Update: October 2018 Systemic lupus erythematosus or SLE
belongs to the group of systemic autoimmune diseases with unknown
etiological factor. The disease develops due to
genetically determined failures of immune regulation that determine
the formation of organo-specific antibodies to cellular antigens
nuclei and leading to the development of so-called immune inflammation in
tissues of organs. This disease is otherwise called multisystem
inflammatory disease as the lesion occurs
almost all organs and systems: joints, skin, kidneys, head
brain and so on.
The risk group for SLE includes young women who are
in childbearing, especially the Negroid race – about 70%
SLE cases are diagnosed in this particular population group. but
SLE can develop absolutely at any age, even during
newborn. Among the children’s population, the disease is most
common in the age group of 14-18 years old, and more often sick
girls. About the causes of lupus erythematosus, symptoms and
treatment of the disease is our article.
Withчины развития СКВ
The true causes of the development of SLE to date
installed. There are several theories of the development of systemic red
lupus that are controversial and have both confirmatory and
and disproving factors:
- Genetic theory. According to this theory, the disease is
genetically determined. but конкретный ген, провоцирующий
development of SLE, not yet detected.
- Viral Theory Revealed that patients with SLE, often
Epstein-Barr virus is detected.
- Bacterial theory. Proved that the DNA of a number of bacteria can
stimulate the synthesis of anti-nuclear autoantibodies.
- Hormonal theory. In women with SLE, very often
An increased level of the hormones prolactin and estrogen is found.
Also noted is the frequent primary manifestation of SLE during
pregnancy or after childbirth, when the female body undergoes
huge hormonal changes.
- The action of physical factors. It is known that ultraviolet
radiation is able to trigger the synthesis of autoantibodies by the skin cells (in
people predisposed to SLE).
None of the above theories can explain the reason
development of the disease with one hundred percent accuracy. Therefore
SLE is described as a polyetiologic disease, i.e. having
Types of hard currency
The disease is classified according to the stages of the disease:
Острая форма, когда возникает красная волчанка,
symptoms are characterized by a sudden and abrupt manifestation:
significant temperature rise up to febrile indices,
rapid damage of several organs, high immunological
Подострая форма характеризуется периодичностью
exacerbations, however, with a lesser degree of severity of symptoms,
rather than in the acute course of SLE. Organ damage develops in
during the first 12 months of the disease.
Хроническая форма отличается долговременным
manifestation of one or several symptoms. Especially characteristic
combination of SLE with antiphospholipid syndrome in chronic form
Pathogenesis of SLE or what happens in the body
Under the influence of a certain causative factor or at their
combined in conditions of dysfunction of the immune system occurs
�”Exposure” of DNA of different cells. These cells are perceived
own body as alien or antigens. Body immediately
begins to produce particular protein antibodies that are
specific for these cells and protect against them. As a result
interactions of antibodies and antigens form immune complexes,
fixed in certain organs.
This process leads to the development of immune inflammatory
reaction and cell damage. Most commonly affected
connective tissue cells, therefore, SLE disease is referred to
diseases of this particular body tissue. Connective tissue
widely represented in all organs and systems, so almost
the whole body is involved in the pathological lupus process.
Immune complexes with fixation on the vascular walls can
provoke thrombosis. Circulating same antibodies
have a toxic effect and lead to anemia and
Discovery of scientists
One of the last two studies, according to scientists, was
open mechanism controlling the aggression of the human body
against own tissues and cells. This opens up new opportunities.
to develop additional diagnostic techniques and allow
develop effective areas of SLE treatment.
This discovery occurred when the American Office of Control
drug quality was going to sound a decision about
use of the biological drug “Benlist”. This new
the drug “Benlista” (USA), is now approved for use for
treatment of lupus erythematosus.
The essence of the discovery is as follows.
With СКВ организм вырабатывает антитела против собственной ДНК,
which are called antinuclear antibodies (ANA). In this way,
blood test for ANA in a patient with suspected SLE will allow
correctly interpret the diagnosis.
The main mystery of SLE was the mechanism of DNA penetration of cells.
out. In 2004, it was found that explosive cell death
neutrophils lead to the release of their contents, including nuclear
DNA, outward in the form of filaments, between which are easily tangled
pathogenic viruses, fungi and bacteria. Healthy people like
neutrophilic traps easily break up in the intercellular
space. People with SLE also have antimicrobial proteins.
