The human animal shares a planet with beings that could pose a serious threat to its life. From times lost in oblivion, we learned to flee from wild creatures and/or of enormous size, natural predators that would finish with us in a simple blink of an eye. Real “walking dangers” that lurked in the darkness.

Yet the most ruthless killers of the human species are so tiny that they hide from our naked eye. We are talking about microorganisms, especially viruses and bacteria, whose voracity has led to the extermination of hundreds of millions of people in very sad recent and remote historical episodes.

In order to survive, which is the purpose of existence, the body needs to orchestrate a series of defenses through which to deal with such pathogens when they inadvertently burst inside. Such an army forms the immune system, a biological battalion of great effectiveness and efficiency.

However, sometimes this same resource (so essential to life) is “confused” and attacks the organism with unusual virulence. Such is the case with systemic lupus erythematosus (SLE) , the most common form of lupus, which will be discussed in this article.

What is systemic lupus erythematosus?

Systemic lupus erythematosus (SLE) is an autoimmune disease , that is, an entity whose origin lies in the altered functioning of the physiological mechanisms with which the body fights external pathogens or other threats to life (such as parasites, viruses or bacteria).

Thus, the defensive arsenal would turn against oneself, affecting very different organs and functions. In this case, the skin, kidneys, joints and brain would be particularly sensitive. As we can see, the consequences can be potentially serious and even fatal.

It is estimated that the prevalence of SLE is around 35-45 affected per 100,000 people , being much more common (4:1) in women than in men (as in other autoimmune diseases, such as multiple sclerosis or Sjogren’s syndrome). Its course is variable and there may be periods of more severe symptomatology. Also, despite the diverse clinical expression of SLE, not all signs arise at the same time, but they may be present at any time. Sometimes such a presentation makes diagnosis difficult, since their peculiarities are spaced out and hardly “interconnected”.

The initial symptoms (which usually occur at puberty but may debut even in the early fourth decade of life) are fever, fatigue, and joint pain; similar to what may be seen during a viral infection (such as “regular” flu). Sometimes a kind of rash can also appear on the face , which is shaped like a butterfly. An earlier onset is suggestive of genetic alterations and extreme rarity, which would require detailed DNA analysis.

Without a doubt, the most important danger faced by all SLE patients lies in complications involving vital organs. This led to the unfortunate circumstance that, a few years ago, virtually all of them died within the first decade after diagnosis (as the process is more aggressive and there is a greater risk of serious bacterial infections). Today, scientific advances in diagnosis and treatment allow up to 92% to live beyond the first few years of the fight against lupus .

Let’s see how this disease could be expressed. It is basic to remember that not all the symptoms must appear at the same moment, but they can appear over time and with different forms of severity.

Symptoms of systemic lupus erythematosus

The following are symptoms of SLE. As noted, this medical condition often progresses in such a way that not all symptoms will be equally intense , or even continuously present. Therefore, it is most common that they manifest themselves abruptly during the acute episodes (or outbreaks), and that they diminish in the intervals between them. However, it is quite possible that fatigue or skin problems may persist in some form. We proceed to delve into the clinical expression of this disease.

1. Skin disorders

Between 66% and 75% of subjects with SLE have skin problems, half of which worsen during direct exposure to sunlight. The most common body locations affected are the face, back, chest, and arms. A butterfly rash is likely to appear over the cheeks or bridge of the nose and is a particularly characteristic symptom of this health condition (in 25% of cases it is the first symptom). In fact, there is the discoid form of SLE, where skin lesions rise as the cardinal feature.

The most common thing in such a pathology is that red discs appear on the skin, with an evident relief to the touch and absence of pain. When time passes, pigmentation marks may appear where the reddish discs were, as well as scar tissue . In the cases in which they emerge on the scalp, there are usually unpopulated areas of hair that alter the personal appearance and may cause a mismatch in self-image. Other times the person presents calcium deposits under the skin (calcinosis), especially when there is some degree of renal failure.

There are many skin problems in SLE, and they require thorough follow-up by the relevant professionals. It is possible that, over time, tissue inflammations will generate permanent marks and even damage the blood vessels. In these cases, one would say that one is suffering from vasculitis, which looks like small purple spots, as well as widely distributed bruises on the body. As one gets older, it is possible that small blackish spots may appear, especially on the tips of the fingers or toes, which would suggest a gangrenous process (and require immediate attention).

Other common skin symptoms in SLE are erythema palmaris or livedo reticularis (abnormal blood flow that leaves a residual blue tone, especially on the legs or face) and petechiae, reddish spots caused by thrombocytopenia or a shortage of platelets. This alteration in blood density is also frequent in SLE, although it does not usually cause bleeding (fortunately).

2. Ulcers

Ulcers are very common problems in SLE, especially those located in the oral cavity, but also in the nostrils and vagina. That is, any mucous membrane in the body may be involved . In general, these are asymptomatic lesions, although they can be painful if they are handled (e.g. when brushing). Sometimes they cause open fissures, in which slight bleeding occurs (although this can be copious if combined with low platelet levels).

3. Alopecia

Most people with SLE report that their hair is excessively fragile, so that it even breaks with the discreet tugging of an everyday hairstyle. Structural analyses point to the thinning of the diameter of the hairs located on the head , which is revealed by the low density of the hair and a particular unkempt appearance. This test (trichoscopy) allows us to differentiate this problem from alopecia areata, whose aetiology is usually genetic but whose expression is very similar to that of SLE (due to the diffuse extension of hair loss).

