Lupus erythematosus



Lupus erythematosus

Is chronic auto immune disease

that can be fatal, though with recent medical advances, fatalities are becoming increasingly rare

the attacks the body’s cells and tissue, resulting in inflammation and tissue damage. SLE can affect any part of the body, but most often harms the, , , the skin ,joints ,lung .kidney ,blood vesseles and central nervous system.

The course of the disease is unpredictable, with periods of illness (called flares) alternating with remission.

Lupus can occur at any age, and is most common in women, particularly black young people

Lupus is treatable, mainly with stroid and immunosupresive and, though there is currently no cure. However, many people with Lupus lead long and substantial lives

Classification:

systemic lupus erythematosus

drug induce lupus

discoid lupus

subacute cut lupus

neonatal lupus

Signs and symptoms SLE is one of several diseases known as "the great imitators. because its symptoms vary so widely it often mimics or is mistaken for other illnesses, and because the symptoms come and go unpredictably. Diagnosis can be elusive, with patients sometimes suffering unexplained symptoms and untreated SLE for years. Common initial and chronic complaints fever malaise

,arthrlagia ,skin rash,fatigue ,mylagia .

Joints manifestations:arthralgia, arthritis mono or oligo or poly type deforming but not erosive like RA. ,myositis and myopathy also happen, affect 70%.

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Dermatological manifestations

As many as 30% of patients present with some dermatological symptoms (and 65% suffer such symptoms at some point), with 30% to 50% suffering from the classic malar rash Patients may present with discoid lupus (thick, red scaly patches on the skin.alopacia,mouth,nasal and vaginal ulcer.

Musculoskeletal manifestations: Patients most often seek medical attention forjoint pain 90% of them during the course of the illness with or with out muscle pain unlike RA lupus arthritis less disable with out distraction of the joint few than 10% have joint deformites of hand and feet.

Hematological manifestations

Anemia and iron deficiency may develop in as many as half of patients and low platelets and WBC may be due to the disease or a side-effect of pharmacological treatment. patients may have antiphosphlipid syndrome with thrombosis where autoantibodies to phospholipids are present in the patient's serum. Abnormalities associated with antiphospholipid antibody syndrome include a paradoxical prolonged PTT (which usually occurs in hemorrhagic disorders) and a positive test for antiphospholipid antibodies with anticardiolipin may be positive and false positive VDRL.

Cardiac manifestations:

Patients may present with inflammation of various parts of the OF THE HEART like myocarditis, endocarditis and pericarditis , endocarditis of SLE is characteristically non-infective[libman-sacks]mostly involve mitral and tricaspid.atherosclrosis occur more in SLE patients.

Pulmonary manifestations

Like plurtic involvement and hemmorgic lung, intersial fibrosis and infection .pulmonary infraction and hypertension.

Renal invlovment: one of the major that involved in SEL and cause morbidity and mortality after infection. and lead to hypertension ,it manifested by edema and oliguria and diagnosed by GUE where found RBC cast ,granular cast, and proteinuria with increased creatinine and blood urea, Because of early recognition and management of SLE, end stage renal failure occurs in less than 5% of patients.

There are six stages of renal involvement.

Neurological manifestations:

10% have CNS affection manifested by seizer or psychological abnormities or headache and septic meningitis.

Other:

Systemic vasculitis , GIT involvement, lupus cystitis, and pancreatis.

Causes:

Still the cause unknown. its chronic inflammatory disease ,type three hypersensitivity,with potential type 2 involvement. characterised by the body's production of antibodies against the nuclear components of its own cells. There are three mechanisms by which lupus is thought to develop: genetic predisposition, environmental triggers and drug reaction (drug-induced lupus).

Genetics

Lupus run in families, but no single "lupus gene" has yet been identified. Instead, multiple genes appear to influence a person's chance of lupus developing when triggered by environmental factors. The most important genes are located at 6 chromosome. where mutations may occur randomly .