LL37 and HNP do not allow the destruction of nuclear DNA.
These proteins and DNA residues together can activate
plasmacytoid dendritic cells which in turn
produce proteins (interferon) that support the immune response.
Interferon forces neutrophils to release more trap filaments,
supporting an endless pathological process.
Thus, according to scientists, the pathogenesis of lupus erythematosus
consists in a cycle of neutrophil cell death and chronic
tissue inflammation. This discovery is important for both diagnosis and
for the treatment of SLE. If one of these proteins can be a marker
SLE – this greatly simplifies the diagnosis.
Another interesting fact. Among 118 patients,
participating in another research aimed at detecting
vitamin D deficiency in patients with connective tissue diseases.
Among 67 patients with autoimmune diseases (rheumatoid
arthritis, lupus erythematosus) vitamin D deficiency is found in 52%, among
51 patients with pulmonary fibrosis of a different nature – in 20%. What confirms
the need and efficacy of adding to the treatment of autoimmune
Diseases courses of vitamin D.
Symptoms заболевания зависят от стадий развития патологического
process. In acute primary manifestations of lupus erythematosus suddenly
- fever up to 39-39 С
- joint pain
Often patients can pinpoint the start date of clinical
manifestations – so sharp is the symptoms. 1-2 months
formed a clear lesion of vital organs. If the disease
progresses further, then a year or two patients die.
In subacute, the first symptoms are less pronounced,
pathological process develops more slowly – organ damage
arises gradually over a period of 1-1.5 years.
In a chronic course of several years, one is constantly manifested.
or a few symptoms. Exacerbation of the disease occurs rarely
the work of vital organs is not disturbed.
Basically, the initial manifestations of SLE do not have specificity,
pass easily with anti-inflammatory drugs, or
on their own. Remission is different duration of the flow. Early or
late exacerbation of the disease occurs, most often in the autumn-summer
periods due to increased solar radiation, while
the condition of the skin in patients is deteriorating. With time
Symptoms of organ damage occur.
- Skin, nails and hair coat
Involvement in the pathological process of the skin – the most frequent
symptoms of lupus erythematosus in women whose occurrence is associated with
some causal factor: prolonged exposure to the sun
light, stay in the cold, psycho-emotional shock (see
urticaria – symptoms, treatment, allergy to the sun).
Very characteristic of SLE is the reddening of the skin near the nose and
cheeks, shaped like the wings of a butterfly insect. Besides the face
erythema appears on open skin areas – upper limbs,
neckline. Erythema is prone to peripheral overgrowth.
When discoid lupus erythematosus, erythema skin changes
inflammatory edema. This area is gradually compacted and through
some time atrophy with the formation of the scar. Foci of discoid
lupus are found in different parts of the body, indicating that
Another symptom of SLE is capillary, which manifests itself
redness, swelling and numerous hemorrhages in the form of
small points localized on the fingertips, soles and
Hair lesion in SLE is manifested by gradual partial or
complete baldness (see the causes of hair loss in women). During the period
exacerbations are characterized by a change in the structure of the nails, often resulting in
to the atrophy of the periungual roller.
Focal alopecia or generalized, pruritus and urticaria
– are the most characteristic symptoms of SLE. In addition to skin
manifestations of patients worried about headaches, pain in the joints,
changes in kidney and heart function, mood swings from euphoria to
- Mucous membranes
The mucous membranes of the mouth and nose are more common: redness appears,
erosions (enanthema) on the mucous membranes and small ulcers in the mouth are formed (see
ointment for stomatitis, aphthous stomatitis in adults, treatment). With
the formation of cracks, erosion and ulceration of the red border of the lips
Lupus cheilitis occurs. Lesions become dense
bluish-red plaques that are painful when eating, tend to
to stitches, have clear boundaries and are sometimes covered with scaly
- Musculoskeletal system
Up to 90% of patients with SLE have joint damage. Suffer small
joints, more often fingers (see arthritis of fingers).
The pathological process spreads symmetrically, leading to pain
and stiffness in the joints. Necrosis of bone often develops.
aseptic nature. In addition to the joints of the hand, the femoral are affected
and knee joints, which leads to their functional
failure. If the ligamentous apparatus is involved in the process, then
contractures of a non-permanent nature develop, and with severe
during SLE – dislocations and subluxations.
