Systemic Lupus Erythematosus: Manifestations, Involvement with Disability

Systemic lupus erythematosus is a chronic inflammatory and autoimmune disease. It is an immune disorder that causes damage throughout the body.

Systemic lupus, systemic lupus erythematosus, SLE.

Chronic means that it takes place over a long period of time, namely a lifetime.

It is autoimmune because the body's own defences, and therefore immunity, are involved in the development of the disease, which is directed against the body's own cells, tissues, organs and entire systems.

From the skin and joints, the problems also affect the cardiovascular system, blood, kidneys, lungs, but also the nervous system and others.

People affected by this disease have a higher rate of risk complications. Difficulties develop according to the predominance of damage to a given organ or organ system.

From heart, blood vessel and blood disorders, to kidney, skin and joint problems and other ailments.

Due to its wide range of activities, it can manifest itself differently for everyone.
The course and severity are also individual and variable.

It is therefore not possible to point to clear-cut symptoms and progression.
This sometimes makes it difficult to diagnose.

Of course, even in lupus there are some typical symptoms.

It is also characterised by alternating periods of calm and flare-ups of health problems.

Interesting facts about the disease:
It affects women more often than men, and thus up to 9 out of 10 patients are female.
It can affect children, adults and the elderly.
It develops mostly between the ages of 15 and 45, most commonly between the ages of 20 and 30.
The incidence of new cases is approximately 10 people per 100,000 inhabitants.

Autoimmune basis...

In autoimmune disease, it refers to the process when some components of the immune system react to the body's own cells, tissues and structures. 

This process damages them, resulting in the association of various symptoms and disorders, depending, for example, on the organ affected and the extent of its damage.

Components of immunity attack the body's own antigens. 

The process of autotolerance, i.e. the ability to tolerate these self antigens, is also impaired.

The exact cause is unknown, but it is thought to be a combination of several factors, ranging from genetics, hormonal regulation and the influence of the external environment, such as UV radiation, chemicals or infections.

We are familiar with a large number of autoimmune diseases and disorders in which this pathologically disturbed process plays a role.

Examples include psoriasis, thyrotoxicosis, type I diabetes, multiple sclerosis, rheumatoid arthritis, scleroderma, and others.

Want to know more about lupus?
How does it arise and manifest?
Is there a treatment?
Read on.

What is lupus

Systemic lupus is a severe chronic autoimmune disease. It is multi-organ in nature and also damages vital organs such as the heart, brain and kidneys.

The development of the disease is characterized by the overproduction of autoantibodies against intracellular antigens.

Systemic = damage to multiple systems. 
Lupus = Lupus is Latin for "wolf", typical skin manifestations that resemble the spots on a wolf.
Erythematosus = red skin manifestations. 

Lupus, i.e. wolf.

It is believed that doctors in the past were reminded of the skin changes by the spots on the face of the wolf. But also because they resembled wolf bite marks.

Moreover...

Lupus may also come from French "masque loup", i.e. "wolf mask", because this disease can manifest itself as an inflammation of the skin of the face, which has the outline of a carnival mask.

As in the past, women were more likely to be affected, by a ratio of 9:1. It also occurs in childhood. However, the main period of development is between the ages of 20 and 45.

A prerequisite for this age range are also the aforementioned hormonal changes.

Why does lupus arise? The next section will try to answer that.

Autoimmune diseases have diverse causes. The same is the case with lupus.

The exact triggering cause is not yet known.
It affects approximately 5 million people worldwide.
0.5 million in Europe.

However, a multifactorial basis and the action of various factors are assumed.

Genetic predisposition, hormonal control and the influence of the external environment come to the fore, through UV radiation, chemical substances to the infectious basis.

In the development of the disease, an autoimmune response is reported when B lymphocytes attack the body's own antigens. Abnormal activation of B cells directed at the body's own tissues and organs, leading to their damage and malfunction.

= disorder of autotolerance and immunity. 
Genetic predisposition + hormonal component + exogenous factor + age = systemic lupus erythematosus (SLE)

Some risk factors involved in the development of SLE:

• age
• genetic basis, HLA, HLA-DR3 or HLA-DR2 disorder
• partly familial predisposition
• hormonal changes, puberty, pregnancy, hormonal contraception, estrogen and prolactin or androgen levels
• external factors
  • UV radiation and sun exposure
  • chemicals (hydrazines, dyes, aromatic amines, silicone, asbestos, vinyl chloride)
  • certain medications (isoniazid, procainamide, anticonvulsants)
  • infection, mainly viral
  • certain food ingredients such as mushrooms, psoralens, smoked
  • stress and excessive psychological burden
  • smoking
  • long-term physical stress
  • lack of rest

SLE is not an infectious and communicable disease. 

The symptoms of systemic lupus are varied and diverse. The main reason for this is that it is a multi-organ disease that affects multiple structures, organs and organ systems, even at the same time.

It affects the skin, joints, heart, lungs, kidneys, brain or digestive system. In addition to the disorder of individual organs, there are also general manifestations.

According to the predominance of severity, SLE is subsequently divided into several forms, such as chronic cutaneous, with less severe organ involvement - subacute, and with severe organ involvement - acute.

