist of occasional diarrhea or rashes. High dose therapy may damage the retina of the eye, causing vision problems. Eye examinations are, therefore, required every six to twelve months. With the low doses of anti-malarials used in the treatment of lupus, the risk of eye complications are extremely low. Clinical studies show that continuous treatment with antimalarials may prevent lupus flares from recurring.
  • Cytoxan, Imuran, methotrexate, and Leukeran are in a group of agents known as cytotoxic or immunosuppressive drugs. These medications are used to suppress the immune system in patients with more serious lupus manifestations such as lupus nephritis and neurologic disease in whom treatment with corticosteroids has failed. Side effects can be serious and include gastrointestinal complications, alopecia, increased risk for infections, sterility, hemorrhagic cystitis, bladder fibrosis, and possibly cancer. Researchers are looking into using combination therapies such as cyclophosphamide and prednisone for lupus nephritis. It is more effective in preserving renal function than prednisone alone an can reduce the likelihood of end-stage renal failure.
  • In combination with medications, there are preventive measures which can help to reduce the risk of lupus flares. Photosensitivity is an abnormal reaction to UV rays and results in exacerbation of lupus symptoms. About 1/3 of lupus patients are photosensitive and should avoid prolonged direct exposure to the sun. All patients should use sun precautions and apply a sunscreen with an SPF 15 or greater; avoid sun exposure between 10 a.m. and 4 pm and wear protective clothing and wide-brimmed hats. Fluorescent and halogen lights can also emit UV rays, which can aggravate lupus. There are plastic light shields available that block UV emissions. The fatigue found in lupus can be an overwhelming and debilitating symptom therefore proper and adequate rest is vital in dealing with the disease. Some patients require a nap during the day as well as 8 to 10 hours of sleep at night. Lupus patients also need to learn how to alternate activities with periods of rest. Staying in bed can cause weakness, but overdoing it can cause lupus to flare.   Nutrition and a well-balanced diet are also essential components to successfully living with a chronic disease like lupus.  There is no "lupus diet." People with lupus should eat well-balanced meals that are low in fat, low in salt, high in fiber and low in sugar. People on corticosteroids (Prednisone) should limit their sugar, fat and salt. A restricted diet may be prescribed when hypertension, kidney disease, edema or diabetes is present. Nutritional counseling may also be beneficial to some people. If you find that certain foods make you feel bad or cause a "flare-up" of your lupus, you should avoid those foods. Lupus patients are encouraged to do low impact exercise as tolerated. This can prevent muscle weakness and fatigue. Walking, stretching, range of motion, swimming, low impact aerobics and bicycling can help keep you strong and prevent thinning of bones or osteoporosis . Remember to alternate your activities with rest periods. Be careful of doing heavy weight lifting or high impact exercises as this may make your lupus worse. If you feel overly tired or have increased discomfort for more than two hours after exercising, the session was probably too much and the next session should be made shorter. Moist heat is better for aching joints than dry heat. Soaking in a tub, Jacuzzi or taking a warm shower can help make your joints feel better. The time to use ice and/or cold packs is within 36 hours of an injury. Moist heat and topical analgesic cremes are usually beneficial for muscle and joint pain but should be approved by the patient’s physician. Call your doctor if your temperature is over 99.6. It could be an infection or a lupus flare. Smoking is detrimental to anyone’s health, especially those suffering from chronic disease. Cigarette smoke contains chemicals, which can cause flares of cutaneous lupus. It can make symptoms of Raynaud's disease worse by impairing circulation (blood flow), and there can also be stomach problems from medications in people who use tobacco.  