Environmental triggers:

The second mechanism may be due to environmental factors. These factors may not only exacerbate existing lupus conditions, but also trigger the initial onset. They include certain medications like antidepressant and antibiotics , extreme stress, exposure to sunlight, hormones, and infections. Some researchers have sought to find a connection between certain infectious agents like bacteria or virus but no pathogen is linked. UV radiation has been shown to trigger the photosensitive lupus rash, but some evidence also suggests that UV light is capable of altering the structure of the DNA, leading to the creation of auto antibodies.

: Drug reactions

is a reversible condition that usually occurs in patients being treated for a long-term illness. Drug-induced lupus mimics systemic lupus. However, symptoms of drug-induced lupus generally disappear once a patient is taken off the medication which triggered the episode. There are about 400 medications currently in use that can cause this condition, though the most common drugs,procaimaide,hydralzine and quindine.

Non-SLE forms of lupus:

Discoid (cutaneous) lupus is limited to skin symptoms and is diagnosed via biopsy of skin rash on the face, neck or scalp and ANF USUALLY negative ,10% change to SLE.

Risk factors

While doctors don't know what causes lupus in many cases, they have identified factors that may increase your risk of the disease, including:

• Sex. Lupus is more common in women than in men.

• Age. Although lupus affects people of all ages, including infants, children and older adults, it's most often diagnosed between the ages of 15 and 45.

• Race. Lupus is more common in blacks and in Asians.

• Sunlight. Exposure to the sun may bring on lupus skin lesions or trigger an internal response in susceptible people. Exactly why ultraviolet radiation has this effect isn't well understood, but scientists suspect that sunlight may cause skin cells to express certain proteins on their surface. Antibodies that are normally present in the body then latch onto these proteins, initiating an inflammatory response. Damaged skin cells also seem to die more frequently in people with lupus, leading to even more inflammation.

• Certain prescription medications. Drug-induced lupus results from the long-term use of certain prescription drugs. Although many medications can potentially trigger lupus, examples of drugs most clearly linked with the disease include the antipsychotic chlorpromazine, high blood pressure medications such as hydralazine, the tuberculosis drug isoniazid and the heart medication procainamide, among others. It usually takes several months or years of therapy with these drugs before symptoms appear, and even then, only a small percentage of people will ever develop lupus.

• Infection with Epstein-Barr virus. Almost everyone has been infected with a common human virus called Epstein-Barr virus. Epstein-Barr virus causes nonspecific signs and symptoms, such as fever and sore throat. Once the initial infection subsides, the virus remains dormant in the cells of your immune system unless something reactivates the virus. For reasons that aren't clear, recurrent Epstein-Barr infections seem to increase the risk of developing lupus.

• Exposure to chemicals. It's difficult to prove that chemicals can cause or increase the risk of a disease. But some studies have shown that people who work in jobs that involve exposure to mercury and silica may have an increased risk of lupus

Path physiology:

a disturbance of the normal functioning of the body. One manifestation of lupus is abnormalities in apoptosis, a type of programmed cell death in which aging or damaged cells are neatly disposed of as a part of normal growth or functioning.

Tests and diagnosis

Diagnosing lupus is difficult because signs and symptoms vary considerably from person to person. Signs and symptoms of lupus may change over time and overlap with those of many other disorders. For these reasons, doctors may not initially consider lupus until the signs and symptoms become more obvious. Even then, lupus can be challenging to diagnose because nearly all people with lupus experience fluctuations in disease activity. At times the disease may become severe and at other times subside completely.

American College of Rheumatology criteria for a lupus diagnosis

The American College of Rheumatology (ACR) has developed clinical and laboratory criteria to help physicians diagnose and classify lupus. If have four of the 11 criteria at one time or individually over time, probably have lupus. Your doctor may also consider the diagnosis of lupus even if you have fewer than four of these signs and symptoms. The criteria identified by the ACR include:

• Face rash, which doctors call a malar rash, that is butterfly shaped and covers the bridge of the nose and spreads across the cheeks

• Scaly rash, called a discoid rash, which appears as raised, scaly patches

• Sun-related rash, which appears after exposure to sunlight

• Mouth sores, which are usually painless

• Joint pain and swelling that occurs in two or more joints

• Swelling of the linings around the lungs or the heart

• Kidney disease

• A neurological disorder, such as seizures or psychosis

• Low blood counts, such as low red blood count, low platelet count (thrombocytopenia), or a low white cell count (leukopenia)

