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 erythematosus

. Subacute cutaneous lupus erythematosus

. 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 .

.

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.

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.

Diagnosis:

Some physicians make a diagnosis on the basis of the ACR classification criteria but other used it for researches only . and patients may have lupus but never meet the full criteria.

patients must meet the following three criteria to be classified as having SLE: (i) patient must present with four of the below eleven symptoms (ii) either simultaneously or serially (iii) during a given period of observation.

1- malar rash

2-..Discoid lupus

3-Discoid lupus

4-Oral ulcers: include oral or nasopharyngeal ulcers

5-Arthritis: nonerosive arthritis of two or more peripheral joints, with tenderness, swelling or effusion

6-Renal disorder: More than 0.5 g per day protein in urine, or cellular cast seen in urine under a microscope.

7- Neurologic disorder , Seizures or psychosis

8- Serositis: Pleuritis, pericarditis

9-Hematologic disorder, Hemolytic anemia, leukopenia, white blood cell count ................
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