Systemic lupus erythematosus: what it is, how to recognize it and how to treat it

Systemic Lupus Erythematosus (SLE) afflicts about 1 in 1,000 people in Europe, mostly females who are affected 9 times more frequently than males. 

The peak incidence is between the ages of 20 and 35, and the course is chronic-recurrent. This means that, in most cases, SLE patients experience alternating periods of relative clinical quiescence of the disease (remission) and periods of flare-ups (flare). 

SLE is a prime example among all so-called “systemic” autoimmune diseases in that it can occur in virtually any organ and system. 

The most frequent symptoms complained of by patients with SLE are at the skin and joint level. The characteristic erythema of the disease, the so-called “butterfly” rash, affects the face in the region straddling the nose. 

Then there are many types of erythema caused by lupus, usually in areas exposed to sunlight, which is recognized as a risk factor for flare-ups. 

Another characteristic symptom is joint pain, sometimes accompanied by: 

• fatigue;
• fever;
• weight loss;
• enlarged lymph nodes.

Other fairly frequent manifestations of the disease are: 

• inflammation of serous membranes such as, for example, the pleura or pericardium;
• reduction in the number of white blood cells, red blood cells, or platelets. 

A minority of patients then develop severe forms with kidney, heart or central nervous system involvement. 

There is no single cause that can determine the occurrence of autoimmune diseases in the individual. Lupus is also no exception, and it is known that there must be a combination of a genetic predisposition and one or more triggering events. The most important ones include: 

• viral or bacterial infections;
• exposure to the sun;
• physical trauma;
• emotional stress;
• taking certain medications;
• hormonal changes. 

Since this is a disease that, as anticipated, can give manifestation of itself in any organ and apparatus, the difficulty of diagnosis arises especially for atypical forms. 

In classic cases, the combination of characteristic symptoms and signs with the demonstration of more or less disease-specific autoantibodies may be sufficient in experienced hands. The most frequently positive autoantibodies in SLE include: 

• ANAs (antinuclear antibodies);
• anti-dsDNAs (anti-DNA double helix antibodies);
• antiphospholipids, the latter particularly important because of their association with thrombosis and pregnancy-related complications. 

In atypical cases, it is essential as a first step to rule out alternative hypotheses and, secondly, to collect all the elements, clinical and laboratory, that are useful for a correct framing of the pathology. Sometimes, a therapeutic attempt may also be used as a confirmatory test. 

Pharmacological treatment of the disease is based for almost all patients on:

• antimalarials as agents that can reduce the likelihood of flare-ups;
• corticosteroids in the acute phases. 

When required, depending on the severity of symptoms or type of organ involvement, traditional immunosuppressants or biologic drugs may be added. 

Along with drug therapy, it is of paramount importance for the patient to associate lifestyle norms that reduce the likelihood of having a flare-up as much as possible, as any relapse of the disease can cause permanent damage to the affected organ in the long term. 

The first behavioral rule to implement is protection from sunlight. “What we generally recommend to our patients is to avoid the sun between 10:00 a.m. and 4:00 p.m. and in any case always with total sunscreen (SFP 100+).”

Other useful rules to increase the effectiveness of drugs and reduce the likelihood of organ damage are: 

• cessation of cigarette smoking;
• proper sleep hygiene;
• regular physical activity;
• healthy diet to reduce overweight. 

To improve quality of life, psychological support is also often important, possibly accompanied by meditation and mindfulness pathways, which are potentially beneficial in almost any problem characterized by chronic pain, as well as reducing stress, a known risk factor for disease flare-ups. 

Since this is a condition that predominantly affects young girls of childbearing age, it is important to address the issue of family planning early on. In fact, pregnancy is now possible even in women with SLE, provided there is adequate consultation with the referring specialist to identify the most appropriate time to plan it and minimize the risk of complications.