• Positive anti-nuclear antibody tests, which indicate that you may have an autoimmune disease

• Other positive blood tests that may indicate an autoimmune disease, such as a positive double-stranded anti-DNA test, positive anti-Sm test, positive anti-phospholipid antibody test or false-positive syphilis test

Laboratory tests

Your doctor may order blood and urine tests to determine your diagnosis, including:

• Complete blood count. This test measures the number of red blood cells, white blood cells and platelets as well as the amount of hemoglobin, a protein in red blood cells. Results may indicate you have anemia, which commonly occurs in lupus. A low white blood cell or platelet count may occur in lupus as well.

• Erythrocyte sedimentation rate. This blood test determines the rate at which red blood cells settle to the bottom of a tube in an hour. A faster than normal rate may indicate a systemic disease, such as lupus. The sedimentation rate isn't specific for any one disease, but it may be elevated if you have lupus, another inflammatory condition or an infection.

• Kidney and liver assessment. Blood tests can assess how well your kidneys and liver are functioning. Lupus can affect these organs.

• Urinalysis. An examination of a sample of your urine may show an increased protein level or red blood cells in the urine, which may occur if lupus has affected your kidneys.

• Antinuclear antibody (ANA) test. A positive test for the presence of these antibodies — produced by your immune system — indicates a stimulated immune system, which is common in lupus and other autoimmune diseases. A positive ANA doesn't always mean that you have lupus, however. ANA levels can be elevated if you have an infection or if you're taking certain medications. If you test positive for ANA, your doctor may advise more-specific antibody testing and refer you to a rheumatologist, a doctor who specializes in musculoskeletal and autoimmune disorders such as arthritis or lupus.

• Chest X-ray. An image of your chest may reveal abnormal shadows that suggest fluid or inflammation in your lungs. It may also show an enlarged heart as a result of a buildup of fluid within the pericardium (pericardial effusion).

• Electrocardiogram (ECG). This test measures the pattern of electrical impulses generated in your heart. It can help identify irregular rhythms or damage.

• Syphilis test. A false-positive result on a syphilis test can indicate anti-phospholipid antibodies in your blood, another indication of lupus. The presence of anti-phospholipid antibodies has been associated with an increased risk of blood clots, strokes and recurrent miscarriages.

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Complications

Inflammation caused by lupus can affect many areas of your body, including your:

• Kidneys. Lupus can cause serious kidney damage, and kidney failure is one of the leading causes of death among people with lupus. A blood test called serum creatinine level is used to monitor kidney function. Signs and symptoms of kidney problems may include generalized itching, chest pain, nausea, vomiting and weight gain.

• Central nervous system. If your central nervous system is affected by lupus, you may experience headaches, dizziness, memory problems, behavior changes, even seizures.

• Blood and blood vessels. Lupus may lead to blood problems, including anemia and increased risk of bleeding or blood clotting. It can also cause inflammation of the blood vessels (vasculitis).

• Lungs. Having lupus increases your chances of developing an inflammation of the chest cavity lining (pleurisy) that can make breathing painful. You may also be more susceptible to a noninfectious form of pneumonia.

• Heart. Lupus can cause inflammation of your heart muscle (myocarditis and endocarditis), your arteries (coronary vasculitis) or heart membrane (pericarditis). Having lupus also greatly increases your risk of cardiovascular disease and heart attacks. Controlling high blood pressure and high blood cholesterol, not smoking, and getting regular exercise are essential to help reduce the risk of heart disease.

• Infection. People with lupus are vulnerable to infection because both the disease and its treatments — corticosteroid and cytotoxic drugs, in particular — affect the immune system. And in a vicious cycle, infection can bring on a lupus flare, increasing the risk of infection even more.

• Cancer. Having lupus appears to increase your risk of cancer — especially non-Hodgkin's lymphoma, which affects the lymph system; lung cancer; and liver and bile duct cancers. Immunosuppressant drugs that are sometimes used to treat lupus also can increase the risk of cancer.

