There are many people living with a diagnosis of Lupus, an autoimmune disease that can affect various organs. Benedetta de Mattei interviewed the professor. Fabrizio Conti – Head of the Lupus Clinic of the Policlinico Umberto I and Full Professor of Rheumatology at the Sapienza University of Rome – to understand who affects, what are the symptoms and causes of this disease.
WHAT IS LUPUS?
Systemic Lupus Erythematosus (SLE) is a chronic inflammatory autoimmune disease that can affect various organs and systems. The disease can manifest itself with different degrees of severity ranging from mild clinical pictures to very serious situations.
It is estimated that there are over 5 million people with SLE worldwide. It is a disease that affects young women, the peak incidence is observed between 15 and 40 years, with a female-male ratio of about 9: 1. More rarely, the onset is in pediatric or advanced age and, in such cases, the female-to-male ratio drops to 2: 1.
WHAT ARE THE CAUSES OF THIS DISEASE?
SLE is believed to develop to protect against multiple causes some internal others external to the organism. Among these the factors most often called into question are a genetic preparation, hormones and environmental factors. The interaction of these factors determines the activation of the autoimmune process with the production of autoantibodies, that is, directed towards constituents of the organism, which are found in the blood of the patients, even years before the clinical manifestations.
Many researchers today believe that environmental factors such as viruses or chemicals or can trigger disease if they encounter an individual who is genetically predisposed.
Up to now a specific and unique environmental factor has not been identified; the most studied are ultraviolet rays (UVB in particular), infections (in particular the Epstein-Barr virus has been called into question several times), environmental contaminants, estrogen, smoking and emotional stress.
THE MOST COMMON SYMPTOMS
Manifestations of SLE can vary greatly from patient to patient, being able to observe severe forms with the commitment of several organs and extremely mild in which the disease does not modify the normal quality of life.
– Systemic Manifestations: the most frequent at the onset and on the occasion of flare-ups of the disease are above all fever, fatigue, generalized malaise and weight loss.
– Mucocutaneous Manifestations: The commitment to the skin and mucous membranes is found in a high percentage of patients with SLE And the most typical skin manifestation And the malar rash or butterfly erythema (acute cutaneous lupus), an area of redness located on the bridge of the nose and extending laterally to the cheekbones, often triggered by exposure to sunlight. This manifestation must be differentiated from others that can remember it but are not SLE such as rosacea, seborrheic dermatitis and atopic dermatitis.
Skin lesions, sometimes ring-shaped, can appear symmetrically at the level of the shoulders, arms and trunk and do not result in scars (Subacute cutaneous lupus). The chronic cutaneous form, on the other hand, is characterized by erythematous, rounded plaques, with well-defined, slightly raised margins, which can be localized at the level of the face, the retro-auricular region and on the scalp, and can evolve into scarring. Finally, some patients can present manifestations affecting the skin and mucous membranes that are not specific to SLE such as alopecia, ulcers in the mucous membrane of the mouth and nose, liveso reticularis (a purplish marbling of the skin in the shape of a reticulum mostly at the level of the lower limbs), Raynaud’s phenomenon, hives, vasculitis, skin ulcers and more.
– Musculoskeletal Involvement: Most SLE patients suffer from joint pain which is often the onset of the disease. This is mostly a mild inflammation of the joints which, unlike rheumatoid arthritis, rarely results in permanent bone damage. Small joints in the hands and wrists are most often affected, but large joints (elbows, shoulders, knees, ankles) may also be involved. Very popular is the involvement of the tendons (tenosynovitis). Sometimes there is true arthritis: in this case the joints are swollen, red and painful.
– Neurological Manifestations: The involvement of the nervous system during SLE includes a broad spectrum of neurological and psychiatric manifestations currently referred to as neuropsychiatric lupus. The patient may complain of headaches, psychiatric manifestations such as psychosis, anxiety and depression, personality disorders, memory and concentration deficits, epilepsy (which often precedes the diagnosis of SLE by years), strength or speech deficits due to ischemic disorders, peripheral neuropathy and others.
– Cardio-pulmonary manifestations: The most frequent manifestations affecting the heart and lungs are represented by pleurisy and pericarditis, or inflammation of the sheets that line the lungs (pleura) and heart (pericardium) with possible consequent production of effusion (inflammatory fluid) inside these cavities.
– Renal manifestations: The kidney involvement in up to 50% of SLE patients even if in the European populations it is lower, around 30% of cases. The kidney effort can manifest itself with an increased loss of protein in the urine and with the accumulation in the blood of substances that are normally filtered by the kidneys and excreted in the urine. often in some cases these manifestations are very striking and quickly lead the patient to consult the doctor for the resulting symptoms (eg marked and often persistent swelling of the legs and feet), very nephropathy in the course of SLE is particularly subtle and not very symptomatic.
CARE and PROGNOSIS
Treatment of SLE must be based on both the use of drugs than on patient support and education. The recognition by the patient himself of the symptoms of the disease and illnesses allows the therapeutic strategies to be implemented earlier, which in any case can always be with the patient. Furthermore, timely information on the pathology and the drugs used to treat it, allows a better acceptance of the therapy. The disease is characterized by phases of acuteness alternating with periods of remission, the prognosis varies from mild to severe cases but has nevertheless notably improved in recent decades thanks to the possibility of making the diagnosis earlier and better therapeutics. All patients could be treated with antimalarials. The refractory or more severe cases also with cortisone and immunosuppressive drugs. We know that it is essential, after the acute phases, to reduce the dose of cortisone as much as possible to aim for a possible suspension, which however is not always possible. In addition to drugs, lifestyle changes may be suggested (cessation of cigarette smoking, weight control, physical activity). In the end, it is important to avoid direct exposure to ultraviolet rays (sun and UV lamps) and use creams with a high protection factor to avoid possible flare-ups of the disease.
Benedetta de Mattei