LUPUS

LUPUS
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