The main symptoms and characteristic warning signs of lupus include:

• malar or butterfly rash - a rash resembling a butterfly
  • redness on the face in the area of both cheeks, joining at the root of the nose
• sensitivity to light - "sun allergy"
  • the skin reacts violently after exposure
  • the covered skin under the clothing is free from the presence of a skin reaction
• defects of the mucous membranes of the mouth and nose
• joint inflammation + swelling and pain especially in small joints
• kidney damage and impaired function
• neurological problems such as headaches, mood, memory and concentration disorders, depression, even convulsions
• damage to blood vessels and the heart
  • Raynad syndrome = purple ends, fingers cool
  • atherosclerosis
  • anemia
  • inflammation of blood vessels
  • enlarged small coils
• lung problems
• damage to the digestive tract
• visual disturbances such as scratching and irritation of the eyes, inflammation
• overall symptoms
  • fatigue
  • exhaustion
  • increase in body temperature
  • loss of appetite
  • weight loss

Table: possible symptoms occurring in SLE

In the diagnosis, the patient's medical history is important. A person describes his difficulties that lead to the diagnosis of an autoimmune disease. To it is added the examination of the patient.

Laboratory blood tests, more precisely blood counts, which give an overview of the levels of blood components or blood sedimentation and CRP, are also important. Evidence of autoantibodies is added.

For the diagnosis are established: criteria for the classification of systemic lupus erythematosus.

Criteria for diagnosis = presence of symptoms:

1. butterfly rash - redness of the cheeks and root of the nose
2. circular rash
   • circular skin changes
   • hair loss in a circular shape
   • face, hairy part, neck, shoulders, arms, upper chest
3. photosensitivity - sensitivity to light and exaggerated reaction to skin illumination
4. defects in the cavity of the mouth and nose, small wounds on the mucous membranes of the mouth and nose
5. inflammation of the joints
   • swelling and pain in small joints
   • hands and fingers, wrists, elbows, knees, ankles
   • without deformities as in rheumatism
6. serositis = inflammation of the pleura and pericardium
7. kidney involvement - the most common complication
   • impaired function associated with high blood pressure
   • swelling of the lower limbs, eyelids
   • blood and protein in the urine
8. neurological problems - headaches, memory and concentration problems, mood changes, depression, and other mental disorders
9. haematological problems
   • haemolytic anaemia - a reduction in the number of red blood cells
   • leukopenia - a decrease in the amount of white blood cells
   • thrombocytopenia - a decrease in platelets, associated blood bruising and bleeding in the digestive tract, urine and others
10. immunity disorder
11. antibody values

The course of the disease varies both in severity and in the association of difficulties. There is no one typical course that can be used to assess this disease. 

Therefore, the presence of variable and heterogeneous symptoms is the reason for frequent diagnostic errors and prolonged search for the correct disease.

SLE is characterized by periods of quiescence (remission) that alternate in time with the presence of acute discomfort (exacerbation).

In addition to the typical symptoms, ranging from general weakness and fatigue to skin manifestations, there are associated health complications that arise from the involvement of a specific organ or organ system.

As listed in the symptom table, symptoms from damage to any organ and organ system can be added. 

The occurrence of multiple symptoms and their mutual combinations is a possibility.
Skin and joint manifestations are accompanied by organ involvement.

Symptoms can come on gradually, but also all at once. Sometimes it is a milder course that does not limit a person's daily activities. Other times it is the cause of disability and significantly reduces the quality of life of the affected person.

The course can be aggravated by physical exertion, stress and mental strain, as well as a heavy and inappropriate diet. 

The disease mostly affects women, but may have slightly different features in men.

It has been reported that the course may be more severe in men, with a worse prognosis. Also, the diagnosis of the disease occurs on average after the age of 30, with women mainly diagnosed before the age of 30.

The overall prognosis therefore depends on the organ involvement and impairment of organ function.
Examples include severe stroke or heart attack, as well as kidney damage associated with kidney failure and high blood pressure.

The kidneys are one of several blood pressure regulators in the human body.

Systemic lupus and pregnancy

Lupus usually develops in young women during the fertile period. Therein lies the assumption of frequent involvement of young women in synergy with hormonal influence.

If a woman manages to become pregnant, the pregnancy must be considered as a risk pregnancy, i.e. a high-risk pregnancy. 

Fertilization occurs mostly in the period of decline of the disease without the presence of acute difficulties. 

The problem is that, in the first place, pregnancy itself and hormonal changes can lead to an exacerbation, i.e. a flare-up.

Exacerbation, i.e. the re-emergence of the disease, means that the disease moves from a phase without symptoms (remission) to a symptomatic phase, i.e. with symptoms present.

At that time, it can put the pregnancy at risk.

The second component is that the disease can negatively affect the course of pregnancy. The risk is the development of pre-eclampsia up to miscarriage.

The risk of miscarriage is reported to be 20-30%. 
SLE is a cause of infertility and the inability to bear a fetus. 

It is believed that the main mechanism is thrombosis of fetal blood vessels, the consequence of which is fetal anemia. An under-blooded fetus does not get enough oxygen and nutrients, which will cause its death.

As prevention, the application of blood medications is used during pregnancy. These prevent blood clotting and the formation of thrombi.

Increased risk is head in women with the occurrence of antiphospholipid syndrome,
already at the time before pregnancy.

The fetus is at risk of developing lupus neonatorum.

Generally...
Although the disease cannot be completely cured, its treatment effectively helps to alleviate complications and prolong the survival of people affected with SLE.