• Bone tissue death (avascular necrosis). This occurs when the blood supply to a bone diminishes, often leading to tiny breaks in the bone and eventually to the bone's collapse. The hip joint is commonly affected, although avascular necrosis can occur in other bones as well. Avascular necrosis can be caused by lupus itself or by high doses of corticosteroids used to treat the disease.

• Pregnancy complications. Women with lupus have an increased risk of miscarriage. Some women with lupus experience a flare during pregnancy. Lupus increases the risk of high blood pressure during pregnancy (preeclampsia) and preterm birth

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Treatments and drugs

Treatment for lupus depends on your signs and symptoms. Determining whether your signs and symptoms should be treated and what medications to use requires a careful discussion of the benefits and risks with your doctor. As your signs and symptoms flare and subside, you and your doctor may find that you'll need to change medications or dosages.

Common medications used to treat lupus

Three types of drugs are commonly used to treat lupus when your signs and symptoms are mild or moderate. More aggressive lupus may require more aggressive drugs. In general, when first diagnosed with lupus, your doctor may discuss these medications:

• Nonsteroidal anti-inflammatory drugs. Aspirin or other nonsteroidal anti-inflammatory drugs (NSAIDs) such as naproxen sodium (Aleve) and ibuprofen (Advil, Motrin, others) may be used to treat a variety of signs and symptoms associated with lupus. NSAIDs are available over-the-counter, or stronger versions can be prescribed by your doctor. Check with your doctor before taking over-the-counter NSAIDs because some have been associated with serious side effects in people with lupus. Side effects of NSAIDs include stomach bleeding and an increased risk of heart problems.

• Antimalarial drugs. Although there's no known relationship between lupus and malaria, these medications have proved useful in treating signs and symptoms of lupus. Antimalarials may also prevent flares of the disease. Hydroxychloroquine (Plaquenil) is the most commonly prescribed antimalarial. Side effects of antimalarial drugs include vision problems and muscle weakness.

• Corticosteroids. These drugs counter the inflammation of lupus, but can have serious long-term side effects, including weight gain, easy bruising, thinning bones (osteoporosis), high blood pressure, diabetes and increased risk of infection. The risk of side effects increases with higher doses and longer term therapy. To help reduce these risks, your doctor will try to find the lowest dose that controls your symptoms and prescribe corticosteroids for the shortest possible time. Taking the drug every other day can also help reduce side effects. Corticosteroids are sometimes combined with another medication to help reduce the dose, and therefore the toxicity, of both drugs. Taking calcium and vitamin D supplements while using corticosteroids can reduce the risk of osteoporosis.

Treatment for specific signs and symptoms

What treatments you may consider depend on your signs and symptoms. Treatments for specific signs and symptoms include:

• Joint pain and swelling. Pain in your joints may be initially controlled with NSAIDs. If you experience more significant joint pain, you and your doctor may consider antimalarial drugs or corticosteroids.

• Skin rashes. Avoid skin rashes by staying out of the sun, wearing sunblock year-round and keeping your skin covered. Despite your best attempts to avoid the sun, even indoor fluorescent lighting can trigger skin rashes in people with lupus. Skin rashes are sometimes treated with topical corticosteroids. These creams are applied to the affected area to reduce the inflammation in your skin cells. Oral steroids or antimalarial drugs also can be used.

• Fatigue. Fatigue is treated by determining the underlying cause. Your fatigue may be caused by difficulty sleeping, depression or poorly controlled pain. In these cases, your doctor would treat the underlying cause. If you and your doctor can't determine a cause for your fatigue, you may consider medications such as corticosteroids and antimalarial drugs.

• Swelling around the heart and lungs. Swelling around your heart and lungs that causes chest pain may be controlled NSAIDs, antimalarial drugs or corticosteroids.

Treatment for aggressive lupus

Life-threatening cases of lupus — those including kidney problems, inflammation in the blood vessels, and central nervous system problems, such as seizures — may require more aggressive treatment. In these cases, you and your doctor may consider:

• High-dose corticosteroids. High-dose corticosteroids can be taken orally or administered through a vein in your arm (intravenously). A high-dose regimen of corticosteroids may help control dangerous signs and symptoms quickly, but can also cause serious side effects, including infections, mood swings, high blood pressure and osteoporosis. To minimize side effects, your doctor will give you the lowest dose needed to control your signs and symptoms and then reduce the dosage over time.