- Respiratory system
Most often affects the lungs with the development of bilateral
pleurisy, acute lupus pneumonitis and pulmonary hemorrhages.
The last two pathologies are life threatening.
- The cardiovascular system
In the prevailing majority of cases endocarditis develops.
Liebman-Sachs with involvement in the pathological lupus process
mitral valve. Valves fold together and form
stenotic heart disease. In the case of pericarditis leaflets
the pericardium becomes thicker. Myocarditis provokes pain in
chest area, increasing heart size. Often suffer
small и средние сосуды (в их числе жизненно важные коронарные
arteries and vessels of the brain), as a result of which patients often die
due to brain stroke and ischemic heart disease.
- Nervous system
Neurological symptoms are diverse, ranging from migraine and
ending with transient ischemic attacks and strokes.
Possible epileptic seizures, cerebral ataxia, chorea.
Peripheral neuropathy develops in a fifth of the patients, with
where inflammation is considered a very unfavorable phenomenon
optic nerve leading to loss of vision.
- Kidney. Severe SLE leads to the formation of lupus
jade of different types.
When is lupus erythematosus diagnosed in children, initially
there are signs in the form of lesions of the joints (volatile arthralgia
character, acute and subacute periarthritis) without progression, and
also typical erythematous rash skin lesions,
anemia appears. It should differentiate SLE with atopic
dermatitis in children.
Chronic lupus erythematosus is differentiated from flat red
depriving tuberculosis leukoplakia and lupus erythematosus, early rheumatoid
arthritis, Sjogren syndrome (see dry mouth, dry
eyes, photophobia). With поражении красной каймы губ хроническую
SLE differentiated from abrasive pre-caberous cheilitis
Manganotti and actinic cheilitis.
Since the defeat of the internal organs is always similar to the flow
various infectious processes, SLE differentiate from disease
Lyme, syphilis, mononucleosis (infectious mononucleosis in children:
symptoms), HIV infection (see HIV infection symptoms — myth or
Treatment of lupus erythematosus
The treatment is chosen individually for a specific patient.
The complex of therapeutic measures is carried out in the outpatient setting.
Indications for hospitalization are:
- persistent hyperthermia for no apparent reason
- life-threatening conditions: malignant renal
insufficiency, acute pneumonitis or bleeding from the lungs
- neurological complications
- severe thrombocytopenia, a significant decrease in red blood cells and
- lack of efficacy from outpatient treatment
Systemic lupus erythematosus in the acute period is treated with hormonal
drugs (prednisone, corticosteroid ointment, see list
hormonal ointments for psoriasis) and cytotoxic drugs (cyclophosphamide) by
pattern. Nonsteroidal anti-inflammatory drugs (Diclofenac, etc., see
list of pain shots) are shown in the presence of hyperthermia and development
lesions of the musculoskeletal system.
With локализации процесса в том или ином органе проводится
consultation of a specialist and the appropriate
People with SLE should avoid being under the straight lines.
sun rays. Exposed skin should be oiled.
cream from UV rays.
Immunosuppressive therapy with own stem cells
very effective, especially in severe cases. In most
cases, autoimmune aggression stops, and the patient’s condition
It is of great importance to maintain a healthy lifestyle, failure
from bad habits, feasible exercise, rational
food and psychological comfort.
Prognosis and prevention
It should be noted that complete cure of SLE to achieve
Prognosis for life with adequate and timely treatment
favorable. About 90% of patients survive 5 or more years after
the onset of the disease. The prognosis is unfavorable for early start
diseases, high activity of the process, the development of lupus
jade, joining the infection. Life prognosis is unfavorable
with the development of SLE in males.
Due to the unknown etiology of primary prevention of SLE is not. For
prevention of exacerbations should avoid direct insolation and
protect the skin as much as possible (clothing, sunscreen, etc.).
Preventing the exacerbations of SLE in children is to organize
home schooling, infection prevention and immunity strengthening.
Vaccination can conduct only in the period of absolute remission.
Introduction of gamma globulin is possible only if there are absolute
Автор: врач-гигиенист, эпидемиолог