  • Transcript of "SLE"

    1. 1. SLE BYDR. EHAB ELTORABY MD GENERAL MEDICINERHEUMATOLOGY &IMMUNOLOGY UNIT
    2. 2. Agenda Definition of SLE Causes of SLE Clinical Picture of SLE Investigations of SLE Treatment of SLE
    3. 3. Definition Systemic lupus erythematosus (SLE) is a multi-system auto-immune disease that is caused by tissue damage resulting from antibody and complement fixing immune complex deposition It is characterized by states of exacerbation and remission
    4. 4. Definition (Cont.) The immune system loses the ability to differentiate between foreign cells and it’s own cells and tissues Antibodies against the immune system are formed The immune complexes that are formed build up in the tissue causing inflammation, injury to the tissue, and pain
    5. 5. INCIDENCE OF LUPUS Between 500,000 to 1.5 million Americans have lupus. 80-90% of lupus patients are female. 80% of lupus patients are between 15 and 45 years of age. Lupus affects more African Americans, Asian Americans, Hispanics, and Native Americans than Caucasians.
    6. 6. WHAT CAUSES LUPUS? Genetics Hormones Environment
    7. 7. Etiology The specific cause is unknown Genetic factors may play a role Environmental agents Drugs or other chemical agents Dietary factors Ultraviolet radiation Infectious agents
    8. 8. LUPUS IS… Different for each person. A disease that ranges from mild to life threatening. Characterized by flares and remissions.
    9. 9. TYPES OF LUPUS Discoid or Cutaneous Lupus (DLE) Drug Induced Lupus (DIL) Neonatal Lupus Systemic Lupus Erythematous (SLE)
    10. 10. DISCOID LUPUS Affects the skin, hair or mucous membranes. Identified by a rash or lesions. Diagnosed by biopsy of rash. 10% will evolve into SLE. Treatment includes topical or interlesional steroids; antimalarials.
    11. 11. Discoid Rash
    12. 12. DRUG INDUCED LUPUS Develops after long-term use of certain medications. Most common in men over 50 years old. Symptoms are similar to SLE. Most important treatment is to recognize medication and discontinue use. Once medication is stopped, symptoms usually disappear completely within 6 months.
    13. 13. NEONATAL LUPUS Occurs when the mother’s antibodies cross over the placenta to the baby. Can affect the skin, heart, liver and/or blood of the fetus and newborn. Good prenatal care can prevent most problems.
    14. 14. SYSTEMIC LUPUS ERYTHEMATOSUS Can affect any organ in the body including the joints, skin, lungs, heart, blood, kidney, or nervous system. Can range from mild to life threatening. No two people will have identical symptoms.
    15. 15. COMMONSYMPTOMS OF SLE  Achy joints  Fever  Fatigue  Skin Rashes
    16. 16. OTHER LUPUS SYMPTOMS Chest pain  Headache Hair loss  Dizziness Mouth sores  Depression Photosensitivity  Seizures Anemia  Memory disturbances Repeated miscarriages
    17. 17. ORGAN INVOLVEMENT WITH LUPUS Kidneys  Blood vessels Lungs  Blood Central nervous  Heart system
    18. 18. DIAGNOSING LUPUS Medical history (including family history) Complete physical examination Laboratory tests Skin or kidney biopsy
    19. 19. ACR DIAGNOSTIC CRITERIASkin criteria Systemic criteria1. Butterfly rash 5. Arthritis2. Discoid rash 6. Serositis3. Photosensitivity 7. Kidney disorder4. Oral ulcers 8. Neurologic disorder Laboratory criteria 9. Hematologic abnormalities 10. Immunologic disorder 11. Antinuclear antibody
    20. 20. Criterion Definition Malar Rash Rash over the cheeks Discoid Rash Red raised patches DIAGNOSIS resulting in the development of or increasePhotosensitivity Reaction to sunlight, in skin rash Oral Ulcers Ulcers in the nose or mouth, usually painless Arthritis Nonerosive arthritis involving two or more peripheral joints (arthritis in which the bones around the joints do not become destroyed) Serositis Pleuritis or pericarditis (inflammation of the lining of the lung or heart)Renal Disorder Excessive protein in the urine (greater than 0.5 gm/day or 3+ on test sticks) and/or cellular casts (abnormal elements the urine, derived from red and/or white cells and/or kidney tubule cells)
    21. 21. Criterion Definition Neurologic Seizures (convulsions) and/or psychosis in the absence of Disorder drugs or metabolic disturbances which are known to cause DIAGNOSIS such effects Hematologic Hemolytic anemia , leukopenia , lymphopenia or Disorder thrombocytopenia. The leukopenia and lymphopenia must be detected on two or more occasions. The thrombocytopenia must be detected in the absence of drugs known to induce it. Antinuclear Positive test for antinuclear antibodies (ANA) in the absence Antibody of drugs known to induce it. Immunologic Positive anti-double stranded anti-DNA test, positive anti-Sm Disorder test, positive antiphospholipid antibody such as anticardiolipin, or false positive syphilis test (VDRL). Adapted from: Tan, E.M., et. al. The 1982 Revised Criteria for the Classification of SLE. Arth Rheum 25: 1271-1277.
