Lupus Erythematosus disseminated
The lupus erythematosus (SLE) is a systemic disease, ie involving several organs, connective tissue, which manifests itself differently in different individuals. The word is associated lupus.
His presentation is multifaceted, with damage or muscle, skin manifestations, kidney damage, but also problems blood inflammation in the lungs or heart and neurological or psychiatric, fever. These attacks are often accompanied by fatigue or latent passenger, a weight loss and irritability. This heterogeneity of symptoms sometimes makes diagnosis difficult lupus is multifaceted and specific to each individual, sex and age groups. The evolution is conditioned by kidney damage and brain of the LED. There is however a decline in activity of this disease for women after menopause.
Some diseases cousins are sometimes associated with lupus, such as Raynaud's syndrome or syndrome Goujerot-Sjögren.
Nine women are affected for a man. Its prevalence is approximately 40 cases per 100 000. It is five times higher among black people.
It mainly affects young women between 20 and 40 years and is unusual before age 7.
Etiology (Causes and factors)
The causes remain unknown.
Some medications (procainamide, quinidine, hydralazine) can give syndromes lupiques limited damage to joints and skin.
Sun exposure is typically a factor favouring outbreaks of the disease.
A recent infection with the virus Epstein-Barr could play a role in triggering the disease.
There is also a genetic factor, with a concordance among twins almost four times higher than among fraternal twins. Similarly, some HLA groups are more frequently found , and deficits in some additional factors.
Mechanism of the disease
The lupus erythematosus is primarily an autoimmune disease: antibodies specific molecules themselves are produced by the immune system of the individual patient. These molecules abnormally recognized as foreign issues:
* Spliceosomes (useful alternative splicing)
* A ribonucleoprotein complex cytoplasmic.
These entities are present in cellular virtually all cells and therefore likely to be detected when a cell is lysée. This in turn led to the destruction of other nearby cells, causing symptoms being raised and the seriousness of the disease.
The reasons for the female clear of the disease are not clear although there is a hormonal influence as evidenced by the more frequent outbreaks of lupus among postmenopausal women aynt hormone replacement therapy.
The first manifestations
The first outbreak appears often after a particular event: stress, pregnancy, taking medication, exposure to sun, which usually leads the person to make a causal relationship. In fact these events serve as revealing in a disease that already existed.
The symptoms are not specific lupus, they can think of other diseases. Given the extreme polymorphism, the diagnosis is often difficult. It will be made only after a thorough interrogation and examination of blood. It uses the criteria of the Association of American rheumatologists (ARA).
Four of these criteria must be present to say a diagnosis of lupus. They are present in 96% of LED:
1. rash on the face of a butterfly wing,
2. eruption of discoid lupus,
4. mouth ulcers or nasopharyngées,
5. polyarthritis non-erosive,
6. pleurisy or pericarditis,
7. nephropathy: proteinuria> 0.5 g/24 h or cylindrurie,
8. comitiales crises or psychosis,
9. haematological disorders: hemolytic anemia or leukopenia <4000/mm ³ or lymphopenia <1500/mm ³ thrombocytopenia or <100 000/mm ³
10. immunological abnormality: THE presence of cells (cells Hargrave) or anti-native DNA or antibodies sm or false positive serology syphilitic,
11. presence of a high rate antinuclear.
The demonstrations show rheumatological disease once in two, and are almost constant during the evolution (90%).
25% of events rheumatological are simple arthralgia (pain), schedule inflammatory (especially at rest and relatively calmed down after a mobilization of articulation). They are migratory and transient.
In 75% of cases, these are real arthritis involving the joint pain and inflammatory signs (redness, warmth local increased volume increase of articulation), variable. This may be a trace or acute rheumatoid fever, sometimes bilateral and symmetrical, but is most often asymmetrical. It can be as subacute or chronic. The arthritis are contemporary or not a violation of other organs (kidneys, brain…).
Lupus is different from rheumatoid arthritis by its little or no destructive, joint deformities are secondary to ligament injuries capsulo-rheumatism Jaccoud with deformities of hands reducible without destroying joints.
If the first sign is a destructive monoarthrite should always refer to an infection.
The joints most often affected are:
* Metacarpophalangeal joints phalangiennes (in your fingers);
* Proximal interphalangeal joints (base of the fingers);
* Carp (wrist);