In some cases lupus causes scattered location “bald spots” that do not remain for long (the affected region recovers as the hair follicles regenerate hair). However, recurrent skin changes, which eventually lead to scarring, do cause irreversible local hair loss. In this case, the characteristic plaques distributed in various points of the head would become evident , which may be a reason for embarrassment or concern .

4. Breathing problems

SLE can affect the very structure of the lungs, the capillaries that enable blood oxygenation and even the diaphragm (muscle that contributes to ventilation and/or breathing). Most commonly, pleuritis, a specific/localized inflammation of the membrane (serous consistency) covering the lungs, occurs. In this case, it is common to refer to more or less intense chest pain, accompanied by dyspnea (effort to breathe) and a feeling of shortness of breath. All this is aggravated by laughing, coughing, breathing in deeply or holding speech for a long time.

When this inflammation persists, it is likely that serious problems will be targeted, such as interstitial lung disease (which is related to scar tissue in these organs, limiting their flexibility and volume) or a thromboembolism in the region that irrigates the adnexal tissue (vascular obstruction that limits blood conduction and releases substances resulting from this situation). In addition, many authors suggest that there may be an increased likelihood of lung cancer, as well as bleeding that requires immediate intervention.

5. Fatigue

Fatigue is a common symptom in SLE, to the extent that it affects 80% of sufferers and merits the descriptive “label” of lupus fatigue. It is a very disabling phenomenon, which sometimes has dramatic consequences (forced abandonment of work or other personal responsibilities) and even conditions the emotional experience (increased anxiety and sadness). The latter can be related in a two-way way to fatigue, as it has been assumed that depression also accentuates the loss of energy and/or vitality.

Respiratory alterations, the restriction of oxygen or the use of certain drugs are at the base of the problem ; and explain why the symptom acquires such a relevant scope in this population, in the same way as it happens in other autoimmune clinical pictures (such as multiple sclerosis). This fatigue is not always easy to diagnose or evaluate, since it would only depend on the subjective account of the person who refers it (fatigue from the first hour of the day, together with an irresistible need to interrupt activities that require some moderate/intense physical effort and problems in the development of daily tasks).

6. Sensitivity to sunlight and cold

Exposure to sunlight can cause an exacerbation of the skin problems patients present , worsening their consequences and their appearance. This is why they often avoid direct exposure to ultraviolet light or light from certain artificial sources (fluorescents), which can affect their serum vitamin D levels.

This photosensitivity also causes a high number of people with SLE to suffer new eruptions on particularly sunny days.

The cold also has a great impact on people suffering from SLE. During the coldest months of the year, many of them report that their fingers and toes acquire blue or white tones at low temperatures (and also in situations of acute stress), which is known as Raynaud’s Phenomenon. Although it may occur spontaneously in individuals who do not suffer from any disease (primary), it is most commonly associated with some form of peripheral (secondary) vasculitis. Its duration is usually about 10-15 minutes.

7. Joint and muscle pain

Inflammation is undoubtedly the base factor for the muscle and bone pain that plagues those who live with this disease. The arthritis of SLE manifests itself through pain, heat, stiffness and swelling (especially at the beginning of the day) over the small and distal joints of the body (hands, feet, wrists, ankles, elbows, etc.).

Most of the time this commitment is bilateral in nature, although it rarely degenerates into structural deformation. As the hours go by it usually softens a bit and its effect on activity is reduced. Up to 90% report some problem of this type.

Inflammation of the muscles is also very possible , which contributes to the above-mentioned fatigue and leads to intense general weakness. The most commonly affected areas are the shoulders, arms, thighs, pelvis and neck. This symptom tends to be one of the most disabling in SLE, although it can be improved with specific physiotherapy.

8. Neurological impairment

SLE can have its impact, too, on the central nervous system. Although we still do not know the exact reasons for this, a not inconsiderable percentage of SLE patients report feeling disoriented and having problems remembering or communicating what they think/feel.

These are effects that are expressed in a fluctuating way, but often constrain basic autonomy and/or reduce quality of life. Moreover, it seems that this cognitive decline is closely related to fatigue and depression.

On the other hand, migraine-type headache is up to twice as frequent among those who suffer from this disease than in the general population. It is also likely that at some point in the course of the disease, the blood vessels in the brain become severely inflamed, which is a very serious situation. Characteristic symptoms are seizures, rigidity and high fever (hyperthermia); although occasionally psychosis-like processes (hallucinations and delusions) may also occur.

9. Alteration in other organs and systems

Lupus can affect the heart, kidneys, skin, brain, intestines and lungs in an extraordinary way ; thus becoming a systemic picture that requires the assistance of many health professionals. From heart valve conditions to declining glomerular filtration rate (GFR), the SUI patient is exposed to a number of risks that he or she must be aware of in order to maintain proper health.

Renal replacement therapies (hemodialysis, peritoneal dialysis, etc.) or other intervention strategies on other potentially affected organs (drugs, surgeries, transplants, etc.) may be essential during the evolution of SLE.

Bibliographic references:

  • Bernknopf, A., Rak, K. and Bailey, T. (2011). A review of systemic lupus erythematosus and current treatment options. Formulary (Cleveland, Ohio), 46, 178-194.
  • Martínez-Godoy, M., Oliva Gutiérrez, E., Zapata Zúñiga, M. and Sánchez-Rodríguez, S. (2012). Systemic Lupus Erythematosus: General features, immunopathogeny and relevant antigens. Archives of Medicine, 8(12), 109-132.