    22. 22. Malar Rash
    23. 23. Oral Ulcers
    24. 24. Systemic Lupus Erythematosus Head and Neck Manifestations  Malar rash first sign in 50%  Erythematous maculopapular eruption after sun exposure  Oral ulceration
    25. 25. Musculoskeletal System  All joints can be affected, however the wrists, knees, ankles, elbows, and shoulders are most common
    26. 26. Musculoskeletal System(Cont.)  Hand deformities can include ulnar deviation and subluxation, swan neck deformities, and subluxation of thumb interphalangeal joints  Reversible subluxation has been observed in the knees
    27. 27. Cardiovascular System Pericarditis is the most common cardiac manifestation myocarditis Endocarditis with characteristic lesion of the cardiac valve
    28. 28. Pulmonary System Pleurisy is the most common manifestation of pulmonary involvement Interstitial lung disease Pulmonary embolisms
    29. 29. Gastrointestinal Sytem Difficulty swallowing Gastric reflux disease Anorexia, nausea, or vomiting may be present Pancreatitis
    30. 30. Raynaud’s Phenomena Http://www.dermis.net/doi a
    31. 31. Renal manifestations Tend to appear within the 1st 2yrs of SLE Almost ½ have asymptomatic urine abnormalities  Proteinuria - dominant feature  Haematuria – almost always present but not in isolation
    32. 32. Nervous System Neuropsychiatric manifestations of lupus occur frequently  May be mild to severe  Any location in the nerve system may be affected (brain, spinal cord, peripheral system)
    33. 33. Nervous System (Cont.) Central Nerve System  Acute confusional state  Psychosis  Anxiety disorder  Headache  Cerebrovascular disease
    34. 34. Nervous System (Cont.) Peripheral Nerve System  Cranial neuropathy  Mononeuropathy  Polyneuropathy
    35. 35. COMMON LABORATORY TESTS Antinuclear Antibody (ANA) Anti DNA Anti-Sm Anti-RNP Anti-Ro Anti-La
    36. 36. OTHER LABORATORY TESTS CBC (RBC, WBC, platelets)  Rheumatoid Factor Urinalysis  Skin biopsy Sedimentation Rate (ESR)  Kidney Biopsy
    37. 37. EFFECT OF LABORATORY TESTS WITH INCREASED LUPUS ACTIVITYC reactive protein (CRP) Sedimentation rate (ESR) CBC (WBC, RBC, Anti DNA platelets) Liver and Kidney Complement Function tests Serum albumin CPK Urine protein or cell casts
    38. 38. LUPUS TREATMENT  Team effort  Tailored  Tentative
    39. 39. LUPUS PHYSICIANS  Family Practitioner  Internist  Rheumatologist  Clinical Immunologist  Dermatologist  Nephrologist  Other specialists
    40. 40. COMMONLUPUS MEDICATIONS NSAIDs Antimalarials Corticosteroids Immunosuppressants Investigational (research)
    41. 41. NSAIDs Aspirin Ibuprofen Indomethacin
    42. 42. CORTICOSTEROIDS  Prednisone  Prednisolone  Methylprednisolone  Hydrocortisone
    43. 43. ANTIMALARIALS Chloroquine
    44. 44. IMMUNE SUPPRESSANTS Cyclophosphamide Azathioprine
    45. 45. PREVENTIVE MEASURES  Sun precautions  Rest  Nutrition/diet  Exercise  Moist heat  Prevent infection  Don’t smoke
    46. 46. Th a n k Y o u
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