• Immunosuppressive drugs. Drugs that suppress the immune system may be helpful in serious cases of lupus, but can cause serious side effects. The most commonly used immunosuppressive drugs include cyclophosphamide (Cytoxan) and azathioprine (Imuran). The drug mycophenolate mofetil (CellCept), another immunosuppressant, can be used to treat lupus-related kidney problems. All three of these drugs can be taken orally, and cyclophosphamide can also be given intravenously. Side effects include an increased risk of infection, liver damage, infertility and an increased risk of cancer.

High-dose corticosteroids can be combined with immunosuppressive drugs to reduce the dosage of each drug, which may reduce the risk of side effects. Sometimes, even with aggressive treatment, your kidneys may fail. In that case, you may need kidney dialysis or, if kidney failure is permanent, a kidney transplant.

Clinical trials

Researchers are developing new treatments for lupus in clinical trials. These studies give people with lupus the chance to try new treatments, but they don't guarantee a cure. If you're interested in a clinical trial, discuss the options with your doctor to determine the benefits and risks.

Treatments being studied in clinical trials include:

• Stem cell transplant. A stem cell transplant uses your own adult stem cells to rebuild your immune system. Doctors hope this treatment can help people with life-threatening cases of lupus. Before a stem cell transplant you're given a drug that coaxes the adult stem cells out of your bone marrow and into your bloodstream. The stem cells are then filtered from your blood and frozen for later use. Strong immunosuppressive drugs are administered to wipe out your immune system. Then the adult stem cells are put back into your body where they can rebuild your immune system.

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• Dehydroepiandrosterone (DHEA). Some clinical trials have shown that a synthetic form of the hormone DHEA may improve quality of life in people with lupus, though others haven't found this. Your body uses DHEA to make male and female sex hormones. Researchers are conducting further clinical trials to better understand whether DHEA may be useful for people with lupus.

• Rituximab (Rituxan). Rituximab decreases the number of B cells, a type of white blood cell, in body and has shown some promise in treating lupus in people who haven't responded to other immunosuppressants. However, rituximab has been linked to a fatal brain infection in two people with lupus.

• New antilymphocyte agent :

Belimumab, a recently approved monoclonal antibody for

the treatment of active SLE, targets the following molecules

B lymphocyte stimulator (BLyS; also known as BAFF, B-cell activating factor).

Lifestyle changes:

Other measures such as avoiding sunlight or covering up with sun protective clothing can also be effective in preventing problems due to photosensitivity. Weight loss is also recommended in overweight and obese patients to alleviate some of the effects of the disease, especially where joint involvement is significant.

Prevention:

While most infants born to mothers with lupus are healthy, pregnant mothers with SLE should remain under a doctor's care until delivery. Neonatal lupus is rare, but identification of mothers at highest risk for complications allows for prompt treatment before or after birth. In addition, SLE can flare during pregnancy and proper treatment can maintain the health of the mother for longer. Women pregnant and known to have the antibodies for anti-Ro (SSA) or anti-La (SSB) should have echocardiograms during the 16th and 30th weeks of pregnancy to monitor the health of the heart and surrounding vasculature

Prognosis:

In the 1950s, most patients diagnosed with SLE lived fewer than five years. Advances in diagnosis and treatment have improved survival to the point where over 90% of patients now survive for more than ten years and many can live relatively asymptomatically. The most common cause of death is infection due to immunosuppression , as a result of medications used to manage the disease. Prognosis is normally worse for men and children than for women. Fortunately, if symptoms are present after age 60, the disease tends to run a more benign course. The ANA is the most sensitive screening test and ANTI Sm [antismith] is the most specific and the anti Ds-DNA antibody is also fairly specific and often fluctuates with disease activity. The ds-DNA titer is therefore sometimes useful to diagnose or monitor acute flares or response to treatment.

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